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Unlabelled: The aim of the study was to investigate the clinical presentation and prognosis of recurrent facial nerve palsy (RFNP) in children. The files of 182 patients referred to the Schneider Children's Medical Centre of Israel for neurological evaluation of isolated peripheral facial nerve palsy between October 1992 and December 1998 were reviewed. RFNP was found in 11 patients (9 females, 2 males), with an incidence of 6%. In two males, the aetiology was traced to Melkersson-Rosenthal syndrome and these patients were separated from the rest of the group. Three children had two episodes of facial nerve paresis which completely resolved clinically within several weeks. Six other children underwent electrophysiological studies. Two of the latter with residual neurological damage, and one child with abnormal blink reflex only, showed decreased facial nerve conduction velocity and abnormal blink reflex. Three children with complete recovery had disturbed blink reflex only with normal nerve conduction. Brain imaging studies as well as laboratory work-up were non-contributory in all cases.Conclusion: The frequency of recurrent facial nerve palsy in children was similar to that in adults. The most significant factors in the evaluation of recurrent facial nerve palsy are medical history and physical findings at diagnosis and after short follow-up. In our patients, electrophysiological studies did not have either clinical or prognostic significance. The rate of full clinical recovery is about 70%, lower than in Bell palsy. [ABSTRACT FROM AUTHOR]

Aim: This study compared prevalence and characteristics of headaches between pediatric patients with chronic kidney disease and pediatric patients with transplanted kidneys and identify factors associated with headaches in the entire cohort., Methods: We interviewed 87 children and adolescents with either chronic kidney disease or transplanted kidney, regarding the prevalence of headaches and their characteristics. We reviewed hospital charts for medical history and blood test., Results: Twenty-two patients (25.3%) reported experiencing headaches, of them 15 (68%) had migraine. The prevalence was greater among those with chronic kidney disease than among those after kidney transplant: 36.6% vs 15.2%, P  = .03. Headache, mostly migraine, was associated with lower glomerular filtration and higher phosphate level., Conclusions: In a pediatric population, headaches were less prevalent among patients after kidney transplantation than among patients with chronic kidney disease. The lower headache rate after kidney transplantation may be related to improvement in homeostasis and electrolyte balance.

Purpose of Review: This work aimed to review the epidemiology, clinical criteria, and primary and secondary diagnoses of pediatric thunderclap headache and to compare to adult thunderclap headache., Recent Findings: Thunderclap headache among children aged 6-18 years are rare; this headache presented in 0.08% of the patients admitted to a pediatric emergency department in a tertiary pediatric center. In that recent single-center study, thunderclap was a headache of grade 10 on the pain scale and conferred a benign course. Contrary to adults, in children, most thunderclap headaches are due to either a primary thunderclap headache or another type of primary headache. A number of case reports have attributed pediatric thunderclap to reversible vasoconstriction syndrome and bleeding due to intracranial aneurysm. However, 3-year data from a pediatric emergency department of one center did not find these reasons to be causes of secondary thunderclap headache. This may be due to the rarity of these diagnoses in children compared to adults. Four of the 19 patients with thunderclap headache reported in that single study had secondary thunderclap; the causes were infection in three and malignant hypertension in one. All the patients had a benign course. Although urgent imaging and lumbar puncture are required in the workup of pediatric thunderclap, severe causes are very rare. More research is needed to investigate pediatric thunderclap headache., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Adult abrupt severe non-traumatic headache (thunderclap) is often related to serious underlying etiologies such as subarachnoid hemorrhage. However, data are sparse regarding thunderclap headache in the pediatric population., Objective: The aim of the study was to evaluate the prevalence, characteristics and causes of thunderclap headache in the pediatric and adolescent population, aged 6-18 years, presenting to a pediatric emergency department., Methods: The electronic database of a tertiary care pediatric emergency department was searched for children presenting with acute headache during 2016-2018. Headache severity was defined by pain scales, either a visual analogue scale or by the Faces Pain Scale-Revised., Results: Thunderclap headache was diagnosed in 19/2290 (0.8%) of the included patients, all of them with a pain score of 10/10. All the patients had a benign course. Primary headache was diagnosed in 15/19 (78.9%), six patients had migraine and eight were diagnosed with primary thunderclap headache. Four of the 19 patients were diagnosed with secondary headache: three with infectious causes and one with malignant hypertension., Conclusions: Thunderclap headache is rare among children and adolescents presenting to the emergency department. This headache is generally of a primary origin. Extensive evaluation is still needed to rule out severe diagnosis problems.

Objectives: To describe the characteristics and treatment of trigeminal neuralgia in children attending a dedicated pediatric headache clinic., Background: Data on trigeminal neuralgia as a cause of headache are largely derived from adult studies. Little is known about the etiology, symptoms, treatment, and outcome of the disorder in children and adolescents., Methods: A case series study was undertaken. The database of a headache clinic within a tertiary, university-affiliated, pediatric medical center was searched for all patients aged 3-18 years presenting with clinical and epidemiological features of trigeminal neuralgia or trigeminal neuropathy from January 2015 to December 2019. The diagnosis was revised for the present study according to the criteria of the International Classification of Headache Disorders, third edition. Data on demographic parameters, clinical symptoms, treatment, and outcome were collected from the medical files., Results: Of the 1040 patients who presented to our clinic during the study period, five (0.5%) were diagnosed with trigeminal neuralgia. Mean patient age was 15.1 ± 3.0 years (range 9.5-17.5; 95% CI 10.8-18.9). All had idiopathic type: purely paroxysmal in one and with concomitant continuous pain in four. Findings on herpes serology in all five and magnetic resonance imaging were normal in four patients. In the fifth, a vascular ring was noted from the superior cerebellar artery around the right trigeminal nerve without radiologic evidence of vascular nerve compression. All patients were initially treated with carbamazepine: one reported partial relief, two did not respond, and two had severe adverse effects. Three patients were switched to gabapentin but only one responded well. Three patients were treated with nerve block., Conclusion: Trigeminal neuralgia accounted for only a small proportion of patients seeking treatment for headache in pediatric headache clinic over a 5-year period. Unlike findings in adults, vascular compression was not the underlying mechanism in any of our patients. The response to pharmacologic treatment was poor. Nerve block may serve as an alternative when pharmacologic treatment fails., (© 2020 American Headache Society.)

Background/objective: The International Headache Society lists infantile colic under "episodic syndromes that may be associated with migraine" in the ICHD3-beta version of its classification of headache disorders. The aim of this study was to examine whether this association is specific to migraine or applies to all pediatric headache types., Methods: A cross-sectional historical study was conducted including 219 patients aged 3-18 years who presented to a tertiary pediatric headache clinic in 2016-2017. Parents were asked a series of questions to determine if their child had had infantile colic as defined in the ICHD3-beta version. The prevalence of a positive history of colic was compared between children diagnosed with migraine or other primary headache types., Results: There were 132 girls (60.2%) and 87 boys (39.8%) of mean age 12.8 ± 3.48 years at presentation. Migraine headache was diagnosed in 170 patients (77.6%) and other types primary of headache (9 in total) in 49 patients (22.3%). Fifty-one patients had a history of infantile colic. They included 45 patients in the migraine group (26.5%) and 5 in the comparison groups (10.2%); the difference in the rate of colic was statistically significant ( P = .0196; OR 3.17, 95% CI 0.1.17-6.17). There was no association of specific migraine parameters or symptoms with infantile colic., Conclusion: There appears to be an association of infantile colic with pediatric migraine but not with other types of pediatric headache. These findings reinforce the theory that infantile colic has common pathogenic roots with migraine.

This study sought to investigate the need for thrombophilia screening in pediatric migraineurs. The cohort included 45/824 children (5.5%) aged 3-18 years with migraine who were tested for thrombophilia at a tertiary pediatric headache clinic. Results were analyzed by background factors and indications for screening. Rates of thrombotic factors were compared with a healthy historical control group. At least 1 thrombotic factor was positive in 19/45 patients (42%). The total thrombophilia risk rate was higher in patients with aura (n = 32). Lipoprotein(a) was the factor most often abnormal in the thrombophilia group of all factors tested (8/19, 42%), regardless of migraine type or gender. It was the only factor with a significantly higher prevalence in the migraine than the historical control group. Full thrombophilia testing in migraine in pediatric headache clinics does not seem to be justified. The high prevalence of elevated lipoprotein(a) in children with migraine warrants further investigation.

Background: Allodynia is prevalent in adults with migraine and has been associated with long disease duration and severe course. Studies of the pediatric population are sparse. The aim of this study was to evaluate the rate of cephalic cutaneous allodynia in children and adolescents within the first 6 months of migraine onset and to identify associated clinical and migraine-related parameters., Methods: The electronic database of a tertiary pediatric headache clinic from 2014 to 2017 was retrospectively searched for all children and adolescents diagnosed with migraine headache within 6 months or less of symptom onset. Cephalic cutaneous allodynia was identified by validated questionnaire. Demographics, symptoms, and headache-related parameters were compared between patients with and without allodynia., Results: The cohort included 119 patients, 69 girls (58.0%) and 50 (42.0%) boys, of mean age 11.6 ± 3.6 years. Mean time since onset of migraine disease was 3.6 ± 1.8 months. Cephalic cutaneous allodynia was reported by 31.1% of patients. It was significantly associated with female gender ( p = 0.03), older age at admission ( p = 0.037), older age at onset ( p = 0.042) migraine with aura ( p = 0.002), and higher rate of awakening pain ( p = 0.017)., Conclusions: Cephalic cutaneous allodynia may occur in children and adolescents already in the first 6 months of migraine onset. Contrary to adult studies, we found no association of allodynia with migraine frequency or long disease duration. Allodynia was significantly associated with migraine with aura, female gender, and awakening pain. A genetic tendency may contribute to the appearance of allodynia in the pediatric age group.

We investigated the prevalence of Mycobacterium marinum lymphadenitis and describe 4 children with the disease. The database of the microbiology laboratory of a tertiary pediatric medical center was searched for all cases of nontuberculous mycobacterial lymphadenitis from 1996 to 2016. M. marinum lymphadenitis was defined as isolation of the pathogen from a lymph node or from a skin lesion with an enlarged regional lymph node. M. marinum was isolated from lymph nodes in 2 of 167 patients with nontuberculous mycobacterial lymphadenitis and from skin lesions in 2 children with skin lesions and regional reactive lymphadenitis, yielding a 2.4% prevalence of M. marinum lymphadenitis. All 4 affected children were younger than 7 years and had been referred for evaluation of enlarged lymph nodes. Preauricular/submandibular and inguinal lymph nodes (n = 2 each) were involved. Three patients had skin traumas and visited the same natural spring. The diagnosis was delayed because a history of aquatic exposure was initially missed. Two children were managed with anti-mycobacterial antibiotics and 2 by observation only. All showed good resolution., Conclusion: A detailed history, specifically regarding exposure to spring water sources, in cases of lymphocutaneous syndrome can point to the diagnosis of M. marinum infection. What is Known: • M. marinum can cause chronic nodular or ulcerative skin infections. • Lymphadenitis due to M. marinum has rarely been reported. What is New: • M. marinum infection can present as isolated chronic lymphadenitis; it accounts for about 2.4% of all cases of nontuberculous mycobacterial lymphadenitis and it tends to occur in noncervicofacial regions relative to infections of other nontuberculous mycobacterial species. • Careful history taking including water source exposure, especially in association with skin trauma, can point to the correct diagnosis in children with chronic lymphadenitis.

Purpose of Review: The purpose of this work was to review the current literature on the epidemiology and pathophysiology of pediatric obesity and migraine, underlying pathogenic mechanisms that may explain the association between the two disorders, and the effects of treatment., Recent Findings: In children and adolescents, the bulk of the available data support an association between obesity and headache disorders in general, though a small number of studies contradict these findings. Relative to the adult population, however, few studies have focused specifically on migraine, and no wide-ranging meta-analyses have been conducted to date. It seems that the pathophysiology of obesity and migraine in adults holds true for the pediatric population as well. The association between obesity and migraine in the pediatric population is likely to be multifactorial and to involve both central and peripheral mechanisms. More attention is currently being addressed to the role of the hypothalamus and the bioactive neurotransmitters and neuropeptides that modulate energy homeostasis, namely serotonin, orexin, and the adiponectins, in migraine. A few innovative studies have demonstrated some benefit for migraine from weight reduction treatments such as exercise and lifestyle management. Many open questions remain regarding the modifiable nature of the obesity-migraine relationship and its implications in clinical practice. Further studies of these issues are needed.

Background Several studies have reported dialysis-related headache in adults. We investigated headache and its characteristics in pediatric and adolescent patients with chronic kidney disease and patients treated with dialysis, and compared characteristics of patients with and without headache in the entire cohort and separately among dialysis and among chronic kidney disease patients. Methods Patients and their parents who attended a nephrology clinic and hemodialysis unit were interviewed regarding the existence of headache and its characteristics. We reviewed hospital files for medical history, blood test results, and pharmacologic treatment. Headache was defined according to International Headache Society criteria. Results The cohort comprised 60 patients: 39 with chronic kidney disease without hemodialysis and 21 treated with hemodialysis; 39 were males, mean age 11.9 ± 5.3 years. Twenty-six (43.3%) reported experiencing headaches. The hemodialysis group had a higher rate of headache than the chronic kidney disease patients, at 76.2% vs. 25.5%, p < 0.001. In the hemodialysis group, 15 out of 16 reported dialysis-related headache; 14 (87.5%) of these had migraine characteristics. For the entire cohort, headache was associated with hemodialysis, chronic kidney disease grade, lower glomerular filtration rate anemia and a higher parathyroid hormone level. In logistic regression analysis, glomerular filtration rate was significantly associated with headache, odds ratio 2.74 (95% CI 1.56-4.82, p < 0.001). Conclusions A high rate of headache, mostly migraine type, was reported by hemodialysis patients. Hemodialysis, anemia, higher parathyroid hormone levels, phosphate, and lower glomerular filtration rate are strongly associated with headache among chronic kidney disease pediatric and adolescent patients.

Migraine is known to run in families and has long been considered a strongly heritable disorder. We sought to investigate the age of onset of migraine between successive generations. Our retrospective cohort included 102 children with migraine who were referred to a pediatric headache clinic and their affected parent(s). Age at migraine onset was significantly lower in the children with a history of maternal or paternal migraine than in their mothers or fathers ( P < .001). In conclusion, data on parental history of migraine showed that children with migraine were significantly younger at first appearance of the disease than their affected parents.

Objective To compare comorbidities between migraine and tension headache in patients treated in a tertiary pediatric headache clinic. Methods Files of patients with migraine or tension headache attending a pediatric headache clinic were retrospectively reviewed for the presence of organic comorbidities. Additionally, patients were screened with the self-report Strengths and Difficulties Questionnaire to identify nonorganic comorbidities. If necessary, patients were referred to a pediatric psychiatrist, psychologist or social worker for further evaluation. Results The study cohort comprised 401 patients: 200 with migraine and 201 with tension headache. The main organic comorbidities were atopic disease, asthma, and first-reported iron-deficiency anemia; all occurred with statistical significance more often with migraine than with tension headache (Familial Mediterranean fever was six times more frequent in the migraine group than in the tension headache group, but the difference was not statistically significant. Nonorganic comorbidities (psychiatric, social stressors) were associated significantly more often with tension headache than with migraine (48.3% versus 33%; p = 0.03). Conclusions Children and adolescents with migraine or tension headache treated in a dedicated clinic have high rates of organic and nonorganic comorbidities. In this setting, patients with migraine have significantly more organic comorbidities, and patients with tension headache, significantly more nonorganic comorbidities.

Background: The available data on gender differences in clinical migraine parameters among pediatric patients are based on relatively few studies, which did not use the current version of the International Classification of Headache Disorders (ICHD) of the International Headache Society. The aim of the present study was to compare between males and females, demographic and clinical characteristics of children and adolescents with migraines diagnosed according to the ICDIII-beta version., Methods: The electronic database of a tertiary pediatric headache clinic was searched for all children and adolescents diagnosed with migraine headaches in 2010-2016. Data on demographics, symptoms, and headache-related parameters were collected from the medical files. Findings were compared by gender., Results: The cohort included 468 children and adolescents of mean age 11.3 ± 3.6 years; 215 males (45.9%) and 253 females (54.1%). Migraine without aura was documented in 313 patients (66.9%), and migraine with aura in 127 (27.1%); 28 patients (6.0%) had probable migraines. The female patients had significantly higher values than the male patients for the following parameters: age at admission (p = 0.042, Cohen's d 0.8303, 95% CI 0.614-0.992); age at migraine onset (p = 0.021, Cohen's d 0.211, 95% CI 0.029-0.394); rate of migraine with aura (OR 2.01, 95% CI 1.29-3.16, p = 0.0056); headache frequency (p = 0.0149, Cohen's d 0.211, 95% CI 0.029-0.3940); rate of chronic migraine (p = 0.036, OR 1.54, 95% CI 1.02-2.34); and puberty (OR 3.51, 95% CI 2.01-6.35, p = <0.001). Males had a higher rate of vomiting (OR 0.62, 95% CI 0.41-0.93, p = 0.018). Further analysis by pubertal stage revealed that pubertal females, but not prepubertal females, had a significantly higher rate of migraine with aura than did males (41.1% versus 28.9%; OR 1.42, 95% CI 0.85-2.37, p = 0.039)., Conclusion: Female children and adolescents with migraine treated in a tertiary pediatric headache clinic were characterized by a higher rate of chronic migraine and migraine with aura, a lower rate of vomiting, and older age at onset relative to males. These findings might be influenced by the better description of migraine symptoms by females owing to their better verbal ability.

Objective/background: To investigate the characteristics of vomiting in pediatric migraineurs and the relationship of vomiting with other migraine-related parameters., Methods: The cohort included children and adolescents with migraine attending a headache clinic of a tertiary pediatric medical center from 2010 to 2016. Patients were identified by a retrospective database search. Data were collected from medical files. The presence of vomiting was associated with background and headache-related parameters., Results: The study group included 453 patients, 210 boys (46.4%) and 243 girls (53.6%), of mean age 11.3 ± 3.7 years. Vomiting was reported by 161 patients (35.5%). On comparison of patients with and without vomiting, vomiting was found to be significantly associated with male gender (54% vs 42.1%, P < .018), younger age at migraine onset (8.0 ± 3. years vs 9.6 ± 3.7 years, P < .001), younger age at clinic admission (10.5 ± 3. years vs 11.6 ± 3.6 years, P = .002), higher rate of awakening headache (64.1% vs 38.7%, P < .001), lower headache frequency (10.5 ± 10.3 headaches/month vs 15.0 ± 11.7 headaches/month, P < .001), higher rate of episodic vs chronic migraine (67% vs 58.7%, P < .001), and higher rates of paternal migraine (24.1% vs 10.1%, P < .001), migraine in both parents (9.3% vs 3.1%, P = .007), and migraine in either parent (57.5% vs 45.5%, P = .02)., Conclusions: The higher rate of vomiting in the younger patients and the patients with awakening pain may be explained by a common underlying pathogenetic mechanism of vomiting and migraine involving autonomic nerve dysfunction/immaturity. The association of vomiting with parental migraine points to a genetic component of vomiting and migraine. It should be noted that some of the findings may simply reflect referral patterns in the tertiary clinic., (© 2017 American Headache Society.)

Aim: Migraine is known to run in families and has long been considered a strongly heritable disorder. This study sought to evaluate the relationship between age at onset of pediatric migraine and family history of migraine., Methods: Review of the medical files of the headache clinic of a tertiary pediatric medical center yielded 344 children with migraine for whom details on migraine in family members were available., Results: Mean age of the cohort was 11.69 ± 3.49 years, and mean frequency of headache per month, 13.68 ± 11.26. Mean age at migraine onset in patients with a negative parental history was10.48 ± 3.39 years; in patients with one parent with migraine, 8.84 ± 3.72 years; and in patients with both parents with migraine, 7.32 ± 3.22 years (p < 0.001).The duration of migraine attacks (in hours) was significantly longer in patients with any family member with migraine than in those with no family history (p = 0.026)., Conclusions: Among children attending a tertiary pediatric headache clinic, migraine appears at a younger age in those with parental history of migraine than in those with a negative family history. The findings suggest that having a genetic background of migraine makes a child more susceptible to migraine earlier in life than a child without a family history., (© International Headache Society 2014.)

The link between body weight and headache has hardly been examined in children. The aim was to evaluate the association of obesity and migraine in selected pediatric patients and compare the findings with the literature. Data on clinical symptoms, headache type, and body mass index standard deviation score were derived from the medical files of 245 patients with migraine and 87 with tension headache. Comparison of the 3 subgroups of migraine patients of normal weight, overweight, and obese with the corresponding body mass index standard deviation score subgroups of patients with tension-type headache yielded no statistically significant differences in frequency of headache attacks per month, or duration of headache attacks in hours. These results call into question earlier reports linking headache and obesity in children. Differences in findings between our study and those in the literature highlight several factors that should be addressed in further studies. A larger sample size may reveal more significant results., (© The Author(s) 2014.)

Objective/background: Arachnoid cysts are generally identified incidentally on brain imaging, although they occasionally cause symptoms because of expansion or bleeding. This study aims to describe patients in whom an arachnoid cyst was identified on magnetic resonance imaging (MRI) study performed for the evaluation of headache in a pediatric headache clinic and to highlight the clinical dilemma posed by this finding., Methods: A retrospective descriptive study design was used. The electronic database of a tertiary pediatric headache clinic was searched for all newly admitted patients with headache who underwent MRI evaluation in 2008-2013. The indications for imaging were based on clinical practice parameters recommended by the Subcommittee of the American Academy of Neurology. Clinical and imaging parameters were collected from the files. Findings were compared between patients with and without an arachnoid cyst., Results: Of the 250 (31%) of 800 patients who met the inclusion criteria, 11 (4.4%) had an arachnoid cyst. Two patients had a ruptured cyst with midline shifting and a large subdural collection. Both presented with headache, vomiting, phonophobia, and photophobia. In the other 9 asymptomtic patients with an arachnoid cyst, imaging showed only a mild mass effect without midline shifting; their symptoms were considered unrelated to the cyst. The patients with a symptomatic arachnoid cyst were referred for surgery, with good outcome., Conclusions: Arachnoid cysts are found in a small percentage of brain scans performed for evaluation of headache in the setting of a hospital-based pediatric headache clinic. For the long run in these clinical settings, most of the cysts are asymptomatic. Precise anamnesis, neurologic examination, and imaging performed according to accepted practice guidelines may help clinicians determine if the headache and symptoms are caused by the cyst or if they should seek primary headache diagnosis with overlapping symptoms. The clinical distinction between symptomatic and asymptomatic patients (symptoms that are directly related to the arachnoid cyst) may be difficult. Family history of migraine may help in the diagnosis of asymptomatic patients., (© 2014 American Headache Society.)

Background: Both cervical and occipital pain has been reported in pediatric patients with migraine. There are no descriptions of anatomical changes on conventional brain magnetic resonance imaging that can explain the pathophysiology of headache with cervical and occipital pain in this age group. Our aim was to evaluate the frequency of cervical and occipital pain in children and adolescents with migraine as opposed to other types of headache and to seek corresponding anatomic abnormalities on brain magnetic resonance imaging., Methods: The cohort included 194 patients with headache attending the ambulatory headache clinic of a pediatric tertiary medical center. Data were collected by medical file review and revision of conventional magnetic resonance scans., Results: Patients were divided into two groups: migraine headache (n = 125) and other types of headache (n = 69). Occipital pain was reported by 16.4% of the patients and cervical pain by 4.1%; neither type of pain was characteristic of migraine headache in particular. Brain magnetic resonance imaging did not show any anatomic changes specific to migraine or other headache types, regardless of the presence of occipital or cervical pain., Conclusions: Occipital and cervical pain are not characteristic symptoms of any headache group in the pediatric age group, and their presence or absence does not correspond to changes on conventional brain magnetic resonance imaging., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Congenital pancreatic lipase (PNLIP) deficiency is a rare monoenzymatic form of exocrine pancreatic failure characterized by decreased absorption of dietary fat and greasy voluminous stools, but apparent normal development and an overall good state of health. While considered to be an autosomal recessive state affecting a few dozens of individuals world-wide and involving the PNLIP gene, no causative mutations for this phenotype were so far reported. Here, we report the identification of the homozygote missense mutation, Thr221Met [c.662C>T], in two brothers from a consanguineous family of Arab ancestry. The observed genotypes among the family members were concordant with an autosomal recessive mode of inheritance but moreover a clear segregation between the genotype state and the serum PNLIP activity was evident. Based on biophysical computational tools, we suggest the mutation disrupts the protein's stability and impairs its normal function. Although the role of PNLIP is well established, our observations provide genetic evidence that PNLIP mutations are causative for this phenotype.

Background: Recurrent pain symptoms in children are associated with psychiatric comorbidities that could complicate treatment. We investigated the prevalence of psychiatric comorbidity in children with recurrent headache or recurrent abdominal pain and evaluated the screening potential of the Strength and Difficulties Questionnaire compared with the Development and Well-Being Assessment (DAWBA)., Methods: Eighty-three outpatients aged 5-17 years attending a tertiary medical center for a primary diagnosis of migraine (n = 32), tension-type headache (n = 32), or recurrent abdominal pain (n = 19), and 33 healthy matched controls completed the brief self-reporting Strength and Difficulties Questionnaire followed by the Development and Well-Being Assessment. Findings were compared among groups and between instruments., Results: The pain groups were characterized by a significantly higher number of Development and Well-Being Assessment diagnoses (range 0-11) than controls and a significantly greater prevalence (by category) of Development and Well-Being Assessment diagnoses (P < 0.001 for both). Anxiety and depression were the most prevalent Development and Well-Being Assessment diagnoses. Comorbidities were more severe in the headache groups than the controls (P < 0.001). In general, any diagnosis by the Development and Well-Being Assessment was associated with a significantly higher Strength and Difficulties Questionnaire score (P < 0.001). Abnormal scores on the emotional, conduct, and hyperactivity Strength and Difficulties Questionnaire scales were significantly predictive of a Development and Well-Being Assessment diagnosis (P < 0.003)., Conclusion: Children referred to specialized outpatient pediatric units for evaluation of recurrent pain are at high risk of psychopathology. The Strength and Difficulties Questionnaire may serve as a rapid cost-effective tool for initial screening of these patients., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objectives: Studies have reported an association between migraine and white matter hyperintensities on T2-weighted brain magnetic resonance imaging (MRI) in adults. The aim of the present study was to evaluate white matter MRI brain findings in pediatric patients with migraine., Methods: The medical files and imaging scans of all 194 patients who underwent brain MRI at the headache clinic of a tertiary medical center in 2008-2011 were reviewed., Results: Mean age was 10.9 ± 3.5 years. Migraine was diagnosed in 131 patients and other disorders in 63. In the migraine group, findings on physical and laboratory examinations were within normal range. White matter lesions were identified on MRI scan in 14 children with migraine (10.6%) and none of the children with other disorders ( P  = 0.006). In 13/14 patients, the lesions were focal with a variable distribution; in the remaining patient, confluent periventricular hyperintensities were documented., Conclusions: In a headache clinic of a tertiary pediatric medical center, white matter lesions are found in about 10% of pediatric patients with migraine.

Levetiracetam has been authorized for use in Israel as an add-on therapy for intractable epilepsy since May 2006. The aim of the present study was to document its effectiveness for this indication in children, adolescents, and young adults. The medical files of 78 patients aged 0.5-39 years (mean, 14.2 years) treated at our center for intractable epilepsy were reviewed. All received levetiracetam as add-on therapy following a failure to respond to at least 3 anti-epileptic drugs. Fifty-two patients (67%) had partial epilepsy and the remainder had primary generalized epilepsy. The epilepsy was symptomatic in 57%, cryptogenic in 27%, and idiopathic in 15%. Average age at first seizure was 4.1 years. In 45% of patients, the number of seizures was reduced by half with levetiracetam treatment; 11.5% of the cohort achieved complete remission. There was a statistically significant correlation between clinical seizure control and improvement in the electroencephalography findings (p = 0.0012). The drug was well tolerated, with a retention rate of 69% after one year. The most common adverse effects were irritability and impulsiveness, in 26.9% of patients. Severe behavioral side effects (psychosis, confusion) were experienced by 6.4%. In conclusion, levetiracetam is an effective and tolerable add-on agent for use in most epileptic children, adolescents, and young adults who fail to respond to at least 3 antiepileptic drugs and should be the treatment of choice in this setting. Despite the relatively high rate of behavioral side effects in this study, the retention rate at one year was high., (Copyright © 2012 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

This study evaluated the effectiveness of nonpharmacologic measures combined with low-dose propranolol or amitriptyline for treating severe pediatric migraine. Data were collected from the medical files of 118 patients (mean age, 12.54 ± 3.14 years S.D.). All were treated with nonpharmacologic measures. In addition, 93 children received propranolol (mean initial dose, 0.4 ± 0.17 mg/kg/day S.D.), and 25 received amitriptyline (mean initial dose, 0.26 ± 0.1 mg/kg/day S.D.). Twenty patients were switched from propranolol to amitriptyline during treatment. In both groups, headache frequency was reduced by >50% per month in ~80% of patients. Patients without aura responded significantly better to propranolol than patients with aura (P = 0.02). No significant difference was evident in response to pharmacologic treatment by migraine frequency or type (episodic chronic). No significant difference was evident in response to amitriptyline between patients with or without aura. The response rate was higher than previously reported for placebo. Low-dose propranolol and low-dose amitriptyline, when combined with nonpharmacologic measures, are equally effective in reducing the frequency of migraine in children. Propranolol is preferred because of its lower risk of side effects. An additive effect of nonpharmacologic measures may allow for a reduction in drug dose., (Copyright © 2012 Elsevier Inc. All rights reserved.)

The aim of the study was to determine if early steroid treatment of infantile spasms is associated with ocular complications years after its termination. Twenty-five patients with infantile spasms who underwent prolonged treatment with intramuscular synthetic adrenocorticotropic hormone (ACTH) and oral prednisone were evaluated for ocular complications 2 to 33 years after treatment cessation. Patients were followed by an ophthalmic examination that included anterior and posterior segments and measurement of intraocular pressure. Intraocular pressure was normal bilaterally in all patients. Findings on anterior segment examination were unremarkable. On posterior segment examination, 3 patients had an increased cup/disc ratio with normal intraocular pressure. In 2 patients, the increased ratio was considered an anatomical variant. Posterior segment findings in 2 patients were attributed to their background disease. In conclusion, early treatment with high-dose synthetic adrenocorticotropic hormone and oral prednisone for infantile spasm is apparently not associated with a risk of occular complications on long-term follow-up.

This study evaluated possible long-term effects of prolonged high-dose glucocorticosteroid administration in infancy. Thirty patients (16 male, 14 female; age 4.8-33 years) who had completed treatment with adrenocorticotropic hormone (ACTH) followed by glucocorticoids for infantile spasms at a tertiary pediatric hospital at least 2 years previously were invited to undergo quantitative bone ultrasound. The mean speed of soundZ score was -1.085 ± 1.079 for the radius and -0.22 ± 1.19 for the tibia on the nondominant side (P = .0022). The difference from the reference mean (0) was statistically significant for the radius (P < .001). There were no significant differences in radial or tibial mean speed of soundZ scores by age (prepubertal versus pubertal/postpubertal). In conclusion, a high percentage of patients treated with glucocorticoids for infantile spasms have a low radial speed of soundZ score later in life. Long-term follow-up can help to prevent and treat impairments in bone density, especially in non-weight-bearing organs.

The aim of the study was to evaluate changes in electroencephalogram (EEG) recordings during the course of acute lymphoblastic leukemia (ALL) in children. The study group consisted of 48 children with ALL who underwent a total of 72 EEGs at various stages of the disease. The medical files were reviewed for pertinent clinical data, and the EEGs were evaluated for changes in brain activity. Abnormal background activity was noted in 52.2% of the EEGs done at 1-10 days of therapy, in 43.5% of those done at 10-60 days, and only 4.3% of those done at later stages (p=0.037). These findings, together with earlier reports, suggest that early-stage ALL, even before treatment, may be associated with excessive slow EEG activity, which improves over time. The EEG changes, by themselves, are not an indication of central nervous system leukemia or a predictor of later seizures or other central nervous system involvement., (Copyright © 2011 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.)

Nontuberculous mycobacterial infections in immunocompetent children usually presents as chronic lymphadenitis involving the neck and face. Mycobacterium avium complex is the most common pathogen, although recent series found Mycobacterium haemophilum, to be a major cause of chronic lymphadenitis in otherwise healthy children. The authors describe a 9-month-old baby who presented with a 4-month history of preauricular lymphadenitis. Mycobacterial culture yielded Mycobacterium haemophilum. A no-intervention approach was chosen. At the 6-month follow-up, the swelling had ameliorated and the skin showed a small scar with near-normal overlying skin color. A literature review of the clinical manifestations and diagnosis of Mycobacterium haemophilum lymphadenitis and of the different management options for nontuberculous mycobacterial lymphadenitis in otherwise healthy children is presented.

Background: Pathologic breast conditions are rare in childhood and adolescence. The spectrum of breast disease in the pediatric age group is different from that in adults, and most lesions are benign., Objectives: To describe the causes and characteristics of breast asymmetry in adolescents with normal endocrine profiles and sexual development., Methods: The files of patients with a diagnosis of breast asymmetry referred to a tertiary pediatric center from 1990 to 2007 were reviewed for history and findings on physical examination with or without imaging, treatment and outcome., Results: Eleven patients aged 12.5 to 18 years were identified. The cause of the breast asymmetry was traced to unpreventable medical factors in eight patients (physiologic, Poland anomaly, scleroderma), preventable/iatrogenic factors in two patients (chest tissue biopsy, thoracic drain), and possible combined medical-iatrogenic factors in one patient (scoliosis treated with a body brace). All patients were referred for breast reconstruction after full breast development., Conclusions: Severe breast asymmetry in adolescence may be due to congenital factors, diseases involving the breast tissue, or to the effects of medical treatment, and may have severe adverse psychological and social implications. To prevent iatrogenic breast asymmetry, physicians should be made aware of the sensitivity of the breast tissue and should avoid unnecessary tests/procedures that involve the chest wall. In most cases a precise medical history and physical examination can differentiate between physiologic and non-physiologic causes.

Objective: To evaluate the effectiveness of nonpharmacologic treatment for migraine in children younger than age 6 years., Background: The mean age of onset of migraine in children is 7.2 years for boys and 10.9 years for girls. Treatment consists of individually tailored pharmacologic and nonpharmacologic interventions. However, data on migraine management in preschoolers are very sparse., Methods: Demographic, clinical, and outcome data were collected from the files of patients with migraine who attended a pediatric headache clinic. Only those treated by nonpharmacologic measures, namely, good sleep hygiene, diet free of food additives, and limited sun exposure, were included. Clinical factors and response to treatment were compared between children younger than 6 years and older children., Results: Of the 92 children identified, 32 were younger than 6 years and 60 were older. There was no difference between the age groups in most of the demographic and clinical parameters. The younger group was characterized by a significantly lower frequency of migraine attacks and shorter disease duration (in months). Mean age of the patients with no response to treatment (grade 1) was 10.588 +/- 3.254 years; partial response (grade 2), 9.11 +/- 4.6 years; and complete response (grade 3), 8.11 +/- 3.93 years (P = .02). The percentage of patients with complete to partial response as opposed to no response was significantly higher in the younger group (P = .00075)., Conclusion: As the primary option, conservative therapy for migraine appears to be more effective in children younger than 6 years than in older children, perhaps because of their shorter duration of disease until treatment and lower frequency of attacks.

The characteristics of nontuberculous mycobacteria cheek lesions in 7 children were reviewed. The lesions usually presented as nontender erythematous nodules and were associated with a positive purified protein derivate tuberculin skin test. Mycobacterium haemophilum was isolated in 4 cases (57%) and Mycobacterium avium complex in 3 (43%). Cytology and imaging were noncontributory. Resolution was prolonged.

Background: Nontuberculous mycobacteria can cause a chronic localized cervicofacial lymphadenitis in immunocompetent children. The recommended treatment is total excision of the affected lymph node. The aim of this study was to describe our experience with an observational approach., Methods: Children with chronic nontuberculous mycobacterial (NTM) cervical lymphadenitis, whose parents opted for conservative treatment, were followed at our center from 1990 to 2004. The diagnosis of NTM was based on mycobacterial culture of lymph node specimens obtained by fine needle aspiration. The clinical laboratory and follow-up data were documented., Results: Ninety-two children with lymph node positive cultures of nontuberculous mycobacterium were included in the study. Mycobacterium avium complex and Mycobacterium hemophilum were isolated in 90% of the cultures. In most cases, the affected lymph nodes underwent violaceous changes with discharge of purulent material for 3-8 weeks. Total resolution was achieved within 6 months in 71% of patients and within 9-12 months in the remainder. At the 2-year follow-up, a skin-colored, flat scar in the region of the drainage was noted. There were no complications., Conclusions: We suggest that the observational approach can be effective for managing NTM lymphadenitis in immunocompetent children.

The database of a major microbiology laboratory in Israel was searched to determine the prevalence of nontuberculous mycobacterial lymphadenitis in immunocompetent children. We observed a 4-fold increase in nontuberculous mycobacteria isolates during 1985-2006, which was attributable mainly to increased detection of Mycobacterium haemophilum starting in 1996.

This study compared the influence of age and immunization status on symptoms in pediatric patients with pertussis. The files of 60 children, aged 7 to 18 years and 20 infants aged up to 6 months, admitted to our pediatric hospital with a diagnosis of pertussis were reviewed. There were no between-group differences in day and night cough or vomiting. The older group had a longer mean interval to diagnosis than the infants, and the infant group had more symptoms of whooping cough, facial redness during cough, cyanosis, a higher white blood count, a higher percentage of lymphocytes, and more abnormal chest X-rays. Statistically significant differences were found between vaccinated and unvaccinated older children for whooping cough and white blood cell count. Daytime and nighttime coughs are common symptoms of pertussis in all pediatric age groups, regardless of vaccination status. High white blood count is related to immunization status, and percentage of lymphocytes is related to age. In infants, symptoms are more grave but are typical, leading to early diagnosis.

Allodynia is a central sensitization state characterized by a perception of pain to normally nonpainful stimuli. The objective of this study was to determine the frequency of allodynia in children with migraine and to identify clinical features specific to this patient group.

The aim of the study was to assess neurologic complications of pertussis infection. A file review of all children (age 7-18 years) in our hospital with serology-positive pertussis infection admitted from 1995 to 2005 yielded six patients with neurologic symptoms in whom electroencephalographic studies were performed. Data were collected on their clinical symptoms, electroencephalographic findings, final diagnosis, and outcome. The six patients accounted for 10% of all children diagnosed with pertussis during the study period. Their ages ranged from 10 to 15.5 years. All the children were referred by their primary physician because of a suspicion of epilepsy on the basis of parental reports of inefficient attempts to breathe during sleep accompanied by high-pitched sounds and sounds of suffocation, and sleepwalking. The children were amnesic for the episodes. However, findings on electroencephalogram taken during sleep were negative in all cases. The final diagnosis was partial arousal parasomnia. The symptoms of parasomnia disappeared with resolution of the symptoms of the pertussis infection. In conclusion, partial arousal parasomnia may be induced by pertussis infection. Further studies in larger groups are required to confirm this association.

Background: The measurement of cerebrospinal fluid (CSF) pressure is necessary for many clinical indications. Its accuracy may be compromised in frightened or uncooperative children who find it difficult to relax sufficiently. The aim of the present study was to evaluate possible effects of general anesthesia on CSF pressure values., Methods: Lumbar puncture was performed under general anesthesia in 15 patients aged 4.5-20 years for the evaluation of headaches associated with a swollen optic nerve. Cerebrospinal fluid pressure was measured with a manometer when the patient was fully anesthetized (opening pressure) and then continuously recorded until the patient regained consciousness. The opening pressure was compared with the lowest pressure measured at the termination of the procedure (end-measurement pressure)., Results: Seventeen pressure measurements were performed in 15 patients. In all but two measurements, differences were noted between the opening and end pressure, ranging from 5 to 13 cmH(2)O. The opening pressure was abnormally high in 16 measurements, and the end pressure was abnormally high in seven. The difference between the two measurements was highly significant (P < 0.001)., Conclusions: Lumbar puncture performed under general anesthesia may yield two pressure measurements. Many factors, such as hypercarbia and the anesthetic agent used, may influence the results. Owing to the dynamic changes in CSF pressure, measurements made under anesthesia may be unreliable.

This report describes a 10-year-old child in whom the clinical and serologic appearance of pertussis coincided with new-onset migraine without aura. The child's mother, who had a history of migraine, also complained of exacerbation of symptoms and was found to be positive for Bordetella pertussis. The migraine improved in both mother and child concomitant with the clinical improvement of symptoms of the pertussis infection. The possible molecular basis of pertussis as a trigger for migraine is discussed.

Background: An estimated 10% of all children are subject to recurrent attacks of abdominal pain of unknown origin. When no organic cause is found, the working diagnosis is usually functional abdominal pain., Objectives: To investigate the possible causative role of occult constipation., Methods: We defined occult constipation as the absence of complaints of constipation on initial medical history or of symptoms to indicate the presence of constipation. The diagnosis was made by rectal examination and/or plain abdominal X-ray., Results: Occult constipation was found to be the cause of RAP in 42.6% of children examined. Treatment consisted of paraffin oil and phosphate enema. In 82.84% of cases the abdominal pain subsided considerably or disappeared within 2 weeks to 3 months of treatment. On telephone interview of the parents at 1-1.5 years after discharge, 96.5% reported that both the abdominal pain and constipation had subsided or disappeared., Conclusions: Occult constipation can be easily identified and treated in a large number of children with RAP who were diagnosed as having functional abdominal pain.

Introduction: Extraventricular hydrocephalus (EVOH), defined as the enlargement of all CSF compartments in the absence of an obstructing lesion, is usually associated with an increased head circumference and a relatively benign clinical course. Occasionally, because of concern about increased intracranial pressure (ICP), treatment with diuretics is initiated. In most cases, surgical drainage or diversion is not indicated. EVOH may follow a more alarming clinical course and be associated with developmental delay and/or other symptoms of increased ICP., Case Report: We describe a 6-month-old girl with EVOH and developmental delay who was treated with temporary drainage of the subarachnoid space. Clinical response was immediate, with stabilization of the head circumference and improvement in motor performance. We propose that such a surgical procedure might be considered for more frequent use in selected cases.

Study Objective: To apply the Arkansas color method in order to evaluate drug compliance and factors that can predict treatment adherence in patients being treated for latent tuberculosis infection (LTBI) with a single daily dose of isoniazid (INH)., Design: Prospective study of adherence of 105 patients aged 1 to 75 years who were treated with a single daily dose of INH for LTBI., Interventions: Patients or their parents were interviewed regarding parameters that may affect compliance. Urine samples were collected and tested for INH metabolites with the Arkansas color method., Results: Nonadherence to treatment was found in 28.5% of patients. There was no statistically significant correlation among the following parameters: gender; age; diagnosis; mode of administration (self or parents); duration of treatment; dose of INH per weight; or interval since last intake of dose. Twenty-six patients were randomly checked for treatment adherence on two separate visits, and nonadherent patients were informed immediately and their condition was fully explained to them. Five of six patients who were nonadherent in the first visit and were examined twice became adherent in the second visit. Three of 20 patients who were adherent in the first visit became nonadherent., Conclusion: Almost one third of the patients who received LTBI treatment with INH were nonadherent to treatment. No factor was found to predict adherence. The Arkansas method can be used by the family physician and is a simple, immediate method to follow-up patients with LTBI who are treated with INH.

Increased levels of lactic dehydrogenase (LDH) in cerebrospinal fluid (CSF) have been reported in association with several intracranial pathologies. We studied LDH isoenzymes in the CSF of children with infantile spasms. CSF samples collected from 12 patients (aged 4-9 months) with infantile spasms were analyzed for total LDH isoenzymes activity, and were compared to samples from 15 normal children. Mean total LDH activity in the CSF was 34.62 +/- 6.52 U/l. Patients with infantile spasms had a lower LDH-1 percentage and higher LDH-3 percentage; the differences from the control group were statistically significant (p < 0.01). LDH-4 and LDH-5 had similar values in both groups. Infantile spasm is apparently associated with a distinct LDH isoenzyme pattern in the CSF. More studies are needed to confirm the rise in LDH-2, LDH-3 and to determine the optimum time of analysis., (Copyright 2003 S. Karger AG, Basel)

A 16-year-old female presented with unilateral blindness in her right eye 2 months after blunt head trauma. Optic nerve edema was demonstrated by funduscopic examination, ultrasonography, and magnetic resonance imaging and failed to respond to medical treatment. Delayed post-traumatic blindness may be a severe complication of head trauma. The late appearance leads to delayed diagnosis and resulting unresponsiveness to treatment. Patients who experience head trauma that could involve the optic nerves should undergo ultrasonography of the optic nerves. An abnormal finding should be followed by an intensive evaluation to determine possible damage.

Background: In our experience, secondary enuresis nocturna is a common complaint among children after a motor vehicle accident. However, as these children are often brought for examination as part of an insurance compensation claim, this complaint is not always reliable., Objective: To describe a series of children in whom secondary enuresis occurred after a motor vehicle accident., Methods and Results: Five children were brought to our clinic for evaluation of secondary nocturnal enuresis. Review of past history revealed a car accident preceding the onset of the enuresis. All but one had additional behavioral symptoms typical of post-traumatic stress disorder. Four children had evidence of head trauma, and one had psychological but no physical trauma., Conclusions: Nocturnal enuresis can occur after a motor vehicle accident due either to purely psychological trauma or organic head trauma. While nocturnal enuresis is generally attributed to organic causes, psychological mechanisms also play a significant role.

Preliminary results have recently shown that an early switch from parenteral antimicrobials to an oral substitute provides an effective means of treating pneumonia in pediatric patients. In a controlled randomized study, 62 children with community-acquired lobar/segmental pneumonia were selected to receive 8 days of cefixime or amoxicillin-clavulanate after an initial therapy of two doses of parenteral ceftriaxone. Enrollment criteria included: age 6 months to 5 years, fever > 38.5 degrees C, white blood cell (WBC) count > or = 15,000/ mm3, and lobar/segmental pneumonia on chest radiograph. Twenty-nine patients were randomized to receive oral cefixime and 33 to oral amoxicillin-clavulanate. The two groups were comparable in the following pretreatment parameters: age, duration of illness, temperature, mean WBC count, erythrocyte sedimentation rate, C-reactive protein, and need for hospitalization. Days of resolution of high fever, tachypnea, cough, grunting, and laboratory test abnormalities were similar in the two groups. Clinical response at the end of treatment showed cure, improvement, and failure in 97%, 3%, and 0%, respectively, in the cefixime group and in 88%, 6%, and 6%, respectively in the amoxicillin-clavulanate group (P = NS). We conclude that young children with community-acquired lobar/segmental pneumonia can be successfully treated with 2 days of parenteral ceftriaxone followed by 8 days of oral cefixime or amoxicillin-clavulanate.

Toxocariasis in children is usually an asymptomatic infection and those with clinical illness have non-specific systemic or local manifestations. We present a 24-month-old boy with bilateral lymphedema of the feet as the main clinical manifestation of toxocariasis. The child presented with limping and nonpitting edema of both feet. Laboratory investigation revealed leucocytosis of < 20,000/mm3 with a differential count of < 50% eosinophils. No other cause of edema was found. The ELISA for toxocariasis revealed a high titer of > or = 1:4,096. The limping and the lymphedema disappeared during the third week of his illness. We suggest that toxocariasis should be considered as a possible cause of lymphedema and eosinophilia in young children.

We have previously reported on high thyroid-stimulating hormone (TSH) concentrations in clinically euthyroid children with congenital hypothyroidism (CH) undergoing appropriate treatment. Whether this TSH is biologically active or not is still unclear. It has been shown that ectopic thyroid tissue does not involute during thyroxine (T4) therapy and thus can continue to secrete thyroglobulin (Tg). This study was undertaken to determine whether the Tg levels in ectopic CH infants represent residual thyroid tissue stimulated by biologically active TSH and whether this Tg can be used to help monitor CH treatment. Among the 51 primary CH children (age 2-14 years) diagnosed and followed up by us, 28 had measurable Tg values (> 2 pmol/l) several years after the T4 treatment had been started. In 8 of the children, Tg was measured as early as the time of diagnosis and followed up for at least 3 years. The Tg levels decreased much more slowly than the TSH levels did, and secondary Tg rises were observed. By 5 months of age, all children had Tg levels less than 25 pmol/l. Although in some infants the Tg levels paralleled TSH behavior, in others the TSH-Tg correlation was not so obvious. In another group of 8 children who had high TSH values despite normal T4, the LT4 replacement dosage was increased by 60% for 1 week (from 3.5 +/- 0.2 to 5.5 +/- 0.5 micrograms/kg/day) in order to examine the TSH-Tg dependence.(ABSTRACT TRUNCATED AT 250 WORDS)