Search

Objective.—To explore in detail the duration of exertional headache attacks and explicitly to identify cases of prolonged exertional headache. Background.—The prevalence of exertional headache in general population samples may exceed 10%. The prevalence and distinguishing clinical characteristics of prolonged exertional headache are not well delineated. Methods.—One author (O.S.) personally interviewed 1838 parishioners (88.6%) of ages 18 to 65 years living in a southern Norwegian commune. Questions relevant to exertional headache were included. Results.—As recently reported elsewhere, exertional headache was present in 12.3% of those questioned. With 1 hour set as a tentative border for short- versus long-duration exertional headache, there were approximately equal numbers with short- and long-lasting attacks; prevalence of short-lasting attacks was 6.3% and long-lasting attack prevalence was 6.0%. In regards to other epidemiologic and clinical features, the long-lasting cases did not seem to deviate from the short-lasting cases; both categories had a moderate female preponderance and similar age of onset. There was a somewhat higher mean number of “migrainelike features” in the long-lasting than in the short-lasting variety of exertional headache. Exertional headache attacks could last up to 24 hours. Conclusions.—There seems to be little reason to subdivide exertional headache into 2 separate varieties according to attack duration. Exertional headache attacks have “migrainelike” features but do not seem to be “form fruste” migraine attacks. The long-lasting variety is not uncommon. [ABSTRACT FROM AUTHOR]

Presents a study to describe a family with some sort of progressive autonomic failure in one generation. Methods; Symptoms, including mydriasis, cardiac arrhythmia, respiratory failure, muscular weakness and hypohidrosis; Results which indicated that the autonomic dysfunction pattern differed somewhat from that of progressive autonomic failure, or Shy-Drager syndrome; Conclusion that the disorder was close to, but not identical to, Emery-Dreifuss muscle dystrophy.

The article focuses on Somatomedin C, that is insulin-like growth factor 1, in adults with Down's syndrome. Reduced body height is a characteristic of persons with Down's syndrome, although human growth hormone (HGH) is within normal limits in this population. The effect of HGH is mediated by somatomedins, also named insulin-like growth factors (IGFs). These are peptide hormones which stimulate anabolism and promote growth. Two types of partly homologous peptides, that is, IGF-1 and IGF-2 have been isolated from human serum. Somatomedin C and IGF-1 are identical.

The article focuses on hormonal and biochemical disturbances in Down's syndrome. Down's syndrome is the most frequent single cause of mental retardation, occurring in about one in every 700 births. The syndrome is associated with mental retardation and a large variety of malformations, and other medical complications evolve over the years. The small stature, small testes and reduced fertility typical of Down's syndrome indicate hormonal disturbances. Hypothyroidism is frequent in these cases and low levels of somatomedin C have been demonstrated as well.

Background and purpose: Studies on the comorbidity of migraine and epilepsy have shown conflicting results. We wanted to explore the epidemiological association between migraine and seizure disorders in a population-based material where case ascertainment was enhanced by individual specialist assessments. Methods: Information concerning migraine and seizure disorders was collected from 1793 participants in an interview-based survey in a circumscribed community. Mixed headache, with features both of migraine without aura and tension-type headache, was excluded from further analyses because of its ambiguous character ( n = 137). Thus, data from 1656 participants were included in the study. Results: The number of subjects with epilepsy was small, and a statistically significant association between migraine and the diagnosis of epilepsy was not found. There was a tendency to more active epilepsy in subjects with migraine (1.0%, 5/524), particularly for migraine with aura (1.8%, 3/168), compared with subjects without migraine (0.5%, 6/1132). Migraine was present in five of 11 subjects with active epilepsy (45%) and in four of 28 (14%) with epilepsy in remission ( P = 0.09). Conclusions: An overall association between migraine and seizure disorders could not be demonstrated, but there was a tendency to more migraine in individuals with active epilepsy. [ABSTRACT FROM AUTHOR]

8: CNS Drugs . 2002;16(6):373-383 Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing (SUNCT) is a syndrome predominant in males, with a mean age of onset around 50 years. The attacks are strictly unilateral, generally with the pain persistently confined to the ocular/periocular area. Most attacks are moderate to severe in intensity and burning, stabbing or electrical in character. The mean duration of paroxysms is 1 minute, with a usual range of 10 to 120 seconds (total range 5 to 250 seconds). Prominent, ipsilateral conjunctival injection and lacrimation regularly accompany the attacks. Nasal stuffiness/rhinorrhoea are frequently noted. In addition, there is subclinical forehead sweating. During attacks, there is increased intraocular pressure on the symptomatic side and swelling of the eyelids. No changes in pupil diameter have been observed. Attacks can be triggered mostly from trigeminally innervated areas, but also from the extratrigeminal territory. There are also spontaneous attacks. An irregular temporal pattern is the rule, with symptomatic periods alternating with remissions in an unpredictable fashion. During active periods, the frequency of attacks may vary from <1 attack/day to>30 attacks/hour. The attacks predominate during the daytime, nocturnal attacks being seldom reported. A SUNCT-like picture has been described in some patients with either intra-axial or extra-axial posterior fossa lesions, mostly vascular disturbances/ malformations. In the vast majority of patients, however, aetiology and pathogenesis are unknown. In SUNCT syndrome, there is a lack of persistent, convincingly beneficial effect of drugs or anaesthetic blockades that are generally effective in cluster headache, chronic paroxysmal hemicrania, trigeminal neuralgia, idiopathic stabbing headache (Äjabs and jolts syndrome'), and other headaches more faintly resembling SUNCT syndrome. Single reports have claimed that carbamazepine,... [ABSTRACT FROM AUTHOR]

Objectives: A family with homocarnosinosis was reported in the literature in 1976. Three affected siblings had spastic paraplegia, retinitis pigmentosa, mental retardation, and cerebrospinal fluid (CSF) homocarnosine concentrations 20 times higher than in controls. Based on the clinical findings and new genetic techniques, we have been able to establish a precise genetic diagnosis., Method: The medical records were re-evaluated, and genetic analyses were performed post-mortem in this original family. SNP array-based whole genome homozygosity mapping and Sanger sequencing of the SPG11 gene were performed. Seven additional Norwegian SPG11 patients and their disease-causing variants and clinical findings were evaluated. Homocarnosine levels in CSF were measured in four of these seven patients., Results: A homozygous pathogenic splice-site variant in the SPG11 gene, c.2316 + 1G>A, was found. The clinical findings in the original family correlate with the heterogeneous SPG11 phenotype. The same variant was found in seven other Norwegian SPG11 patients, unrelated to the original family, either as homozygous or compound heterozygous constellation. Normal homocarnosine levels were found in the CSF of all unrelated SPG11 patients., Conclusions: A re-evaluation of the clinical symptoms and findings in the original family correlates with the SPG11 phenotype. The increased levels of homocarnosine do not seem to be a biomarker for SPG11 in our patients. Homocarnosinosis is still a biochemical aberration with unknown clinical significance., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objectives: To study migraine without aura (MwoA) prevalence in the commune of Vågå, Norway; 1838 (18- to 65-year-old) individuals were included. A special search was made for cervicogenic characteristics in MwoA, as it has been claimed that such characteristics may frequently be present. A comparison with cervicogenic headache (CEH) was made., Methods: The MwoA and tension-type headache (T-TH) diagnosis was based on IHS criteria. CEH diagnosis was based on the principles of The Cervicogenic Headache International Study Group., Results: There were 562 cases of MwoA; prevalence: 31%. There were 425 cases of 'pure' MwoA, i.e. without coexisting T-TH. These 'pure' cases were used for extracting MwoA symptoms. The female/male ratio was 1.69, the corresponding ratio in CEH being 0.71. Typical MwoA symptoms such as nausea/photophobia were most frequently found in migraine. This difference amounted to a factor of > or =2.6. On the other hand, typical CEH traits, like mechanical pain provocation and 'posterior' onset of exacerbations, occurred more frequently in CEH than in MwoA. The difference amounted to a factor of two or more., Conclusions: MwoA and CEH have clearly different characteristics. The differences between MwoA and CEH are staggering. It is unlikely that migraine and CEH are linked in a nosological sense.

Objectives: To describe the prevalence and various clinical characteristics of cervicogenic headache (CEH) in the population at large., Methods: CEH was searched for in Vågå, Norway, where 1838 18 to 65-year-old citizens, i.e. 88.6% of this age group, underwent an interview/clinical examination. The Cervicogenic Headache International Study Group criteria include: (I) unilaterality of head pain, (II) reduction, range of movement, neck, (III/IV) ipsilateral shoulder/arm discomfort, (V/VI) mechanical provocation of similar pain, objectively or subjectively., Results: A prevalence of 4.1% was found. In 41 cases with the highest number of CEH criteria ('core' cases), there was a male preponderance (F/M: 0.71). While cervicogenic traits (mechanical precipitation etc.) were frequently present in CEH, 'migraine traits', like nausea, vomiting, and throbbing seemed to be rarely present. In 97% of the cases, pain exacerbations began in the neck/occipital region., Conclusions: CEH may be one of the three large, recurrent headaches. In this series, there was no female preponderance. Nuchal onset of pain is a characteristic trait.

Objectives: To evaluate the postoperative fate of chronic, hard-to-treat and partly suicidal cervicogenic headache (CEH) patients (n = 32), diagnosed according to the CHISG criteria and treated with a decompression/stabilization operation in the cervical spine: the Smith/Robinson operation., Methods: The cervical levels of affection, singled out by magnetic resonance, anaesthetic blockades and X-ray examinations were mainly at the C4-5, C5-6, C6-7 levels; one or two discs were removed. The study was prospective and controlled., Results: During the 1- to 3-month-long postoperative period of collar-wearing, there generally was pain freedom. The mean time of follow-up was 19.8 months: pain recurrence, known to the authors, appeared after 1-58 months (n = 12). The mean time of improvement was: 14.8 months (range 1-58 months). Five patients stayed well > or =3 years. This is certainly a minimum figure. The patients ultimately were lost to follow-up., Conclusions: For the time being, this operation should preferably be used in selected, chronic, severely afflicted, preferably elderly CEH patients, when other therapeutic approaches are exhausted.

Background: During the Vågå headache epidemiology study, there were indications that various types of work, such as carpentry and hairdressing, frequently seemed to be linked to a combination of neckache and headache., Objectives: A post hoc study was conducted among 1838, 18- to 65-year-old Vågå citizens, looking for patients with combined neck/headache., Design: Face-to-face interview was conducted., Results: Combined neckache and headache were detected in 121 (6.6%) individuals. The bilateral headache originated in the neck; it was mild/moderate, symptom-poor, and frequently provoked by awkward neck positions. No such headache occurred without a neckache. Headache in this group of patients resembles tractor drivers' headache, except for the provoking factor itself., Conclusions: This headache is not listed in headache classification systems/textbooks on headache. This headache must be distinguished from the unilateral cervicogenic headache proper because of the different treatment perspectives.

Objectives: To describe a unilateral headache that in addition to the typical shortlasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating and rhinorrhea (SUNCT) syndrome - traits with excessive and ipsilateral autonomic phenomena - had circumscribed eyelid erythema and adjacent ocular redness., Observations: A 60-year-old female had excessive, right-sided lacrimation and local pain at 15 years of age. Due to steadily increasing discomfort, with lacrimation and swelling over the outer part of the upper eyelid, the right lacrimal gland was removed at 20 years of age, with a suspicion of lacrimal gland adenoma. Preoperatively, symptomatic side mild-degree eyelid erythema/rhinorrhea were integral parts of the attack. After years with minor complaints, she, in the mid-twenties, experienced more long-lasting pain attacks, and pain soon became the main problem. A marked, distinct erythema on the lateral part of the right-sided eyelids and marked, localized 'eye redness' in the adjacent area were main ingredients of the attacks together with eyelid edema and viscous rhinorrhea. There were visible vessels below the eye, and telangiectasia of the upper eyelid., Conclusions: This headache has many similarities with SUNCT - but has several, grossly deviating traits: the temporal aspects, excruciatingly intense pain attacks, and above all marked, lateral eye-lid erythema, and adjacent, massive ocular reddening. This constellation probably alienates it from SUNCT.

Objectives: To describe clinical characteristics of cephalic jabs., Material and Methods: In a population study in Vågå, Norway, 1838 18-65-year-old-parishioners (88.6% of the target group) were subjected to a semi-structured interview, based on a headache questionnaire., Results: Of 627 cases of jabs, 68% had single jabs; 4% volleys, and 28% a mixture of volleys and singlets. Most individuals had experienced only few jabs. Exceptionally, there were multiple attacks per day, even per hour. The ratio between an anterior and posterior location was 2.6. Neck movements and Valsalva manoeuvres seemingly occasionally precipitated attacks. Attacks were generally of mild/moderate intensity. Unilaterality prevailed over bilaterality; but unilateral pain might shift side., Conclusion: Cephalic jabs are generally solitary paroxysms, with rather long intervals between attacks. Jabs do not only occur in the trigeminal area. Occasionally vocalization and more frequently jolts accompany the paroxysm.

Introduction: Arthrogryposis multiplex congenita (AMC) may be associated with multiple developmental defects. In some severely affected newborns with AMC, autopsy studies have suggested a common mechanism of malmigration at the spinal and cerebral levels. To our knowledge, a constellation of arthrogryposis, epileptic seizures, and brain migrational anomalies in adult patients has not previously been described in a clinical material., Material and Methods: Six consecutive adult patients with arthrogryposis multiplex congenita and epileptic seizures form the basis of the present study. Five patients had joint contractures and reduced muscle volume restricted to the lower extremities, whereas one patient had predominantly upper extremity affection. They were studied with magnetic resonance imaging (MRI), EEG, EMG, a neuropsychological test battery, and chromosome analysis., Results: Four of them had clear evidence of migrational brain disorders, demonstrated by MRI, in three of them roughly corresponding to the focal epileptiform EEG activity. Five of the patients had partial seizures, whereas one only had generalized tonic-clonic seizures. The MRI findings included polymicrogyria, pachygyria, and fused schizencephaly. Four had neurogenic EMG changes, one had myopathic EMG features, and one had an unremarkable EMG pattern in affected muscles. All patients with demonstrable migrational disorders showed abnormal neuropsychological features. Three patients were mentally retarded. A chromosome abnormality in the form of a ring chromosome 18 was present in one patient., Conclusion: We suggest that AMC, epileptic seizures, and migrational brain disorders may form the integral parts of a hitherto undescribed syndrome in adults. A wide-spread defect in neuronal migration along the entire neural axis may be the underlying mechanism of the cerebral and the peripheral symptoms.

The symptoms of the hyperventilation syndrome (HVS) are sometimes diffuse, and HVS may resemble other clinical conditions. A diagnosis of HVS was made in 25 patients referred for neurological assessment because of atypical, shortlasting, episodic complaints. The referral diagnoses varied within a wide range. A need for more indepth knowledge about this group of patients thus arose. During a provocation test with forced respiration, all patients reported symptoms similar to those during attacks. Eight patients described numbness or paraesthesias with unilateral predominance. End-tidal pCO2 levels were monitored prior to and during a forced hyperventilation test. Compared with controls, the patients had significantly decreased end-tidal pCO2 even during symptom free periods. After hyperventilation, hypocapnia followed a protracted course in the patient group. Sensory symptoms may be asymmetric and mimic focal cerebral disease. Strained respiration may be denied during spontaneous attacks. Personality characteristics were evaluated with the Minnesota Multiphasic Personality Inventory (MMPI). A mean group profile very similar to profiles reported on "pseudoepileptic" patients was found. The profile indicates a neurotic pattern where patients tend to respond to psychological stress with somatic symptoms.

Forty-four patients with myotonic dystrophy were subjected to various tests for intestinal absorption. A varying number of patients were subjected to the various tests. In one patient (of altogether 11 patients) a pathological vitamin A absorption test was found together with increased faecal excretion of fat and nitrogen. Two of 12 patients showed pathological D-xylose tests. The reason for this may possibly be deficient urine collection. Schilling tests were on the whole normal. Glucose tolerance tests were pathological in 26 of 35 patients. The following pathological findings were made: elevated fasting blood sugar, elevated peak level, delayed return to pretest level, biphasic response and reduced increment in blood glucose levels following loading. The biphasic response seems to be a rather characteristic finding in myotonic dystrophy. The peak concentration was in all except one case reached within 15-60 minutes. Intravenous glucose loading gave normal response curves. The pathological response on oral loading was probably often caused by intestinal motility disturbances and not by malabsorption. Malabsorption seems to be a rare feature of myotonic dystrophy.

Myotonic dystrophy seems to have a hypometabolism of non-thyroid origin. The hypometabolism could hypothetically be caused by : 1) reduction in muscle mass, 2) hydrocephalus ex vacuo, an integral part of the disorder, or 3) the basic disease process itself. Possibility 2) was explored by determining basal metabolic rate (BMR) in 41 patients with dilatation. A BMR of less than -10 per cent was found in eight cases. The ventricles were categorized in three groups according to width. No decrease in mean BMR was found in cases with a marked ventricular enlargement. Thus, ventricular dilatation per se does not seem to lead to hypometabolism.

Pneumoencephalography was carried out in 23 patients with various muscular disorders, i.e. spinal neurogenic atrophy, classified as Wohlfart-Kugelberg-Welander's and Charcot-Marie-Tooth's diseases, and muscular dystrophy. EEG-registrations and psychological testing were carried out. Pneumoencephalography revealed ventricular enlargement and cortical changes in 17 out of 23 cases. Changes were found in all three groups studied; cortical changes were, however, only found in the neurogenic atrophy groups. Cases where there clearly might be exogenous causes for ventricular dilatation were excluded. The changes found, therefore, supposedly form an integral part of the disease process. It should be emphasized that the precaution does not entirely exclude a traumatic etiology in some cases. EEG-registrations (pathological in three out of 20 cases) as well as psychological evaluation (pathological in six out of 14 cases) supported the assumption of organic brain changes. Pneumoencephalography, however, seemed to be the most sensitive parameter for unveiling brain involvement in these disorders.

The dispute about whether migraine and cluster headache are one disorder--the "unified theory"--or two facets of a spectrum of "vascular headache" has not yet been settled. The author discusses various clinical features that unite or divide migraine and cluster headache in this respect: so-called "mixed forms" of vascular headache, corneal indentation pulse amplitudes, partial Horner's syndrome and possible aberrations in histamine metabolism. Evidence is presented showing that there may exist subunits of cluster headache, such as chronic paroxysmal hemicrania (C.P.H.) and a hitherto unreported type that co-exists with recurring bouts of retrobulbar neuritis and a partial factor XII deficiency.

Available evidence indicates that serotonin located within platelets--or lack of it--does not precipitate migraine attacks, and that intravenously administered serotonin is beneficial in migraine. On this premise, it is not likely that the beneficial effect of intravenously administered serotonin is due to replacement of lost intracellular serotonin. If serotonin is effective in relieving migraine pain, this is probably due to extracellular serotonin acting on the cardiovascular system. In other words, serotonin-induced relief in migraine is probably caused by the pharmacological properties of the amine--it probably acts as a drug and not by replacement. The serotonin changes in migraine are probably not primary, but caused by the disease process. Platelets may nevertheless be of importance in the pathogenesis of migraine, and serotonin may be of even more interest. However, interest in platelet serotonin will probably be diminishing in the future.

A double-blind, cross-over study with clonidine (Catapresan) and placebo was carried out in 29 patients with migraine, who had received clonidine during a 4 to 32 months pre-test period (mean 10 months) and who appeared to have benefited from the treatment. Clonidine and placebo were given for 7 weeks each, but only the last 5 weeks in each period were used for the statistical evaluation. In the entire material, clonidine was significantly superior to placebo. In nine of the patients, however, various other factors might have influenced the attack frequency and severity. If these nine patients were excluded, no statistically significant difference between clonidine and placebo was found.

Eleven patients who had sustained whiplash injuries to the neck were studied by pneumo-encephalography, by a variety of psychological tests, and by psychiatric evaluation with a view to assessing whether organic CNS changes might, in some cases, be a consequence of such injuries. Patients with traumatic encephalopathy, and age- and sex-matched groups of patients served as control groups. Pneumoencephalography showed normal findings in the whiplash group as a whole, the width of the ventricular system being significantly smaller than that of patients with traumatic encephalopathy. Psychological tests and psychiatric evaluation indicated that organic brain pathology was present in the whiplash patients, although to a lesser extent than in patients with traumatic encephalopathy.

The catabolism of orally and subcutaneously administered 14C histamine was studied in myotonic dystrophy patients. No definite abnormalities were observed as for subcutaneously administered histamine. The catabolism of orally administered histamine followed two distinct patterns: In patients with a relatively low endogenous urinary conjugated histamine excretion at the time of testing, small quantities of radioactivity were excreted in the stools (mean 0.25 per cent), whereas the quantity of exhaled 14CO2 was in the control range (mean 14.4 per cent), and the exhalation curve diphasic as in controls. In patients with a relatively high urinary conjugated histamine excretion, the catabolic pattern differed: There was a relatively high faecal excretion of radioactivity (mean 20.1 per cent), whereas the exhalation of 14CO2 was markedly reduced (i.e. mean 0.5 per cent, versus a mean control level of 8.9 per cent). Diphasicity of the exhalation curve was not present in these patients. It has previously been demonstrated that a patient with myotonic dystrophy may shift between being a normal and a high conjugated histamine excretor. It is thus possible that all patients with myotonic dystrophy may pass through phases with abnormal catabolism of intestinal histamine.

A headache disorder with shortlasting, frequently occurring (6-18/24 hours) head pain attacks is reported. The pain is excruciatingly severe, unilateral (always on the same side), unaccompanied by visual phenomena, nausea/vomiting, but accompanied by nasal congestion and lacrimation on the symptomatic side. The maximum pain is felt in the temporal region, although during severe attacks the entire hemicranium is involved through the neck, shoulder and homolateral arm in a diffuse way. The attack pattern differs clearly from that of cluster headache both with regard to atrack frequency and the long term temporal pattern. In addition to blood and urine parameters and supplementary neurological/neuroradiological investigations, the following parameters were studied: Urinary histamine excretion (partly increased), kinin parameters (occasionally increased blood kinin and reduced blood kininogen), and corneal indentation pulse amplitudes (attack-induced increase, as in regular cluster headache). The following parameters rendered normal results: prostaglandins, cerebral blood flow, fluorescein appearance time, intrathecal pressure during and between attacks, and muscle biopsy with immunological investigation. The pain attacks can be abolished by continuous indomethacin medication. In spite of the ocular findings it has in common with cluster headache, this headache seems to differ from cluster headache.

Homocarnosine, the brain-specific dipeptide of gamma-aminobutyric acid (GABA) and histidine, was found to be elevated in the CSF, i.e. approximately 20 times the mean control level, in two brothers and one sister. All three were similarly afflicted, i.e. with a progressive spastic paraplegia, progressive mental deterioration and retinal pigmentation. A sister was healthy, and there was no other occurrence of similar symptoms in the family. The clinical symptoms in the affected individuals seem to differ from those in other reported families. The unaffected sister, the father and two maternal aunts exhibited a normal CSF homocarnosine level, whereas the mother, who showed no definite clinical symptoms, showed a markedly elevated CSF homocarnosine level. The explanation for the latter finding remains obscure.

Oral glucose tolerance tests have been carried out on 40 patients with myotonic dystrophy, and 50 hospitalized control patients. A biphasic response was found in 19 myotonic dystrophy patients (49 per cent), compared with 12 of the control population (24 per cent). In 9 patients with myotonic dystrophy (23 per cent), the height of the second peak was greater than or equal to 20 mg/100 ml, the corresponding figure for the control group being 2 patients (4 per cent) (P less than 0.005).