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Background There is a growing body of literature documenting local herpes zoster outbreak following procedures. The mechanism underlying these outbreaks remains elusive. We present a case of zoster following onabotulinumtoxinA (BTX) for migraine and a literature review. Methods Chart and literature review. Case A 72-year-old woman with chronic migraine received BTX injections for 3 years without incident. She had a history of thoracic zoster with subsequent post-herpetic neuralgia. In August 2013, 48 hours after receiving BTX injections, she developed a painful rash in the right V1 distribution consistent with herpes zoster ophthalmicus. One week later the rash had resolved without treatment. Literature review We identified 65 (including 2 from Juel-Jenson) cases of zoster reactivation following minor procedures. These cases tend to be in young patients without specific risk factors. Outbreaks characteristically occur at the level of exposure to local trauma. Discussion Our review suggests that local trauma, regardless of the nature of stimuli, may be sufficient for zoster reactivation. We hypothesize that the stressors in these reported cases exert a local epigenetic influence on viral transcription, allowing for viral reactivation. Conclusion Zoster is a potential complication of BTX administration for chronic migraine in adults. Physician awareness can reduce the significant morbidity associated with this disease.

Giant cell arteritis (GCA) is a medium and large-vessel vasculitis, which is an important cause of secondary headache in older adults. While GCA has a classic presentation occurring after the age of 50, atypical presentations (eg, fever of unknown origin, cough, low or normal erythrocyte sedimentation rate) may lead to a delay in diagnosis. The topography of vascular involvement has implications for disease-related complications, which can result in neurologic disease at multiple levels of the nervous system. The most feared complication, vision loss, fortunately becomes uncommon after initiation of corticosteroids. Corticosteroid treatment should not be withheld while waiting the results of a temporal artery biopsy (TAB), which remains the gold standard for GCA diagnosis. Newer diagnostic modalities, including ultrasound, magnetic resonance imaging, and positron emission tomography can play an important role in directing treatment in cases with negative TAB. After successful control of the disorder, patients should be gradually tapered off corticosteroids, with careful monitoring using both clinical and laboratory parameters to assess for relapse. Corticosteroid-related treatment complications are not uncommon in GCA. There is mixed evidence for use of adjunct corticosteroid-sparing agents (eg, methotrexate), although these should be initiated in the setting of corticosteroid-related morbidity and/or cases with frequent relapse.

The neuralgias are characterized by pain in the distribution of a cranial or cervical nerve. While most often brief, severe, and paroxysmal, continuous neuropathic pain may occur. The most commonly encountered entities include trigeminal, postherpetic, glossopharyngeal, and occipital neuralgia. More unusual cranial neuralgias may occur in periorbital (eg, supraorbital neuralgia) and auricular (eg, nervus intermedius neuralgia) distributions. These disorders may be mimicked by structural and inflammatory/infectious neurologic disease, along with other primary headache disorders (eg, primary stabbing headache). The approach to diagnosis and treatment of this group of headache disorders is reviewed.

Medication overuse headache (MOH) is preventable and treatable. However, MOH is a major contributor to headache-related disability, affecting up to 70% of patients in headache specialty practices (1). The man- agement of MOH is complicated as patients often have difficulty stopping the overused medication and relapse rates following successful detoxification are high, con- sistent with the concept of MOH as a bio-behavioral disorder, with a shared neurobiology to addiction (2). Analgesics appear to carry varying risks for MOH among susceptible patients, with the strongest associ- ations reported for opioid- and butalbital-containing analgesics (3,4). Non-steroidal anti-inflammatory drugs (NSAIDs) carry the lowest risk, and may even be protective in the development of chronic daily head- days 1-7)), allowance for symptomatic treatment using a different medication than that previously overused (starting on day 8), and regular clinic follow-up over a 6-month period. The majority of patients were treated as outpatients (77.7%), but three centers used inpatient detoxification. For these three centers, the patients' dis- tance from the hospital and personal preferences were used to determine inpatient versus outpatient treat- ment. The subjects were mostly female, and afflicted with migraine, although 39 subjects (10.3%) had pure tension-type headache. Subjects were excluded if they had previously failed a prior detoxification protocol, were overusing 'pure' opioids, benzodiazepines or bar- biturates, were currently treated with a preventative medication, and if they had significant psychiatric comorbidity. During the 6-month observation period, a larger proportion of subjects undergoing outpatient detoxification dropped out of the study (33.9%) com- pared with those undergoing inpatient detoxification (12.5%). Based on an intention-to-treat analysis, nearly two-thirds of patients were no longer overusing medications at the end of the study protocol and nearly half had reverted to an episodic headache pattern. Further, less than 10% relapsed during months 2 through 6 (i.e. stopped overusing during month 1, but then restarted overusing between months 2 and 6). Among patients who completed the detoxification treatment, inpatient and outpatient treatment strategies had similar efficacy. The most important conclusion from this study is that MOH is a treatable condition across international borders and healthcare settings, with most patients benefiting from a combination of advice, detoxification

Background Isolated neuralgic pain in the deep ear may arise from either nervus intermedius (NIN) or glossopharyngeal (GPN) neuralgias. Current International Headache Society (IHS) International Classification of Headache Disorders, second edition (ICHD-2) criteria for these cranial neuralgias require the presence of a characteristic trigger. Aim The aim of this article is to report cases of triggerless neuralgic otalgia to better understand a subset of patients for whom there may be diagnostic uncertainty. Methods Methods included an observational cohort series and systematic literature review. Results We identified five female patients with a median age at symptom onset of 58 (range: 47 to 73). Our patients generally experienced an excellent clinical response to carbamazepine. Patients were contacted by telephone at a median follow-up duration of seven years (range: four to 32) from symptom onset, at which time carbamazepine-free remissions were reported by five of five (100%) of the patients. A systematic review of the literature on neuralgic otalgia led us to conclude that NIN was most common among young women (age Conclusions We conclude that the presence of a trigger is not fundamental, and may be impractical, to the diagnosis of neuralgic otalgia, but remains important for specificity between NIN and GPN.

Objectives: As residency programs adjust to new duty hour restrictions, the use of cross-coverage systems requiring handoffs will rise. Handoffs are vulnerable to communication failures when unstructured. Accordingly, we implemented a standardized sign-out process on our inpatient neurology services and assessed its effect on completeness and perceived accuracy of handoffs. Methods: Residents spent the first half of their rotations utilizing unstructured sign-out. They transitioned to a structured sign-out system (using the situation-background-assessment-recommendation format) during the second half of their rotations. We analyzed survey responses before and after implementation to evaluate for an effect. Results: Residents utilizing structured sign-out were significantly more likely to share test results with patients/families prior to shift changes ( p = 0.037), update our electronic service list ( p = 0.045), and feel all important data were being transmitted ( p = 0.041). Overall satisfaction (scale 1–10) increased from 6.2 ± 1.6 to 7.4 ± 1.3 ( p = 0.002). Conclusions: Our findings demonstrate that standardized sign-out improves the completeness and perceived accuracy of handoffs. Such improvement has the potential to improve patient safety and quality of care.

J Clin Hypertens (Greenwich). 2012;00:00–00. ©2012 Wiley Periodicals, Inc. The study of mechanistically defined forms of hypertension may provide insight into the relationship between hypertension and stroke. The author retrospectively studied a cohort of 23 individuals with pseudohyperaldosteronism (PHA), a condition associated with pathologic activation of the distal nephron epithelial sodium channel but low renin and aldosterone levels. During a median follow‐up of 11 years (range: 1–30), 4 of 23 (17.4%) patients had a cerebrovascular event recorded. Intracranial hemorrhage was not observed in any patient. Cerebrovascular events tended to occur in older patients, minorities, and patients with a later diagnosis of PHA and additional vascular risk factors. In addition to strict blood pressure control, patients with PHA should have early evaluation and treatment of other vascular risk factors to reduce the risk of stroke.

While the association between migraine and depression has been well established, there is very little information as to whether effective prophylactic treatment of migraine correlates with improvement in comorbid depression.We analyzed initial visit data from migraine headache patients (n = 961) in the Mayo Headache Registry along with follow-up data (n = 389) from a subset of this cohort. Depressive symptoms were measured utilizing the Beck Depression Inventory II (BDI-II).We found a correlation between BDI-II and migraine frequency for 4 weeks and 3 months prior to the initial visit (Spearman correlation coefficient ρ = 0.335 and 0.349, respectively; p value0.0001 for both). There was a similar albeit weaker correlation between BDI-II vs. migraine intensity over the same periods prior to initial consultation (ρ = 0.147 and 0.170, respectively; p value0.0001 for both). However, there was not a significant difference in BDI-II scores for subjects with aura vs. without aura (p value = 0.12). Among those with follow-up, improvement in BDI-II correlated with a reduction in migraine frequency and intensity (p value = 0.016 and 0.089, respectively). Differences in the degree of improvement of BDI-II score in patients treated with amitriptyline vs. topiramate could not be detected (p = 0.36).BDI-II score was associated with migraine measures but not with the presence of aura at time of clinical presentation. Over follow-up time, an improvement in depression score was correlated with a reduction in migraine frequency, but was not significantly associated with medication type.

The 15th Congress of the International Headache Society was held in Berlin from June 23rd to 26th of 2011. Interesting new data from several areas of the basic sciences of headache were presented. This is a review of some of the most exciting platform and poster presentations of the meeting. Research addressing 3 general areas of interest is presented in this review: pathophysiology, pharmacology, and genetics.

Objectives To report the clinical, laboratory, and radiographic features and the response to plasmapheresis in a patient with encephalopathy, opsoclonus, and myoclonus whose cerebrospinal fluid was positive for N-methyl-D-aspartate receptor–IgG. Design Case report. Setting St Marys Hospital, Rochester, Minnesota. Patient A 27-year-old woman with a history of episodic migraine developed subacute progressive myoclonus, opsoclonus, and encephalopathy. Results Magnetic resonance imaging demonstrated nodular leptomeningeal enhancement in the superior cerebellar folia and subsequent T2 hyperintensities in the periventricular regions and amygdala. A positron emission tomographic scan of the head demonstrated predominantly frontotemporoparietal cortical hypometabolism with sparing of the primary sensory and motor cortices. Cerebrospinal fluid examination revealed a lymphocytic pleocytosis, mildly elevated protein level, elevated IgG index, and positive oligoclonal banding. Autoimmune cerebrospinal fluid screening revealed a neural-specific IgG that bound to synapse-rich regions of mouse hippocampus and cerebellar granular layer; the neural-specific IgG was confirmed to be N-methyl-D-aspartate receptor specific. No neoplasm was detected by physical examination or by whole-body computed tomography and positron emission tomography. A 5-day course of high-dose intravenous methylprednisolone sodium succinate yielded limited improvement, and the patient subsequently required intensive care unit admission following a pulseless electrical activity arrest associated with pulmonary embolism. The encephalopathy improved dramatically after plasmapheresis. Conclusions This case highlights opsoclonus and myoclonus as manifestations of autoimmune N-methyl-D-aspartate receptor encephalitis in the setting of a novel appearance on positron emission tomography, and it shows a remarkable clinical response to plasmapheresis.

A 60-year old Muslim man was admitted to the Dhaka Hospital of ICDDR,B with an exacerbation of his chronic obstructive pulmonary disease. Incidental hyperpigmented skin lesions were noticed overlying the dorsum of his ankles, knees, and elbows. Such asymptomatic areas of thickened, lichenified and hyperpigmented skin are called ‘prayer marks’ and are well-imprinted on the knees, ankles, and forehead. These are secondary to prolonged periods of pressure over bony prominences during prayer. The patient's wife stated that the appearance of the elbow marks had coincided with an increase in his breathlessness and subsequent use of his elbows to rise from daily prayers. Prayer marks extending to the elbows could be a sign of worsening chronic disease.

We describe the case of a 59-year-old woman who presented with progressive bilateral vestibidar hypofunction and who was found to have bilateral granulomatous mass lesions of the mesial temporal lobe. Initially, her condition stabilized neurologically with corticosteroids, but a diagnosis of neurosarcoidosis was delayed because of the unusual presentation and persistently normal chest imaging results and serum angiotensin-converting enzyme (ACE) levels. Approximately 1 year after her initial presentation, the patient died of complications of a myocardial infarction and pulmonary embolism. Sarcoidosis should be considered in the differential diagnosis of idiopathic bilateral vestibular hypofunction even if the chest imaging and serum ACE levels are normal, particularly when there is evidence of a multisystem process.

Objective To identify complications of mastocytosis that impact the nervous system across a large cohort. Patients and methods In this retrospective series, we reviewed the electronic medical records of adult patients with a diagnosis of mastocytosis who were referred to a Neurologist at Mayo Clinic in Rochester, MN from January 1, 1999 to December 31, 2008. Results Thirty patients were identified who presented to a Neurologist with symptoms potentially related to the mast cell disease. Twelve of these patients presented with complex spells involving syncope, which frequently preceded a formal diagnosis of mastocytosis. Nine individuals presented with acute back pain which was ultimately deemed symptomatic of vertebral compression fractures. One individual experienced spinal cord compression from a vertebral mast cell infiltrate. Headaches were reported in 78/223 (35%) total patients with mastocytosis. Although details of headaches were insufficiently ascertained to diagnose most, the five individuals in our referral cohort met International Headache Society (IHS) criteria for migraine. Finally, three individuals (1.3%) were identified with multiple sclerosis occurring at variable times after the mast cell diagnosis. Conclusion Symptoms related to mastocytosis may be encountered by neurologists and mimic many common, often idiopathic syndromes including, syncopal spells, back pain, and headache. In our cohort, multiple sclerosis may be over-represented. Mastocytosis should be considered in patients with these presentations, especially when also accompanied by flushing, abdominal cramping or diarrhea.

We thank Dr Chinitz for his comments regarding the management of mesenteric ischemia. Our article highlighted a case of acute mesenteric ischemia, in which an early invasive approach was not pursued, and the patient later required resection of infarcted bowel. In retrospect, we of course agree that the patient should have undergone immediate angiography and infusion with papaverine, per the American Gastroenterological Association guidelines.1 The presence of a normal blood lactate concentration and lack of peritoneal signs on physical examination erroneously biased the management toward a conservative approach with heparin. The case sends a strong message that a high index of suspicion for infarcted bowel is a requisite in the evaluation of patients with suspected bowel ischemia. In our report, we appropriately referenced invasive mesenteric angiography as the “criterion standard” and stated, “It is critical to exclude acute mesenteric ischemia first because it carries considerable mortality, which can be affected by an early invasive approach.”

A 52-year-old woman (gravida 3, para 3, abortus 0) presented to the emergency department with a recent history of intractable nausea, vomiting, and abdominal pain. Three weeks before presentation, she had started taking oral contraceptives (OCPs), which were prescribed to control dysfunctional uterine bleeding. The bleeding was thought to be secondary to a newly discovered uterine fibroid. The patient's daily bleeding had stopped completely 4 days before admission. Her symptoms had begun 1 day before admission, when she woke up feeling nauseated and could not stop vomiting. She thought she had the flu and postponed coming to the hospital. The patient described lower quadrant abdominal pain and several bloody, soft stools the day before admission. She denied any fevers or diarrhea. Her medical history was unremarkable, and she reported only osteoarthritis and a prior tubal ligation. She denied taking any daily medications except for the OCPs, which she had also taken at the age of 19 years with no problems. The patient had a clinically relevant smoking history, having smoked 1 to 2 packs per day since the age of 14 years. She drank wine socially.

1. An orbital bruit represents increased blood flow through the collateral arterial system and intracranial arterial supply. In the correct clinical context, the presence of an orbital bruit should make the examiner suspect either a severe stenosis or occlusion of either the ipsilateral or the contralateral internal carotid artery (ICA). 2. Orbital bruits can represent compensated perfusion to the contralateral hemisphere. This occurs as flow velocity increases through the ipsilateral ICA and through the anterior communicating artery. Thus, blood flow moves from the asymptomatic side to the occluded vascular territory. 3. When listening for an orbital bruit, auscultate by placing the bell of the stethoscope over the patient’s closed eye. In an effort to eliminate the noise of rhythmic eyelid flutter, the patient should then be instructed to open both eyes and gaze at a point across the room. If necessary, the eyelid can be passively shut using the bell of the stethoscope. 4. Although not featured on many popular stethoscopes, a deep and narrow bell (i.e., the Ford-Bowles stethoscope) is ideal for the purpose of ocular and arterial auscultation. Orbital bruits are also found in systemic illness (severe anemia, thyrotoxicosis) and vascular anomaly (carotid-cavernous fistula, arterial vascular malformation). An 83-year-old right-handed man presented with increasing episodes of right limb shaking over a 9-month period. Each episode lasted several …

Von Hippel-Lindau (VHL) disease is caused by germline mutation of the VHL tumour suppressor gene. Patients frequently develop multiple nervous system tumours, denominated haemangioblastomas. Analysis of affected autopsy tissues suggests that tumourigenesis propagates from developmentally arrested, embryonic cells and progresses with consistent architectural, cytological, and molecular sequences similar to haemangioblastic formation and differentiation in the embryo. In this study, we analysed 156 nervous system tumours, 139 of which had been surgically resected from 83 VHL patients. We demonstrate that large tumours consistently contain epithelioid components characteristic of haemangioblastic differentiation in comparison to small tumours that solely display a poorly differentiated, mesenchymal structure. We further show exclusive activation of HIF2alpha in both small mesenchymal tumours and the mesenchymal component of large tumours, whereas activation of HIF1alpha is associated with epithelioid structure. We also show that the MIB1 proliferative index is variably increased in the epithelioid component of large tumours, with extramedullary haematopoiesis foci within the epithelioid component at 100%. These data provide compelling evidence that nervous system tumourigenesis in VHL disease represents a protracted process of haemangioblastic proliferation and differentiation that parallels haemangioblastic formation and differentiation in the embryo.

edited by Jose Biller, 748 pp., Lippincott Williams & Wilkins, 2012, $99.99 Similar to prior editions, this is a paperback organized into 2 major sections, devoted to diagnosis and treatment, respectively. The format of the book makes it cumbersome at times to locate desired information, often making the reader dependent on the index in the back. For example, if you want to read about the diagnosis of peripheral neuropathy, it is not intuitive whether to jump to the chapter on gait disturbances, lower extremity pain, or acute sensory loss in the diagnosis section. It turns out that the entire discussion of peripheral neuropathy diagnosis is in the treatment section.

edited by David Borsook, Arne May, Peter J. Goadsby, and Richard Hargreaves, 384pp., Oxford University Press, 2012, $145. The Migraine Brain is an expertly authored and edited compendium of insights into human migraine pathophysiology. While the focus is on neuroimaging, there is an appropriate homage to other research perspectives from both animal models and genetic studies. Part 1 is a single-chapter historical perspective on migraine from antiquity through selected 20th century observations. Part 2 provides a concise pathophysiologic review of selected clinical observations, including proposed mechanisms of photophobia, allodynia, and the transition from episodic to chronic headache. Part 3 is labeled a clinical perspective, consisting of somewhat disjointed chapters ranging from a review of migraine prophylactic treatments to a review of the literature on iron accumulation in the migraine brain. Highlights of this section include timely clinical perspectives on both neuroimaging and genetic testing. Part 4 constitutes the heart of the text, with individual chapters devoted to reviews of both interictal and ictal imaging findings as organized by technique. Part 5 concludes the book with perspectives on future directions of neuroimaging research.

Objective: To highlight the utility of magnetic resonance gradient echo (MR GRE) in the early diagnosis of primary angiitis of the central nervous system (PACNS) through a case report. Background PACNS is a rare form of vasculitis. The presenting symptoms of PACNS are often nonspecific, including encephalopathy, headaches, stroke, seizures, and cranial nerve palsies. Currently, biopsy is the gold standard for diagnosing PACNS. The sensitivity and specificity of less invasive testing, including conventional MRI, magnetic resonance angiography (MRA), CSF analysis, and four vessel angiography, are not as robust. Design/Methods: Case-report and literature review. Results: : A 52 year old gentleman presented for workup of episodic vertigo, dysphasia, and headaches. MRI brain revealed confluent subcortical T2 signal hyperintensities in the right frontotemporal and left temporal regions, corresponding small punctuate and linear zones of contrast enhancement, and a subacute left cerebellar hemorrhage. His MR GRE imaging revealed subcortical microhemorrhages and a left cerebellar hemorrhage. Serologic workup revealed normal CBC, electrolytes, INR, sedimentation rate, lactate dehydrogenase, angiotensin-converting enzyme level, myeloperoxidase, proteinase, antiphospholipid, and paraneoplastic antibodies. CSF examination revealed prominent lymphocytosis and negative cytology. MRA and conventional angiography were unrevealing. A cortical biopsy revealed chronic perivascular lymphocytic inflammation with focal granulomatous features and negative beta amyloid stain suggestive of PACNS. The patient was treated with a five day course of intravenous methylprednisolone (1 gram daily), followed by an oral prednisone taper. He reported no further episodes of vertigo or semantic paraphasia following initiation of steroids. Conclusions: Our case demonstrates the utility of GRE in the early diagnosis of PACNS. The presence of cortical and/or subcortical signal hypointensities on GRE (consistent with petechial hemorrhages) can help to distinguish PACNS from many of its mimickers. Such an imaging modality may be useful for promptly establishing the diagnosis and treating with immunosuppression, which is needed to avoid significant morbidity and mortality. Disclosure: Dr. Toledano has nothing to disclose. Dr. Smith has nothing to disclose. Dr. Dhamija has nothing to disclose. Dr. Jones has nothing to disclose. Dr. Moseley has nothing to disclose.

A 43-year-old man presented for neurologic evaluation with acute bilateral hearing loss. Two weeks before presentation, he had bilateral eye pain diagnosed as scleritis. He also noted a subacute history of fevers, night …