Episodic paroxysmal hemicrania: a further case and review of the literature.

Episodic paroxysmal hemicrania was delineated as a clinical entity only two years ago, separating patients whose attacks remained grouped in bouts lasting weeks, from those who started irregularly and lapsed into chronicity or began and continued in the chronic state. A further case of the episodic variety and a review of the nine previously recorded cases is reported. The division into episodic and chronic variants of paroxysmal hemicrania conforms with the classification of cluster headache. The similarity of the two conditions is emphasised although the response to indomethacin in paroxysmal hemicrania is a special feature.     

Chronic paroxysmal hemicrania. A review based on personal cases

Chronic paroxysmal Hemicrania (CPH) is a headache syndrome first described in 1974. Since then about 60 cases have been reported in the world literature. CPH is characterised by headache attacks occurring daily and always strictly unilaterally. The frequency of the attacks varies from 5 to 30 per 24 hours, the single attack having an usual length of 2 to 30 minutes. The prompt response to indomethacin is the decisive diagnostic criterion of this headache syndrome. The clinical picture, the treatment, some aspects of the pathogenesis and the differential diagnosis of CPH are described by means of case reports. To our knowledge these are the first four cases reported in West Germany.     

Chronic paroxysmal hemicrania and hemicrania continua responding to topiramate: two case reports

Chronic paroxysmal hemicrania (CPH) is a rare primary headache syndrome, which is classified along with cluster headache and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing as a trigeminal autonomic cephalalgia (TACs). Hemicrania continua (HC) was previously classified as one of the TACs, but in the recent second classification of the International Headache Society this disorder was moved to the group of other primary headaches. Both CPH and HC are characterised by moderate to excruciating pain requiring pharmacological treatment; furthermore, both conditions are characterised by an absolute response to indomethacin, which represents one of the current diagnostic criteria for these two syndromes. Unfortunately, in about one-fourth of cases treatment with indomethacin may cause adverse events, mostly gastrointestinal. We report one subject with CPH and another with HC intolerant to indomethacin, who responded remarkably well to topiramate.     

Episodic paroxysmal hemicrania with seasonal variation: case report and the EPH-cluster headache continuum hypothesis.

Episodic paroxysmal hemicrania (EPH) is a rare disorder characterized by frequent, daily attacks of short-lived, unilateral headache with accompanying ipsilateral autonomic features. EPH has attack periods which last weeks to months separated by remission intervals lasting months to years, however, a seasonal variation has never been reported in EPH. We report a new case of EPH with a clear seasonal pattern: a 32-year-old woman with a right-sided headache for 17 years. Pain occurred with a seasonal variation, with bouts lasting one month (usually in the first months of the year) and remission periods lasting around 11 months. During these periods she had headache from three to five times per day, lasting from 15 to 30 minutes, without any particular period preference. There were no precipitating or aggravating factors. Tearing and conjunctival injection accompanied ipsilaterally the pain. Previous treatments provided no pain relief. She completely responded to indomethacin 75 mg daily. After three years, the pain recurred with longer attack duration and was just relieved with prednisone. We also propose a new hypothesis: the EPH-cluster headache continuum.     

Episodic ataxia: a case report and review of literature

This report describes the clinical features of a 29 year female presenting with a 3 years history of episodes of cerebellar ataxia, dysarthria and nystagmus lasting 3-5 days, recurring almost every month. Sleep disturbance and buzzing in ears were noted 3-4 days before each episode. No other precipitant factor was present. Family history was negative. She was diagnosed as a case of episodic ataxia type-2 and was successfully treated with acetazolamide, a carbonic anhydrase inhibitor. She was asymptomatic at 2 year followup.     

Antley-Bixler syndrome Description of two new cases and a review of the literature

Antley-Bixler syndrome was first described in 1975, and to date 20 cases have been reported. In addition to brachycephaly, the syndrome is associated with midface hypoplasia, often with choanal stenosis or atresia, bilateral radiohumeral synostosis, multiple joint contractures, femoral bowing and long bone fractures, ``pear-shaped' nose, dysplastic ears and, occasionally, urogenital or cardiac defects. Survival is closely linked to upper airway obstruction. This, in addition to craniosynostosis, also affects mental prognosis. The cluster of malformations and their severity are variable, and while numerous children have died early from respiratory distress, one third of them are alive and have had quite satisfactory development. With early and effective prevention of respiratory complications and early treatment of craniosynostosis, the overall prognosis can be favorable. The mode of inheritance is probably autosomal recessive, and midtrimester prenatal diagnosis is feasible. Genetic counseling depends on accurate prognostic and therapeutic data. We describe two new cases, a 4-year-old boy with unilateral coronal synostosis and radio-humeral synostosis on the same side and an 18-month-old girl with brachycephaly and imperforate anus. Received: 30 October 1996     

Hemicrania continua and chronic paroxysmal hemicrania: a comparison of pupillometric findings.

Pupillometric studies were carried out in 9 patients with chronic paroxysmal hemicrania (CPH), in 10 patients with hemicrania continua (HC) and in age- and sex-matched controls (n = 12-17). Studies were carried out in the basal condition and after instillation of 2% tyramine, 1% OH-amphetamine, or 1% phenylephrine. The pupillary response to tyramine in HC was more marked than in controls on a percentage increase basis, but not so much when the increment is calculated in absolute values. In this series, there was no asymmetrical response (relative miosis on the symptomatic side) like in previous case reports. The pupil reaction was less in HC than in CPH after hydroxyamphetamine instillation and without any marked asymmetry. There was no definite evidence of supersensitivity to a directly acting sympathicomimetic agent, phenylephrine. No gross abnormalities as regard the sympathetic function in HC was thus observed. The asymmetry on tyramine testing differed in CPH and HC (most marked in HC). No major emphasis should probably be attached to this finding, since on testing with OH-amphetamine, a similar substance, this finding was not reproduced.     

Cerebral blepharoptosis: two new cases, literature review and proposal for diagnostic criteria

Abstract Cerebral ptosis (CP) consists of acute and bilateral drooping eyelids due to a right or left middle cerebral artery territory stroke, without involvement of brain stem, third cranial nerve or oculosympathetic fibres. The pathogenesis of CP is still unknown, but most authors have hypothesised that eyelid control is hemispherically lateralised. Two new cases of CP and a complete literature review are reported in this paper. Because no unanimous criteria for CP diagnosis are currently available, a proposal for new criteria is also included.     

Infant botulism: two recent cases and literature review

Infant botulism is a cause for significant pediatric morbidity in the United States, though early recognition and supportive care can greatly improve clinical outcomes. Since the approval of human botulism immune globulin by the United States Food and Drug Administration (FDA) for the treatment of infant botulism in 2003, the importance of prompt initiation of therapy has been emphasized, with clinical suspicion remaining the mainstay of diagnosis. In this report, 2 cases of infant botulism are described. Each presented to the Pediatric Neurology service at our institution in Upstate New York in the spring and summer months of 2007 and were felt to be related to markedly dusty environmental conditions and the probable ingestion of C. botulinum organisms present in soil. Following this, a comprehensive review of the literature regarding infant botulism in the United States is presented, wherein the pathophysiology, clinical features, epidemiology, and treatment are discussed.     

Meningioangiomatosis: report of two cases and literature review

Meningioangiomatosis (MA) is a rare benign intracraneal lesion. The majority of cases are sporadic although the association of this lesion with familial neurofibromatosis (NF) type 2 is well known. NF-associated MA may be multifocal and is often asymptomatic and diagnosed at autopsy. Non-associated cases are usually symptomatic, occurs in children and young adults and frequently arise in leptomeninges and underlying cerebral cortex. In the present work, we describe two new non-associated cases of MA in two boys, seven and one year old with seizures that disappeared after surgical excision. Histopathologically, the lesion was predominantly cellular in one case and more fibrous in the other. From the literature review we concluded that sporadic cases present as single lesions which manifest by seizures or persistent headaches. Rarely MA has been described to coexist with meningiomas. Histopathologically, MA is characterized by a plaque-like proliferation of meningothelial and fibroblast- like cells surrounding small vessels and trapping islands of gliotic cortical tissue. The lesion does not show significant atypia, mitosis or necrosis. Although all cases of MA share unifying features, there are different degrees of histological presentation with cases predominantly cellular and others more fibrous and calcified. This could correspond to different stages in the evolution of the MA. Symptoms disappear with the complete excision of the lesion.     

Chronic paroxysmal hemicrania and SUNCT syndrome

Neurological Sciences -     

复发性Fisher-Bickerstaff综合征2例临床分析

目的探讨复发性Fisher综合征(FS)叠加Bickerstaff脑干脑炎(BBE)的临床特点和疾病命名。方法分析2例复发性Fisher-Bickerstaff综合征的患者的临床资料,并复习相关文献。结果例1表现为复发性睡眠增多、走路不稳、视物成双,肌电图检查提示下肢神经源性损害;例2表现为复发性视物成双、走路不稳,第2次发病出现四肢无力,查体双下肢远端肌力3级,双侧Babinski征阳性,肌电图提示四肢神经源性损害;2例患者除具有眼肌麻痹和共济失调的症状外,都有中枢和周围神经受损的表现;依据患者的病史、查体、临床检测和疾病经过,诊断为复发性Fisher-Bickerstaff综合征,第2例患者叠加吉兰-巴雷综合征。结论具有周围神经损害的BBE和具有中枢神经损害的FS从临床表型上不能截然分开,Fisher-Bickerstaff综合征的概念更能准确地表述这类患者的表型特点。     

Myasthenia gravis and scleroderma: two cases and a review of the literature

Myasthenia gravis is uncommon in patients with scleroderma, and when diagnosed is usually associated with previous use of d-penicillamine. Clinically, both myasthenia and scleroderma may present with fatigue, weakness and bulbar symptoms, so one of diagnoses may be delayed. We report two new cases and review clinical features of 12 other reported cases of co-existing scleroderma and myasthenia gravis, unrelated to previous d-penicillamine therapy. Co-occurrence of myasthenia and scleroderma was reported almost exclusively (13/14) in women with a mean latency of 7.03 years. Most patients (10/11) had seropositive generalized myasthenia, and there were no cases with exclusively ocular symptoms. Three patients with pre-existing myasthenia were safely treated with d-penicillamine. Myasthenia and scleroderma occur in the context of an underlying autoimmune diathesis, but their co-occurrence could be underreported as the recognition of either disorder may be delayed by overlapping clinical symptoms. Our findings also suggest that d-penicillamine may be cautiously used in selected patients with pre-existing scleroderma and myasthenia, when potential benefits outweigh the risk of possible myasthenia exacerbation.     

Eye movement disorders in vitamin B12 deficiency: two new cases and a review of the literature

Eye movement disorders are rarely reported in vitamin B12 deficiency. We describe two cases with eye movement disorder and vitamin B12 deficiency; one with bilateral internuclear ophthalmoplegia and the other with downbeat nystagmus. Both of the patients received replacement therapy but their eye movement disorders did not respond to treatment. We also review the nine previously reported cases.