Mohamed Jiddane

Cerebellar abscesses secondary to occipital dermoid cyst with dermal sinus: Case report

BACKGROUND Hydrocephalus and cerebellar abscesses as the principal manifestations of posterior fossa dermoid cyst are rare. In addition, extradural dermoid cyst of the posterior fossa has been described in only 9 cases in the literature. We present an unusual case of obstructive hydrocephalus due to cerebellar abscesses induced by an adjacent extradural dermoid cyst with complete occipital dermal sinus. CASE DESCRIPTION A 14-month-old child presented with acute raised intracranially pressure, seizures, and meningitis. Neuroradiological studies revealed cerebellar cysts with ring enhancement associated with a contiguous occipital cyst, with compression of the adjacent cisterns and the fourth ventricle causing hydrocephalus. The diagnosis of cerebellar abscesses with congenital occipital defect was briefly entertained. The patient was treated by radical excision of the occipital cyst with hair contents, the dermal sinus, and the abscesses through a suboccipital approach, followed by systemic antibiotic therapy with a good outcome. Pathologic examination revealed a dermoid cyst. CONCLUSION Posterior fossa dermoid cyst should be considered in all children with occipital skin lesions, especially dermal sinus. CT scan and MRI are the methods of choice for further investigation of suspect congenital dermal lesions. Neurosurgical treatment of these malformations should be planned early to prevent the high incidence of infections such as bacterial meningitis and cerebellar abscess. Clinical presentation, diagnostic evaluation, and treatment of these rare lesions are reviewed.

Le syndrome de Sneddon : étude de 15 cas avec artériographie cérébrale

Sneddons syndrome is a rare disease defined by the presence of ischemic cerebrovascular events associated with livedo reticularis. We report a retrospective study of fifteen cases, thirteen women and two men, mean age of 37.93+/-9.77 years. All patients presented one or more cerebral infarcts. Six patients had dementia. Brain magnetic resonance imaging showed several cortical infarcts with white matter involvement. Cerebral angiography performed in all patients, showed a distal arteriopathy in twelve and thrombosis of the right carotid internal artery in one. One patient had antiphospholipid antibodies. Ten patients were treated with antiplatelet agents and five with anticoagulants. The course was favorable in eight patients and stationary in three. Four patients had several recurrent infarcts, one when anticoagulants were discontinued, one taking an anti-sludge-platelet agent and two who were not initially taking any treatment.Resume Le syndrome de Sneddon est une affection rare definie par l’association d’accidents ischemiques cerebraux (AIC) et d’un livedo racemosa. Nous rapportons une serie retrospective de 15 patients, comportant 13 femmes et 2 hommes, âges en moyenne de 37,93 +/- 9,77 ans. Tous les malades ont presente un AIC unique ou multiple. Six patients avaient un syndrome dementiel. L’imagerie par resonance magnetique a note des infarctus corticaux multiples avec une atteinte de la substance blanche. L’angiographie cerebrale faite dans tous les cas a montre des signes d’arteriopathie distale dans 12 cas et une thrombose de la carotide interne droite dans un cas. Des anticorps anti-phospholipides etaient presents a un taux significatif chez un seul patient. Dix patients ont ete traites par des anti-agregants plaquettaires et cinq par des anti-coagulants. L’evolution etait favorable dans 8 cas, stationnaire dans trois cas. Quatre patients ont presente ulterieurement de nombreux AIC, une des l’arret des anticoagulants, une sous anti-agregants plaquettaires et deux n’avaient aucun traitement.

PO23-TH-22 NeuroBehçet's dementia: neuropsychological study of 12 cases

Purpose: Cognitive impairments of Behçet disease have rarely been analysed. We present the neuropsychological study of 12 cases of neuroBehçet with dementia. Methods: Twelve patients with parenchymal neuroBehçet were explored using a battery of neuropsychological tests to assess memory, intellectual, executive, visual-spatial and constructional functions, language, praxis and gnosis. All patients underwent cerebral imaging. Cerebral arteriography was performed in 7 patients. The average follow-up was 76.25 months (range, 24 to 204). Neuropsychological examination was repeated at 3 months, 6 months and one year after diagnosis. All subjects were treated with steroids and intravenous pulse of cyclophosphamide. Results: All patients were men with a mean age of 42.16 years (range, 30 to 58). Dementia was severe in 7 cases and moderate in 5 others. It ocurred after a neurological injury in 9 cases and was inaugural in 3 cases. There were 8 subcortical dementia with disturbances of long term memory (predominant in recall and learning), dysexecutive and visuospatial functions, behavioural change and apathy, two thalamic dementia, one case of severe amnesic syndrome and one other of progressive aphasia. Eight patients improved under treatment. Two patients had severe dementia and two others died. Conclusion: NeuroBehçet’s dementia appears usually after several neurological injuries, it could be reversible if early treatment using steroids and cyclophosphamide is instaured, otherwise it leads to severe and definitive sequelae.