[Atypical CADASIL phenotypes and pathological findings in two new French families]. / CADASIL: aspects phénotypiques inhabituels et observations anatomo-pathologiques dans deux nouvelles familles françaises.

Atypical phenotypes of CADASIL and corresponding anatomical data in two cases are described in 6 members of 2 new French families. In the first family, 4 cases in the same kindred were probably affected, two of them with a predominant psychiatric presentation, two others with dementia and a pseudo-bulbar syndrome of progressive evolution. No history of migraine or ischemic event were documented in any. In the propositus, the diagnosis was documented by skin biopsy, Notch 3 gene mutation and autopsy after the patient had died when 67 years old, 8 years after onset. Brain examination showed a widespread leukoencephalopathy with subcortical infarcts. Characteristic granular lesions of the small arteries of the brain and other organs were observed. In the second family, two cases are reported. One patient died when 63 years old after a subacute evolution mimicking intracranial hypertension. The anatomical diagnosis was retrospectively proven typical of CADASIL with Notch 3 immunostaining of arterial smooth muscle cells. The other case had a progressive evolution over 20 years of limb paresthesia with a mild spasticity diagnosed as a progressive form of multiple sclerosis. It was followed by a pseudo-bulbar syndrome and a mild subcortical dementia without acute ischemic attack. The diagnosis was confirmed by skin biopsy and mutation of the Notch 3 gene. This report illustrates

[Flutter-opsoclonus: report on three cases (author's transl)]. / Le flutter-opsoclonie. A propos de trois observations.

Three cases of spontaneous saccadic ocular movements are reported, each with one or several electrooculographic recordings. Case 1 is a typical ocular flutter during a myoclonic encephalitis with cerebellar signs. Case 2 is an ocular flutter occurring in the course of an acute inflammatory polyneuropathy with cerebellar signs after cytomegalovirus infection. Case 3 began with permanent dissociated opsoclonus, then conjugated opsoclonus and ended with vertical flutter in a patient suffering from bronchial carcinoma. While some definitions are unclear, clinical, electrooculographic and etiological data support a unicist point of view on flutter-opsoclonus.

[Two cases of FSH secreting adenomas (author's transl)]. / Deux cas d'adénome hypophysaire à FSH.

The authors report two cases of FSH secreting adenomas in men and attempt to draw out some features of these very uncommon pituitary tumors. Absence of gynecomastia is emphasized. Diagnosis proceeds from systematic radio-immunoassay of pituitary hormones. Evidence of FSH secretion results from paradoxical gonadotropin stimulation by TRH and secretory granules seen by electronic microscope. Immunofluorescence studies are still to perform. Dynamic testicular secretion and, in one case, histologic examination were performed.

[Cerebral manifestations of sarcoidosis (author's transl)]. / Sarcoïdose à manifestations cérébrales révélatrices.

Sarcoidosis lesions in the brain are relatively rare and can remain latent, or become evident in various forms: meningitic, encephalitic, neuro-endocrinian, vascular, or tumoral. A case is reported of an Antilles patient aged 35 years, in whom the diagnosis was made by examination of an operation specimen, following the discovery of an apparently isolated intracranial hypertension.