[Klippel-Trenaunay syndrome and ischemic neurologic complications]. / Syndrome de Klippel-Trenaunay et complications neurologiques ischémiques.

In 1900 Klippel and Trenaunay defined a new entity characterized by cutaneous angioma associated with varices and hypertrophy of bone and soft tissues. The syndrome is present in the earliest years and becomes accentuated as the child grows. It typically affects one half of the body. We report two cases of Klippel-Trenaunay syndrome. The first patient presented with flaccid paraplegia. On T1-weighted MRI sequences a diffuse heterogeneous high-intensity signal was visible opposite the T8 and T9 vertebral bodies. Spinal cord angiography showed occlusion of the anterior spinal artery issued from Adamkiewicz's artery, suggesting thrombosis. Serum fibrinopeptide A level was very high and compatible with hypercoagulability. The second patient presented with left hemiplegia caused by a right superficial sylvian artery infarct, and carotid angiography showed an image of right internal carotid artery dissection. These two cases suggest that Klippel-Trenaunay syndrome includes a state of hypercoagulability facilitating the occurrence of arterial thrombotic accidents, and an abnormality of the arterial wall capable of determining spontaneous dissection. A study of arterial wall-dependent coagulation factors should establish a link between these two aetiological factors. All this argues in favor of a diffuse vascular pathology, which makes this syndrome close to phakomatoses.

[Bilateral cavernous sinus syndrome: Burkitt's lymphoma]. / Syndrome du sinus caverneux bilatéral: lymphome de Burkitt.

Bilateral cavernous sinus syndrome is usually due to a vascular disease, such as thrombophlebitis or arteriovenous malformation. In a 29-year old woman this syndrome revealed a malignant non-Hodgkin's lymphoma of the Burkitt type. In this case, the physiopathological mechanism was metastatic extension to the dura mater of a systemic lymphoma. The contribution of MRI to the diagnosis is emphasized.

[Protracted recurrent neuroleptic malignant syndrome]. / Syndrome malin des neuroleptiques prolongé et à rechutes.

The natural course of untreated neuroleptic malignant syndrome (NMS) does not usually exceed 3 weeks. Its duration is reduced by dantrolene and/or bromocriptine. We report a patient in whom NMS developed after an injection of haloperidol decanoate and fluctuated during 3 months. This was due perhaps because the initial treatment with oral neuroleptic was pursued, or because the doses of dantrolene were insufficient.

[Memory disorders in chickenpox encephalitis]. / Troubles de la mémoire dans une encéphalite de la varicelle.

A 31-year old man with chicken-pox encephalitis presented with a cerebellar syndrome and disorders of memory. The latter consisted of severe anterograde amnesia with normal retrograde memory, without confabulation or anosognosia. This suggested that the hippocampus was involved, probably functionally, since the outcome was rapidly favourable and the MRI was normal.

[Benign, monomelic juvenile amyotrophy of a hand (Hirayama type): a new case report]. / Amyotrophie juvénile, monomélique, bénigne d'une main (de type Hirayama): 1 nouvelle observation.

Benign, monomelic juvenile amyotrophy of the hand (Hirayama type): new data.--Within the space of a few months a 21-year-old man developed isolated amyotrophy and paresis of the right hand; thirteen years later the clinical picture was unchanged. Medullo-cervical MRI was normal. The EMG confirmed the involvement of the anterior horn. Unilateral chronic distal juvenile amyotrophy located in an upper limb (Hirayama type) was diagnosed on clinical findings. But the spread of electrical abnormalities to the lower limbs suggested a transitional form between this syndrome and other disorders of the anterior horn.