[A case of acute sensory and autonomic neuropathy with regression].

The authors report the case of a 24 year old man with no previous disease who presented with a severe autonomic neuropathy. This included major gastrointestinal dysfunction characterised by decreased peristalsis without distension and paralysis of the gall bladder, and orthostatic hypotension with a normal cardiac tachycardia reflex. There was an associated sensory neuropathy affecting heat sensitivity without motor dysfunction and an increased CSF protein content.

[Cerebral cavernoma: a rare vascular malformation].

The authors have observed 7 cases of intracranial cavernous haemangioma. A review of the literature shows that the diagnosis is suggested by the occurrence of epileptic seizures, signs of expansive process or meningeal haemorrhage. Computerized tomography displays an area of hyperdensity enhanced by injections of a contrast medium.

[Oculomotor forms of myasthenia].

The diagnostic difficulty in purely oculo-motor forms of myasthenia can be due to the lack of fluctuation in symptoms, the occurrence of complete spontaneous remissions, lasting several months or years, the absence of a decremental response in muscle potentials as evidenced by classical repetitive stimulation of the motor nerve, and negative responses to pharmacological testing. Thus, several investigative procedures have been developed to aid in the diagnosis of questionable cases. These include electromyography of proximal muscles and single fiber recordings, the dosage of acetylcholine receptors antibodies (specificity of nearly 100 %), and histological examination of neuro-muscular junction (invariably finding specific lesions of motor end plates).

[Association of ankylosing spondylarthritis, cauda equina syndrome and dilatation of the lumbar cul-de-sac with voluminous arachnoid diverticulum. Clinical, radiological and tomodensitometric study of a case].

The authors report a clinical and radiological observation of cauda equina syndrome associated with ankylosing spondylitis. Although only 32 cases have been reported in the literature over the last twenty years, such an association is probably not exceptional. The mode of onset and the course of the neurological symptoms is quite similar from case to case: ankylosing spondylitis preexisting for many years, and inactive when neurological signs develop, the latter usually being very slowly progressive.

[Incomplete regressive obstruction of the internal carotid artery in a female patient with fibromuscular dysplasia].

The authors report on the case of a 48-year-old woman, with no history of cardiovascular disease, presenting with a progressive right cerebral deficiency syndrome predominating in the parietal region. X-ray, arteriography and CT scan findings led to the diagnosis of right middle cerebral artery ischemic stroke, in the proximal territory, due to a practically complete occlusion of the right internal carotid artery. The patient recovered and a right carotid arteriography performed 8 months after the initial one showed repermeation of the carotid artery, as well as evidence of fibromuscular dysplasia (FMD).

[Occlusive arteriopathy of the circle of Willis with outlined "Moya-Moya" network in a woman with migraine under oral contraception. Regressive course].

A 19-year-old woman with migraine under contraceptive therapy had transient right hemiparesis due to a minor lesion in the left hemisphere, which was probably hemorrhagic rather than ischemic. On the left side, angiography showed nearly complete obstruction of the terminal portion of the internal carotid artery, extending to the initial portion of its terminal branches, with an outlined "Moya-Moya" network. On the right side, moderate annular stenosis of the cervical portion of the internal carotid was visible.

[Involvement of the peripheral and pyramidal nervous system in Crohn disease. Determining role of folic acid deficiency].

We report on the case of a 16-year-old male patient who presented with a peripheral neuropathy remarkable by the severity of pain, the proximal involvement and the association with an underlying myelopathy. All these symptoms coincided with an acute exacerbation of Crohn disease (regional enteritis) involving mainly the duodenojejunal segment, and were ascribed to a major folic acid deficiency, with total recovery following supplementation. In this connection we recall the various neurological symptoms induced by folic acid deficiency.

[Chronic hepatic encephalopathies. Acquired cerebral degeneration not due to Wilson's disease].

We report on 4 cases of perennial hepatic encephalopathy and review similar published cases. The neurological picture consists of a cerebellar syndrome, both static and kinetic, dysarthria, choreo-athetoid abnormal movements and mental deterioration. Symptoms are permanent and usually worsen progressively.

[Wallenberg's syndrome due to a dissecting aneurysm of the vertebral artery].

A 54 year old man without pathologic past history but mild hypertension, obesity and gastric ulcer, presented with a syndrome of Wallenberg. He had complained for five days of progressive and diffuse headache. The neurological condition improved initially, but the patient died suddenly two weeks later.

[Polyneuropathy complicating Horton's disease (author's transl)].

A patient diagnosed as having Horton's disease presented a complex neurological picture dominated by sensory-motor neuropathies of all four limbs, one year after the appearance of signs of temporal arteritis. No other etiological factor, apart from the Horton's disease, was discovered, and the causal relationship between this disease and the neuropathy is discussed. The possibility of dysimmunity factors being involved in Horton's disease is raised and the resulting therapeutic implications discussed.

[Unilateral dysplasia of the circle of Willis with multiple distal stenoses (author's transl)].

Neurological and clinical examinations were found to be normal in a woman aged 40 years who had had a single generalized epileptic seizure. Rediological examinations demonstrated a spiral-shaped calcification above and laterally to the left sella turcica. This corresponded to changes in the C 1 segment of the left carotid sinus, which was partly stenosed distally, with left unilateral abnormalities of the circle of Willis and multiple distal stenoses of the left sylvian and vertebro-basilar arteries.

[Plexus lesions following radiation therapy. Report of nineteen cases (author's transl)].

Nineteen patients with plexus lesions following radiation therapy were investigated: fifteen with brachial plexus, 4 with lumbar or sacral plexus involvement. Symptoms at onset are usually sensory. Motor disturbances occur either simultaneously or after some delay, their course is generally gradual and unfavourable.

[Cisterno-medullary anomalies. Diagnostic problems, surgical treatment (author's transl)].

Under the term cisterno-medullary anomalies are included several disorders: bony and nervous malformations, arachnoiditis of the posterior fossa. As they are frequently associated, a thorough investigation, both anatomical and dynamic, is a prerequisite to any therapeutic attempt. Along with causing damage to the neuraxis, these anomalies interfere with the dynamics of the CSF and may lead to the development of a communicating syringomyelia, whatever the theory proposed.

[Long term administration of oral prednisone or prednisolone in treatment of myasthenia gravis. Report of five cases (author's transl)].

Five patients, four of them with severe or severe generalized myasthenia gravis, were treated by long term orally administrated prednisolone, with following results: one complete remission and two almost complete remissions (in three aged fernale patients), two substantial improvements (one in a male patient, one in a young adult female patient, both thymectomized). The least favourable result was observed in the male patient. Positive results of such treatment were similarly reported by several authors (with an average of 70% complete or almost complete remission, 20% substantial improvement, 7% moderate improvement).

[Tolerance of ligation and of aseptic thrombosis of deep cerebral veins in man. Apropos of 4 cases].

Classically, ligation of the deep cerebral veins is incompatible with survival in man. The authors report four cases, three surgical and one medical, of thrombosis of these veins, confirmed by angiography and well tolerated. This tolerance may be explained by anatomical study of collateral systems and corroborated by clinical and experimental findings.