[HIV infection presenting with bilateral optic neuropathy]. / Neuropathie optique bilatérale révélant une infection par le virus de l'immunodéficience humaine.

We report the case of a 57-year-old man who presented bilateral subacute and painless optic neuropathy after meningopolyradiculitis revealing a primary human immunodeficiency virus infection. Both antiretroviral and steroid treatments were ineffective. Clinical symptoms and evolutive pattern were consistent with a mechanism of microvascular ischaemia of the optic nerve head. Optic neuropathies related to HIV infection are rare compared to those resulting from opportunistic infections. There are several pathophysiological mechanisms involved.

[Myoclonic encephalopathy associated with proton pump inhibitors]. / Encéphalopathie myoclonique: rôle des inhibiteurs de la pompe à protons.

Two men (66 and 73 Years) with a cardiovascular history were hospitalized for rapid onset encephalopathy associated with myoclonia and an extrapyramidal syndrome. On the basis of the French Pharmacovigilance system, this symptomatology has been attributed to the coadministration of a proton pump inhibitor, lansoprazole (15mg/day) with levodopa. Lansoprazole withdrawal led to a normalisation of the situation.

[Vertebro-basilar ischemic strokes and aseptic meningitis, late complications of Fabry's disease]. / Accidents ischémiques vertébrobasilaires et méningite aseptique compliquant une maladie de Fabry.

Fabry's disease, also called angiokeratoma corporis diffusum universal, is a rare cause of stroke in the young. We report the case of a 39-year-old man who presented with relapsing vertebro-basilar ischemic strokes. Biological tests showed the presence of an aseptic meningitis and a biological inflammatory syndrome, suggesting a particularly progressive illness. The possible mechanisms involved in this exceptional association are discussed.

[Hashimoto's encephalopathy: an anatomicoclinical observation]. / Encéphalopathie d'Hashimoto: une observation anatomo-clinique.

Hashimoto's encephalopathy (HE) is a rare neurological complication of chronic lymphocytic thyroiditis. As its clinical presentation is aspecific, other etiologies of acute encephalopathy have to be ruled out. We report the case of a 29-year old woman with neuropsychiatric signs preceding coma, myoclonus and epileptic seizures. Clinical and electroencephalographic features were consistent with the diagnosis of new variant of Creutzfeldt-Jakob disease. However, high titres of antithyroid antibodies in serum directed towards the diagnosis of HE. Despite oral steroids, the patient died five months later. Neuropathological findings ruled out spongiform encephalopathy and disclosed aspecific activated microglia. Our observation suggests that this process could be involved in the pathogenesis of HE. Even in the absence of clinical dysthyroidism, HE diagnosis has to be suspected in the settings of acute encephalopathy associated with seric antithyroid antibodies.

Dementia with prominent frontotemporal features associated with L113P presenilin 1 mutation.

The authors report a presenilin 1 (PSEN1) mutation (L113P) in a family with six cases of dementia. The patients had personality changes and behavioral disorders, whereas spatial orientation and praxis were preserved late in the course of the illness. Neuroimaging features were consistent with the diagnosis of frontotemporal dementia. The authors conclude that PSEN1 mutations can be associated with clinical features of frontotemporal dementia.

[Obsessive-compulsive behavior caused by bilateral lesions of the lenticular nuclei. A new case]. / Comportement compulsif d'allure obsessionnelle par lésion bilatérale des noyaux lenticulaires. Un nouveau cas.

Another case of obsessive behaviour as the consequence of lentiform nuclei lesions is reported. All the aspects of an obsessive neurosis were present: irresistible compulsiveness, acute anxiety in case of non accomplishment, magic-like clues. The symptoms appeared after a carbon monoxide poisoning, in a 31-years-old woman who had previously a pathological personality, of the psychopathic type, but without obsessive features. The onset of compulsions was paralleled by frontal-like psychological changes, but there was no reduction of intellectual or mnesic functions. A previous depressive state continued after the poisoning, so that the patient did not show an empty mind as did the other patients; the loss of "self-psychic activation" was only partial. All these case-reports emphasize the fact that the neuropsychological testing must not be limited to the behaviour and must investigate the "mental content" as well. They also lead us to emphasize similitudes between psychic disorders related to lenticular lesions and those related to frontal lobe lesions, including obsessive-like activities. Such case-reports provide a model in which some components of the obsessive neurosis, hebephrenia and depression are the consequence of an identified brain damage. It can be suggested that the neural circuits involving frontal lobes and parts of the neostriatum and of the pallidum are implicated in the mechanisms of these three types of psychoses.

[Articular manifestations in leprosy]. / Les manifestations articulaires de la lèpre.

Articular manifestations occur in approximately 1% of cases of leprosy, sometimes at onset. They consist in a highly inflammatory polyarthritis, fairly similar to that seen in rheumatoid polyarthritis. They often herald a reactive leprous exacerbation and are dependent upon immunologic disturbances in brittle leprosy (mainly lepromatous). Joint pain should be differentiated from neurologic pain resulting from peripheral neuropathy which is often concomitant. Leprosy should be considered among the causes of polyarthritis, especially in immigrants, but also in residents who have travelled to areas where leprosy is endemic.

Les manifestations articulaires de la lèpre / Articular manifestations in leprosy

Articular manifestations occur in approximately 1% of cases of leprosy, sometimes at onset. They consist in a highly inflammatory polyarthritis, fairly similar to that seen in rheumatoid polyarthritis. They often herald a reactive leprous exacerbation and are dependent upon immunologic disturbances in brittle leprosy (mainly lepromatous). Joint pain should be differentiated from neurologic pain resulting from peripheral neuropathy which is often concomitant. Leprosy should be considered among the causes of polyarthritis, especially in immigrants, but also in residents who have travelled to areas where leprosy is endemic.

[A therapeutic trial in a neurology department]. / Essai thérapeutique dans un service de neurologie.

A study of the therapeutic efficacy of tiapride in various neurological disorders was carried out during the period October 1974 to May 1975. Good results were obtained in headaches following lumbar puncture and removal of CSF, and in agitated states, especially those associated with alcoholism.