[Aquadynia: a role for VIP?]. / Aquadynie: rôle du VIP?

BACKGROUND: Aquadynia (water-related cutaneous pain) is a very rare disorder, recently described. CASE REPORT: A 40 year-old woman suffered from aquagenic pruritus, complicated by paresthesia and pain. There was no clinical argument in favor of a psychiatric disorder, Fabry's disease or any other disease. Clinical and histological cutaneous examinations were normal. Immunohistochemical study of neurotransmitters (substance P, calcitonin gene-related peptide or CGRP, vasoactive intestinal peptide or VIP, somatostatine) did not show any modification in nerve density. However, VIP-immunoreactive epidermal cells were observed. Electromyography and study of somesthesic-evoked potentials were normal. No treatment had provided any efficacy. Clonidine and capsaicin had been prescribed with partial success. DISCUSSION: Three other cases of aquadynia have been reported. Differential diagnoses of aquadynia are aquagenic pruritus and urticaria, hysteria or simulation, Fabry's disease, erythermalgia, peripheral neuropathy or polycythemia vera. The presence of VIP-immunoreactive cells suggests that VIP could be produced by these cells after contact with water. The effects of propanolol and clonidine on aquadynia are in favor of an adrenal component.

[Familial deficiency of C7 associated with adrenomyeloneuropathy]. / Déficit familial en fraction C7 du complément associé a une adrénomyéloneuropathie.

A 24-year old man presented with recurrent meningitis resulting from familial deficiency of a late component of the complement system (C7). Five years later, he developed gait disturbance, mental impairment and loss of hearing. Adrenomyeloneuropathy was diagnosed by a raised plasma long chain fatty acids level.

[Weber syndrome caused by Listeria abscess]. / Syndrome de Weber par abcès listérien.

A 63-year old diabetic man presented with left Weber's syndrome and meningitic syndrome. CSF examination showed moderate lymphocytic pleocytosis and elevated proteins with normal glucose content and sterile culture. Blood cultures yielded Listeria monocytogenes and the patient received ampicillin. While his neurological condition had partially improved, he died of heart failure. Several mesencephalic abscesses were found at autopsy.

[Tuberculosis in human immunodeficiency virus infection. 22 cases]. / Tuberculose au cours de l'infection par le virus de l'immunodéficience humaine. 22 cas.

A retrospective study of 22 bacteriologically documented cases of tuberculosis associated with HIV1 positivity was carried out in the department of infectious diseases of Marseille, south of France, a high prevalence area for HIV infection. Sixty-seven percent of the patients were intravenous drug abusers. Thirty-two percent were classified as having AIDS prior to the diagnosis of tuberculosis. All but one had M. tuberculosis isolated from bronchopulmonary samples; 5 had no parenchymatous radiological abnormalities, 5 had excavated lesions. Four patients had cervical adenopathies, 4 other patients had asymptomatic extra-pulmonary sites of infection. All patients responded well to chemotherapy; no relapse was observed during or after treatment. Eight died, two of them before treatment (cause of death unknown), 6 from other complications of AIDS within the 2 years following the diagnosis of tuberculosis. The low incidence of extra-thoracic involvement and the high frequency of excavated pulmonary lesions are discordant with most US studies, as is the relatively late onset of tuberculosis in the course of HIV infection. We suggest that different local epidemiological factors and maybe the practice of systematic BCG vaccination during childhood in France could contribute to these differences.

[Vertebrobasilar arterial dolichoectasia. Complications and prognosis]. / Dolichoectasies artérielles vertébrobasilaires. Complications et pronostic.

Symptomatic dolichoectasia of the vertebrobasilar system was found in 23 patients (16 males and 7 females, mean age: 62 years) during a 13-year period. Arterial hypertension was noted in 20 cases and associated aortic ectasia in 4. The malformation was identified in all patients on CT completed by angiography in 19, MRI in 7. Autopsy was performed in 5 cases. Fourteen subjects (group 1) presented with a vascular event (ischemic in 13) affecting the brainstem and/or cerebellum. Nine other patients (group 2) had a chronic symptomatology resulting from compression of the cranial nerves, central nervous system and/or CSF pathway. Two patients died of stroke within the first month (rupture of the ectasia in one and occlusion in the other one). The 21 survivors were followed for a mean period of 45.3 months. Eight patients had a stroke, with a significantly higher incidence in group 1 than in group 2 (p less than 0.05). Ten patients (5 in each group) developed progressive dementia possibly resulting from multiple cerebral infarction, hypertensive leucoencephalopathy, and/or hydrocephalus. Twelve patients died during the follow-up (4 of stroke, 6 of profound mental and motor deterioration, one from ruptured ectatic aorta, and the last one of unrecognized cause). The actuarial survival rate was 60% after 3 years of follow-up. Except for the incidence of stroke, inaugural manifestations (stroke vs nervous compression) did not seem to influence the long-term prognosis.

[Hemichorea in acquired immunodeficiency syndrome. Toxoplasmosis abscess in the striatum]. / Hémichorée au cours du syndrome d'immunodéficience acquise. Abcès toxoplasmiques dans le striatum.

Three HIV seropositive patients presented with cerebral toxoplasmosis which was treated by anti-infectious agents. After partial improvement, they developed hemichorea related to striatal infectious lesions. In AIDS patients with cerebral toxoplasmosis, autopsy series have reported a high incidence of basal ganglia abscesses, explaining the occurrence of involuntary movements such as hemichorea.

[Carotid artery occlusion disclosed by cerebral ischemia. Lesional, etiologic and developing aspects]. / Les occlusions carotidiennes révélées par un accident ischémique cérébral. Aspects lésionnels, étiologiques et évolutifs.

The authors report on a series of 100 patients, who underwent an ischemic cerebrovascular accident (CVA) (8 transient attacks, 15 minor CVAs and 77 completed strokes) which revealed internal carotid artery occlusion (unilateral in 93 cases and bilateral in 7), proven by ultrasonography and/or angiography. The computerized tomography scan was normal in 8 cases. The middle cerebral artery territory was involved in 84 patients (isolated or associated (25 patients) with another site), whereas an isolated watershed infarction was the only lesion in the other 8 patients. Twenty-two patients died during the acute phase. Among the 68 survivors followed for a long time, 28 were severely incapacitated. The occlusions were not atherosclerotic in 18 cases (8 spontaneous dissections, 5 cardiogenic emboli, 3 radiation-induced arteries, 2 multiple causes). Eighty-two patients had atherosclerotic thromboses (67 men and 15 women, mean age 63.1 +/- 12.7 years). Among them, 24 had previously experienced minor ischemic strokes. In this group, the two year survival rate was 63 p. 100. Patients with non-fatal atherosclerotic thromboses were followed for an average of 22.2 months, during which time 28.2 p. 100 experienced another stroke.