Membrane tension lowering induced by protein activity.

Using videomicroscopy we present measurements of the fluctuation spectrum of giant vesicles containing bacteriorhodopsin pumps. When the pumps are activated, we observe a significant increase of the fluctuations in the low wave vector region, which we interpret as due to a lowering of the effective tension of the membrane.

[Celiac crisis with quadriplegia due to potassium depletion as presenting feature of celiac disease]. / Crise coeliaque associée à une tétraplégie par hypokaliémie de déplétion révélatrice d'une maladie coeliaque.

INTRODUCTION: Adult coeliac disease revealed by coeliac crisis and quadriplegia due to potassium depletion is an extremely rare situation. CASE REPORT: A 26-year-old woman presented with a suddenly developed weakness of all four limbs and a severe diarrhea. Authors emphasize coeliac crisis, which is a presenting feature of coeliac disease, characterized by acute diarrhea with life-threatening acid base and electrolyte abnormalities. The patient improved with correction of hypokalemia and gluten-free diet. CONCLUSION: A severe acute diarrhea with metabolic and systemic complications, the so-called coeliac crisis, is a possible presenting clinical feature of a previously undiagnosed adult celiac disease.

[Sneddon's syndrome: 15 cases with cerebral angiography]. / Le syndrome de Sneddon: étude de 15 cas avec artériographie cérébrale.

Sneddon's syndrome is a rare disease defined by the presence of ischemic cerebrovascular events associated with livedo reticularis. We report a retrospective study of fifteen cases, thirteen women and two men, mean age of 37.93+/-9.77 years. All patients presented one or more cerebral infarcts. Six patients had dementia. Brain magnetic resonance imaging showed several cortical infarcts with white matter involvement. Cerebral angiography performed in all patients, showed a distal arteriopathy in twelve and thrombosis of the right carotid internal artery in one. One patient had antiphospholipid antibodies. Ten patients were treated with antiplatelet agents and five with anticoagulants. The course was favorable in eight patients and stationary in three. Four patients had several recurrent infarcts, one when anticoagulants were discontinued, one taking an anti-sludge-platelet agent and two who were not initially taking any treatment.

Active membranes studied by X-ray scattering.

In view of recent theories of "active" membranes, we have studied multilamellar phospholipid membrane stacks with reconstituted transmembrane protein bacteriorhodopsin (BR) under different illumination conditions by X-ray scattering. The light-active protein is considered as an active constituent which drives the system out of equilibrium and is predicted to change the collective fluctuation properties of the membranes. Using X-ray reflectivity, X-ray non-specular (diffuse) scattering, and grazing incidence scattering, we find no detectable change in the scattering curves when changing the illumination condition. In particular the intermembrane spacing d remains constant, after eliminating hydration-related artifacts by design of a suitable sample environment. The absence of any observable non-equilibrium effects in the experimental window is discussed in view of the relevant parameters and recent theories.

[T-cell lymphoma revealed by a mononeuritis multiplex: case report and review of literature]. / Lymphome T révélé par une mononeuropathie multiple: étude d'un cas avec revue de la littérature.

INTRODUCTION: Lymphoma occasionally affects the peripheral nervous system. Neuropathy usually appears in patients with known lymphoma but rarely represents the initial manifestation of underlying malignancy. We report a case in which mononeuritis multiplex (MM) was the dominant feature in the clinical presentation of a peripheral T-cell non-Hodgkin lymphoma (NHL). OBSERVATION: A 32-year-old man suffered from an asymmetric progressive sensory-motor peripheral neuropathy. The left peroneal nerve was affected first, then the left median nerve after one month, followed by the left trigeminal nerve ten months later. The electrophysiological study confirmed the diagnosis of axonal sensory-motor MM. Mediastinal adenopathies, splenomegaly, pancytopenia and inflammatory syndrome were also found. An osteo-medullary biopsy showed a T-cell NHL. Nerve biopsy study found an inflammatory lymphoid infiltration without malignant cell supporting the hypothesis of an inflammatory pathogenic process. Chemotherapy including cyclophosphamide, hydralazine, vincristine and prednisone were administered monthly during 8 months. No improvement was obtained. DISCUSSION: It must be emphasised that this case is an uncommon one. On the one hand, NHL is rarely associated with MM and on the other hand, it can exceptionally be revealed by a MM. We were able to find 30 reported cases of distal neuropathy revealing a NHL including, 8 mononeuritis simplex, 9 MM and 13 polyneuropathies. Polyradiculoneuritis cases were excluded from this study because the neuropathy is usually caused by a meningeal infiltration. The neuropathy was in the majority of the cases chronic and axonal. The lymphoma was more often B-cell than T-cell. The B-cell lymphoma was frequently associated with a poor prognosis. All mechanisms were present with a predominance of neurolymphomatosis.

[Neuropsychological and magnetic resonance imaging findings in five patients after carbon monoxide poisoning]. / Troubles cognitifs dus à l'intoxication oxycarbonée: étude neuropsychologique et IRM de 5 cas.

INTRODUCTION: Carbon monoxide (CO) poisoning has been shown to result in cognitive impairments. These disorders have rarely been reported. The present study aimed to evaluate these disturbances in five patients with a neuroanatomical study. METHODS: There were two men and three women with an average of 25 years old. Patients were explored several months after acute CO poisoning. Neuropsychological testing was administered to assess memory, intellectual, executive, visual-spatial and constructional functions, language, praxis and gnosis. Cerebral magnetic resonance imaging (MRI) was performed in all patients using axial, sagittal and coronal slides with T1 and T2 weighted and flair images. None of the subjects had hyperbaric oxygen. They received 7, 5 mg bromocriptine per day. RESULTS: All patients presented cognitive disorders including marked impairment in long term memory with a severe defect in recall performance in comparison to recognition memory. Visual memory was more affected than the verbal one. There were also moderate disturbances in intellectual, executive, visual-spatial and constructional functions. One patient presented alexia agraphia, severe visual disturbances, constructional and dressing apraxia. Four patients had depression and one psychic akinesia. Cerebral MRI studies revealed that all patients had bilateral pallidal necrosis, bilateral hippocampal and moderate cortical atrophy. Fornix atrophy was found in 2 patients and corpus mammillary atrophy in 3 patients. Others lesions were also found: bilateral cerebellar in two cases and cortical in three cases. Treatment with bromocriptine was effective in three cases. There was no improvement in the patients treated 14 months and 5 years following CO poisoning. CONCLUSION: Neuropsychological impairments in CO poisoned subjects include memory, intellectual, executive, and visuospatial defects. In addition to pallidal necrosis, which is a typical feature of CO poisoning, hippocampal and cortical atrophy are often present. Bromocriptine can improve the cognitive disorders.

[Acute Guillain-Barré-like polyradiculoneuritis revealing acute systemic lupus erythematosus: two case studies and review of the literature]. / Polyradiculonévrite aiguë de type Guillain-Barré révélant un lupus érythémateux aigu disséminé: étude de deux cas et revue de la littérature.

The involvement of the peripheral nervous system in systemic lupus erythematosus (SLE) is rare and is dominated by distal symmetric axonal polyneuropathy and multiple mononeuropathy. It usually occurs in late course of the disease. Acute polyradiculoneuropathy of Guillain-Barré syndrome type is very rare and can frequently constitute the first symptom of systemic lupus. We report two cases of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) complicated by respiratory failure due to systemic lupus. In the first case, the pure motor AIDP was the first manifestation of the SLE. The outcome under prednisone treatment was dramatically good with regression of clinical deficit and normalisation of nerve conduction within one month and 12 months of treatment respectively. In the second case the AIDP occurred only one week after diagnosis of SLE and corticotherapy. It was a demyelinating sensory-motor neuropathy. Clinical improvement was obtained after two cures of intravenous gammaglobulin (IVIg). The normalisation of nerve conduction was obtained within 8 months. AIDP is a very rare complication of SLE, but it should be searched as an aetiology of Guillain-Barré syndrome associated to systemic clinical symptoms or to blood inflammation. Corticotherapy could be sufficient, but in some cases the addition of IVIg or plasmapheresis might be necessary.

[Chronic inflammatory demyelinating polyradiculoneuropathy with hypertrophy of spinal roots, brachial plexus and cranial nerves]. / Polyradiculoneuropathie inflammatoire démyélinisante chronique hypertrophique avec atteinte des nerfs crâniens: à propos de deux observations.

We report two patients who presented an atypical chronic inflammatory demyelinating polyradiculoneuropathy with massive nerve root and brachial plexus hypertrophy, and pseudotumoral supraclavicular mass. They also presented an hypertrophy of oculomotor and trigeminal nerves causing an exophthalmos and ocular palsy. Spinal root enlargement and cranial nerve hypertrophy was demonstrated by CT scanner and MRI. Brachial plexus biopsy showed a similar aspect of sural nerve, with an extensive onion bulb formation and perivascular inflammatory cell infiltration. There was an excellent response to steroids in both patients.

[Wilson's disease. Clinical presentation, treatment and evolution in 21 cases]. / La maladie de Wilson. Etude clinique, thérapeutique et évolutive de 21 cas.

PURPOSE: Wilson's disease is characterized by neuropsychiatric symptoms with frequent extrapyramidal and intellectual presentations. They have an insidious evolution that leads to a late diagnosis and less therapeutic effectiveness in the advanced forms. METHODS: We report 21 cases of Wilson's disease with neurological complications, emphasizing clinical semiology, diagnostic means and problems of the therapeutics in our country. RESULTS: The average age at the beginning of the disease was 17.6 years, with a female prevalence (8/13). The signs at first were mostly all neurological (71.4%), then psychiatric (19%) or hepatic (19%). The most common neurological signs were dystonia of members (81%), dysarthria (76%), tremors (76%) or disorders of motoricity (71.4%). Sometimes there were sialorrhea or disorders of the handwriting. The Kayser-Fleischer ring was present in 19 patients. Eighteen patients had clinical and/or biological hepatic involvement. The diagnosis was confirmed by biochemical examinations, which found a low rate of copper in blood, a sinking rate of ceruloplasmin and a very high rate of urinary copper. The cerebral computer tomography shows a cortical and/or subcortical atrophy (37%), and/or a low density of the central grey cores (35%). The treatment was based on D-penicillamine and/or zinc sulfate, according to the availability of the drugs. The evolution was favourable among 18 patients (85%) and not good in 42.8% of the cases. Six of the first patients had poor evolution after many years of follow-up. Finally, only 12 patients (57%) had a very good outcome. The family investigation made among 17 patients revealed 13 family cases. The only predictive factor of a poor evolution was the therapeutic noncompliance (P = 0.006). CONCLUSIONS: The neurological presentations are traditional during the Wilson's disease, but are often ignored. We must suspect the disease in children when faced with disorders of handwriting or school failures and in the adult, when faced with neurological symptoms in a patient having a hepatic disease. We must not hesitate to consider it even given purely psychiatric signs, and we had better know to seek the neurological ones.

[Acute meningomyelitis and polyradiculoneuritis disclosing systemic lupus erythematosus]. / Méningomyélite et polyradiculonévrite aiguës révélant un lupus érythémateux disséminé

A 25-year-old male presented purulent meningitis associated with transverse myelitis. Spinal T2-weighted MRI showed a large spinal cord with an intramedullary high signal. Infection resolved with antibiotic therapy but spastic paraplegia persisted. Four months later, he developed a Guillain-Barré syndrome with clinical and biological signs of systemic lupus erythematosus. Final outcome was fatal despite corticosteroid and immunoglobulin treatment.

[Neuropsychological disorders after bithalamic infarct caused by deep venous thrombosis]. / Troubles neuro-psychologiques après infarctus bi-thalamique par thrombose veineuse profonde.

We report a case of deep cerebral venous thrombosis with bithalamic infarction that led to neuropsychological disorders including left side visuospatial neglect, aphasia and amnesia, as well as frontal and intellectual disorders. After a six month course, the patient showed only slight intellectual deficit and mild anterograde amnesia. Deep cerebral venous thrombosis is uncommon and prognosis is poor. Reports in the literature illustrate the neuropsychological disturbances they provoke but provide little analyzable data. The positive progress in our case demonstrates that bithalamic lesions of venous origin can have a good prognosis.

[Relapsing polyneuropathy in the post-partum period]. / Polyradiculonévrite à rechutes du post-partum.

A patient had five relapses of polyneuropathy: four developed during post-partum. The rapid onset of symptoms with subsequent complete recovery are in favor of a recurrent Guillain-Barré syndrome rather than a chronic relapsing inflammatory polyneuropathy.

[Acute myelitis and Behcet's disease: three case reports]. / Myélite isolée et maladie de Behçet: trois observations.

INTRODUCTION: Neurological manifestations in Behçet's disease usually consist of meningoencephalitis and cerebral thrombophlebitis. Myelitis is rare, especially when it is the only neurological manifestation. EXEGESIS: The authors report three cases of acute myelitis complicating Behçet's disease demonstrated by magnetic resonance imaging. In two cases, Behçet's disease was revealed by myelitis. The three cases were transverse myelitis, two of which were juvenile Behçet's disease and two were meningomyelitis. CONCLUSION: Magnetic resonance imaging can help guide diagnosis of myelitis in Behçet's disease. It can also be useful to assess the results of the treatment. Clinical symptoms of Behçet's disease must be searched for in the case of myelitis, especially for patients from Mediterranean countries.

[Cerebral infarction, pseudolupic syndrome and myxoma of the left atrium]. / Infarctus cérébral, syndrome pseudolupique et myxome de l'oreillette gauche.

Left atrial myxomas are a rare stroke etiology (0.4%). They frequently present general symptoms such as fever, weight loss and an inflammatory syndrome, but the pseudolupic syndrome with cutaneous features and vasomotor symptoms is rarely described. We report the case of a 38-year old man who presented in March 1995 a stroke caused by a left atrial myxoma. There was also pseudolupic clinical and biological manifestations which disappeared after ablation of the myxoma. Cerebral angiography showed a small carotid aspect which evoked carotid dissection or carotid siphon occlusion. The authors discuss neurologic and pseudolupic manifestations of left atrial myxomas, and their physiopathologic mechanism.

[Neck-tongue syndrome: a case caused by atlanto-axial osteoarthritis of tuberculous origin]. / Le syndrome "neck-tongue": un cas par ostéo-arthrite atloïdo-axoïdienne d'origine tuberculeuse.

The neck-tongue syndrome involves paroxystic pain in the nape of neck associated with sensitive disorders of the ipsilateral hemitongue aggravated by movements of the nape of neck. It is attributed to relating fibers of proprioceptive origin which pass through the great nervus hypoglossus from the second cervical stria. The lingual pseudoathetosis is also reported to a proprioceptive deafferentation of the tongue. In most of cases, no etiology has been found. We report a case of neck-tongue syndrome with pseudoathetosis, related to an atloaxoid osteoarthritis of tuberculous origin.

[Pure motor hemiplegia with ipsilateral lingual palsy caused by pontine infarction]. / Hémiplégie motrice pure avec paralysie linguale ipsilatérale par infarctus du pont.

A 36 year old diabetic man developed a pure motor hemiplegia (PMH) associated with an ipsilateral lingual palsy. Magnetic resonance imaging revealed a pontine infarct. Lingual palsies have never been reported in patients with PMH so far, but may be associated with other lacunar syndromes such as the "dysarthria-clumsy hand syndrome". This observation supports the hypothesis that corticohypoglossal pathways may have bilateral and assymetrical projections.

[L-dopa-responsive dystonia: 2 familial cases of adult onset with sleep disorders]. / Dystonie sensible a la L-Dopa: deux cas familiaux a début chez l'adulte avec troubles du sommeil.

A woman and her son had progressive dystonia and chronic insomnia at 32 and 19 years of age respectively. Levodopa was markedly effective at low dose both for dystonia and insomnia without dyskinesia over a 5-year follow-up period.

[Alexia without agraphia in the Arabic language. Neurolinguistic and and MRI study]. / Alexie sans agraphie en langue arabe. Etude neurolinguistique et IRM.

A 33 year-old woman developed an alexia without agraphia, a color anomia, a right hemianopia, an aphasic amnesia and a verbal amnesia. The brain MRI showed the lesions in the left splenium of corpus callosum, forceps major, optic radiations and anterieur temporal lobe. The fact that she measured writing comprehension and had complete recovery of reading impairment despite the persistence of anatomic lesions plead in favour of an active participation of the right hemisphere (RH) on reading; this capacity of the RH may be due to the linguistic particularities of arabic writing.

[Dermatomyositis and Wilson disease]. / Dermatomyosite et maladie de Wilson.

A 15 year-old girl developed both a dermatomyositis and a Wilson's disease. A clinical remission was obtained with steroids and D-penicillamine. The potential role of cupric intoxication in the pathogeny of the muscular syndrome is discussed.

[Thrombosis of the upper longitudinal sinus and syphilitic cranial osteitis]. / Thrombose du sinus longitudinal supérieur et ostéite crânienne syphilitique.

A 33-year old man developed progressive intracranial hypertension with papilloedema due to thrombosis of the superior sagittal sinus (SSS) by extension of a skull osteitis. Serological tests for syphilis were positive in blood, CSF and synovial fluid. Under treatment with penicillin and corticosteroids signs of intracranial hypertension and CT scan abnormalities disappeared. SSS thrombosis persisted at angiographic control.