[Encephalopathy and acute renal failure during acyclovir treatment]. / Encéphalopathie et insuffisance rénale aiguë au cours d'un traitement par aciclovir.

INTRODUCTION: Adverse neurological and renal effects can occur in patients taking acyclovir. Neurotoxicity of acyclovir results from an accumulation of the antiviral and its metabolites in the bloodstream. This can be observed in the elderly or in patients with chronic renal failure, generally in dialysis patients. Acute renal failure results from intratubular crystallization of acyclovir. OBSERVATION: A 78-year-old right-handed woman was admitted in an emergency setting for aphasia. Analysis of the cerebrospinal fluid was normal, but herpetic meningo-encephalitis was suspected and intravenous treatment was initiated with acyclovir. After the second infusion, the patient began to suffer from visual hallucinations, confusion and acute renal failure. Herpes PCR was negative in the cerebrospinal fluid, and the adverse drug reactions regressed completely after 72 hours. CONCLUSION: Renal function has to be checked often in patients given acyclovir for appropriate dose titration. Patients recover prompt from the adverse effects at drug withdrawal.

[Marchiafava-Bignami disease. 3 cases with favorable prognosis]. / La maladie de Marchiafava-Bignami. Trois observations d'évolution favorable.

INTRODUCTION: Marchiafava-Bignami disease is a complication of chronic alcoholism, with acute or subacute demyelination of the corpus callosum. Although subacute and benign forms of the disease have been described since the development of CT scan and MRI, it has usually a poor prognosis. EXEGESIS: We report three cases of Marchiafava-Bignami disease with favorable outcome. One of the patient was comatose upon hospital admission. Interhemispheric dysconnection syndrome was evidenced in two patients. CT scan and MRI showed lesions extending to the callosal white matter in these patients. CONCLUSION: Potential existence of Marchiafava-Bignami disease should be investigated in patients presenting with chronic alcoholism and mental confusion. However, accompanying coma and white matter demyelination should not necessarily be considered of poor prognosis. Clinical evaluation of interhemispheric dysconnection is of value in patients presenting with chronic alcoholism and mental confusion.

[Acute polyradiculoneuritis and Lyme disease]. / Polyradiculonévrite aiguë et maladie de Lyme.

A thirty-three-year-old patient developed polyradiculoneuritis with several post-therapeutic relapses despite excellent response to treatment by intravenous polyvalent gammaglobulin. After the second relapse, positive titres for Borrelia burgdorferi were found in serum and C.S.F. We gave her intravenous antibiotic and clinical signs and electrophysiological data improved. Our report and the literature can distinguish two clinical and electrophysiological presentations of neurological peripheral involvement in Lyme disease: meningoradiculoneuritis with axonal involvement and polyradiculoneuritis with demyelinization.

Clinical and electrophysiological phenotype of a homozygously duplicated Charcot-Marie-Tooth (type 1A) disease.

Type 1A of Charcot-Marie-Tooth disease (CMT1A) is associated with a microduplication of chromosome 17 (region 17p11.2) which contains PMP22, an important gene for peripheral nerve myelination. Patients carrying two duplications are expected to have a more severe phenotype, close to the Dejerine-Sottas syndrome. In this article, we report a family of 5 CMT1A patients in whom the unrelated father and mother carry a 17p11.2 duplication. The 2 daughters carry only one duplication (one given by the father, the other given by the mother), but the son carries two 17p11.2 duplications. Interestingly, the clinical phenotype of the son is more severe (scoliosis) compared to those of his sisters, but his motor nerve conduction velocities are in the range of a heterozygote CMT1A patient. The mechanisms leading to a more severe phenotype for CMT1A are discussed and may not be strictly related to lower nerve conduction velocities.

High incidence of neurologic complications following rapid correction of severe hyponatremia in polydipsic patients.

BACKGROUND: Patients with self-induced water intoxication usually tolerate a large, rapid increase in plasma sodium without developing osmotically induced central pontine myelinolysis. However, we have previously reported a case of clinically suspected pontine myelinolysis in a patient with self-induced water intoxication. The purpose of our study was to investigate if a subgroup of these patients may also be vulnerable to neurologic complications of hyponatremia therapy. METHOD: Over a 10-year period, we identified retrospectively 12 polydipsic patients having a total of 24 episodes of symptomatic hyponatremia with plasma sodium < or = 115 mmol/L. The mode of treatment, the kinetics of correction, and the neurologic outcome were recorded. The presence of alcoholism was noted. RESULTS: Seven patients recovered uneventfully from 19 episodes of symptomatic hyponatremia. Five patients had delayed neurologic complications. Late therapy and/or respiratory arrest might have been associated with the complications for 2 patients. The other 3 patients experienced clinical features of central pontine myelinolysis leading to death in 1. Patients with neurologic complications had a higher maximal 24-hour increase in plasma sodium concentration (21.8 +/- 3.9 vs. 15.5 +/- 5.1 mmol/L, p < .02), and a higher incidence of both overcorrection to hypernatremia and chronic alcoholism, often associated with poor nutrition. All 5 patients became water intoxicated at home, and 2 patients with pontine dysfunction had subacute rather than acute hyponatremia. CONCLUSION: A large rapid increase in plasma sodium may also be detrimental in patients with self-induced water intoxication when they are alcoholic, malnourished, and have nonacute hyponatremia.

[Cerebral ischemic arterial accidents in young adults. 40 cases]. / Accidents artériels ischémiques cérébraux de l'adulte jeune. 40 cas.

We observed 40 patients aged from 15 to 40 years who suffered either a transient ischemic attack or an arterial ischemic stroke. All patients were clinically and physically examined, i.e. chest-X rays, electrocardiograms, biological tests and C.T. scan or magnetic resonance imaging that confirmed the diagnosis of ischemic cerebral infarction. Most patients underwent echocardiography and angiography. The time span between the onset of the ischemic event and angiography was recorded. A few of them had CSF analysis and determinations of antithrombin III, protein C and protein S. The etiology was confirmed in 15 patients (5 cardioembolic diseases, 7 vasculopathies, 3 coagulopathies). Twenty three had well-known vascular risk factors, but also an increase in serum fibrinogen concentration, which might have been associated with specific predisposing factors: oral contraceptives, patent foramen ovale, migraine, craniocervical trauma, acute alcohol intoxication and infectious diseases. No cause was found in 2 patients. We suggest a practical approach and highlight the value of angiography when performed early in the course of the illness to enhance the percentage of positive diagnosis. About 45 p. 100 of the patients followed-up (mean duration: 3 years) were unable to resume normal professional activity.

[Inoperable brain metastases from bronchogenic cancers. Value of combined chemotherapy with cisplatin and 5 fluorouracil]. / Les métastases cérébrales inopérables des cancers bronchiques. Intérêt d'une chimiothérapie associant cisplatine et 5 fluoro-uracile.

We report the results of a combined of chemotherapy with CDDP and 5 FU repeated every 3 weeks in sixteen men (age range 31-73 years) with brain metastases. CT was performed after 2, 4 and 6 cycles to assess efficiency. Response was considered complete when no lesion was found on the CT scan and partial when the lesion shrunk to least half its the total volume. After two cycles, the response rate was 8/16 (50%). Treatment toxicity was mild with only one case of severe but reversible myelotoxicity (grade III). CDDP and 5 FU combined chemotherapy can be a useful treatment for brain metastasis of lung carcinoma.

[A case of mitochondrial myopathy in a family with oculo-pharyngeal myopathy]. / Un cas de myopathie mitochondriale dans une famille de myopathie oculo-pharyngée.

We report the case of a patient with mitochondrial lesions, an old woman belonging by her father and mother to a big family with oculopharyngeal muscular dystrophy. Four patients of this family have typical intranuclear tubulo-filamentous inclusions.

[Type C Niemann-Pick disease: supranuclear ophthalmoplegia associated with deficient biosynthesis of cholesterol esters]. / Maladie de Niemann-Pick de type C: ophtalmoplégie supra-nucléaire associée à un défaut de biosynthèse des esters du cholestérol.

We report a familial case of Niemann-Pick disease type C which lasted until adulthood, and which had the characteristic deficiency in cholesterol esterification from exogenous cholesterol. A review of the literature was performed concerning cases which were seen in adults and were characterized biochemically. This study showed the frequency of supranuclear ophthalmoplegia affecting essentially vertical movements and convergence and of lipid-laden cells in bone marrow which are often sea-blue histiocytes.

[Chemotherapy with cisplatin and 5-fluorouracil in inoperable brain metastases of bronchopulmonary cancers]. / Chimiothérapie par cisplatine et 5-fluorouracile dans les métastases cérébrales inopérables des cancers bronchopulmonaires.

Chemotherapy is not a common treatment for cerebral metastases. The authors report results of combination chemotherapy with cisplatin (CDDP) and fluorouracil (5-FU). Sixteen men (age range 31-73 years) with brain metastases were treated with CDDP 20 mg/m2/day in continuous infusion for 5 days (d 1-5) and 5-FU 1 g/m2/day in continuous infusion for 4 days (d 1-4), and the treatment schedule repeated every 3 weeks. A brain computerized tomography after 2, 4 and 6 cycles was performed to assess efficacy. It was considered that complete response was achieved if no lesion was found on the CT scan, and partial response if at least half of the total volume had decreased. After 2 cycles, the response rate was therefore 8/16 (50%). Treatment toxicity was very mild with only 1 case of severe but reversible myelotoxicity (grade III). It is concluded that chemotherapy combination with CDDP and 5-FU is a useful treatment for brain metastasis of lung carcinoma.

[Meningovascular syphilis. Apropos of 4 cases]. / Méningo-vascularites syphilitiques. A propos de 4 observations.

In four cases of stroke, it is only the systematic practice of serologic tests for syphilis that has permitted the true diagnosis and the true treatment. We observed cerebral, brain-stem and medullar infarction and one case of cerebellar haemorrhage. Meningo vascular syphilis is now the most frequent of the central nervous system lesion. Cerebrospinal fluid is always abnormal in active disease. The decrease of cell count is the best control of the treatment that needs high doses of Penicillin G.

[Multiple cerebral tuberculomas. X-ray computed tomographic diagnosis and follow-up: a case]. / Tuberculomes multiples intra-cérébraux. Diagnostic et contrôles tomodensitométriques de l'évolution: un cas.

A 31-year-old man who had never left France, rapidly developed right cerebellar signs accompanied by an alteration of his general condition. CT brain scan showed 7 intracerebral tuberculomas, six of which were asymptomatic. The patient was treated with three anti-tuberculous drugs and steroids, and made a good recovery. The CT changes disappeared. CT brain scan should be performed in patients with extra-cerebral tuberculosis and no CSF abnormalities.

[Familial anomaly of the seventh cervical vertebra. Radio-clinic comparison in a fourteen member family]. / Atteinte familiale de C7. Confrontation radio-clinique de 14 patients.

We report a fourteen member family presenting with an anomaly of the seventh cervical vertebra. The symptoms associate pain and paresthesias, a C8-D1 syndrome or arterial manifestations. The radiologic lesions noticed are bilateral in twelve cases, either cervical ribs or apophysomegaly of the seventh vertebra's transverse process. There is no correlation between the severity of the clinical impairment and the importance of the radiologic image. We insist on the familial character rarely reported in the literature of an anomaly noticed in radiologic practice.

[A familial case of thoracic outlet syndrome. Clinical, radiological study with treatment (author's transl)]. / Un cas familial de côte cervicale. Etude clinique, radiologique et thérapeutique.

The authors report the clinical and radiological study and the treatment of a fifteen members family (the two parents and their thirteen children) affected with a thoracic outlet syndrome secondary to a cervical rib and or an apophysomegaly of the seventh cervical vertebra. On the fifteen people considered, the father being deceased has not been examined but has an evocative clinical history. The mother has a clinical impairment with a late beginning and no radiological sign. Twelve children have clinical and radiological signs, one has radiological without clinical signs. Three children underwent a surgical treatment, the others only medical treatment. There is no satisfactory correlation between the clinical features and electrical investigations (electromyography, nerve conduction studies) and radiological examinations. The semiology is vascular, neurologic or mixt. Roos test seems more reliable than that of Adson, Calb and Roth, and Saunders. Eight grand-children out of fourty have been examined. Five have clinical and radiological signs, three have casual radiological signs. The radiological impairment of this family contrasts with the normal frequency of cervical anomalies in the population which is 0,12 to 1%. The family impairment of the thoracic outlet syndrome is rarely reported.