[Listeria monocytogenes abscess of the brain]. / Abcès du tronc cérébral à Listeria monocytogenes.

INTRODUCTION: Listeriosis commonly involves the central nervous system. Meningoencephalitis and rhomboencephalitis are the most frequent manifestations. Brain abscesses are rare. CASE REPORT: We report the case of a 63-year-old man treated with steroids for a long period; he was hospitalized for hemiparesis, confusion and fever. Clinical examination revealed meningeal signs, right hemiparesis and Parinaud syndrome. Initial CT scan was normal. The CSF contained 520 white cells/mm3 with predominance of polymorphonuclear neutrophils. An acute meningo- rhombencephalitis in an immunodepressed patient was suggested. The diagnosis of listeriosis was confirmed by blood cultures. Amoxicillin and gentamycin were started. The outcome on day 4 was severe with coma and tetraparesis. Brain MRI revealed a left peduncle abscess which descended deep into the brain reaching the internal capsule. The final clinical outcome involved residual right hemiparesis and left oculomotor nerve (III) palsy. CONCLUSION: Brain stem abscess is an uncommon form of listerial central nervous system infection. Listeria monocytogenes infection should be considered in patients with altered cell-mediated immunity that develop local neurologic deficits, a diagnosis which pursued rapidly with repeated blood cultures. Successful treatment requires early antibiotic therapy with ampicillin and gentamycin.

[Crossed anarthria and a dissociated lateralisation of language]. / Anarthrie croisée et latéralisation dissociée du langage.

INTRODUCTION: Crossed anarthria cases are uncommon and rather old. OBSERVATION: We report the case of a right-handed 55-year-old man who presented crossed pure anarthria due to a hemorrhage in the premotor cortex (feet of F1 and F2) and in the high part of Pierre-Marie's quadrangle. CONCLUSION: The study of different tasks (articulation, verbal fluency, direct object word-generation from a verb) showed a dissociated lateralisation of his language. Lexico-semantic and grammatical tasks are processed in the left hemisphere. Articulation programming occurs in the right hemisphere.

Effects of a new prolactin inhibitor, CV 205-502, in the treatment of human macroprolactinomas.

The effects of a new PRL inhibitor, CV 205-502 (CV), on human macroprolactinomas were studied in nine patients according to a prospective protocol. Five patients had undergone surgery leaving tumor remnants and persistent hyperprolactinemia. The four others were de novo patients, two of whom had received short term treatment with Parlodel. Plasma PRL levels ranged from 235-6050 micrograms/L before treatment. The doses of CV used in this trial ranged from 0.075-0.600 mg. Plasma PRL normalized in eight of the nine patients during treatment with CV. The time to normalize varied from 2 weeks to 9 months, and the doses from 0.075-0.450 mg. A tumor volume reduction of more than 50% was obtained in all four patients who had not been operated on before CV treatment. Only one of the five patients with postoperative tumor remnants had no reduction in tumor size. The drug was generally well tolerated, and no patient interrupted the treatment. Slight and short-lasting gastrointestinal symptoms were noted in several patients, and a single episode of fainting occurred in one patient when the drug was not taken at bedtime as instructed. A noticeable and persistent weight loss with anorexia was noted in two patients. Since CV 205-502, administered in a single daily dose, has tolerable side-effects and is effective in reducing PRL secretion and tumor size, it can be considered to be a useful treatment for macroprolactinomas.

Immunolabelling of mitochondrial superoxide dismutase and of Hsp60 in muscles harbouring a respiratory chain deficiency.

In mitochondrial encephalomyopathies, impairment of the electron transfer chain may lead to overproduction of reduced oxygen species because oxygen consumption is decreased. Whether heat shock proteins (Hsp) are induced or not in mitochondria against oxidative stress is questionable. Muscle ragged-red fibres are the histological hallmark of most respiratory chain deficiencies in humans. They exhibit abnormal mitochondria which accumulate mainly under their sarcolemma. Within these fibres, immunolabelling demonstrated strong expression of mitochondrial manganese-dependent superoxide dismutase and a lack of expression of mitochondrial Hsp60 within the subsarcolemmal spaces. In contrast, Hsp60 was overexpressed within the intermyofibrillar mitochondria. These findings suggest enhanced generation and dismutation of superoxide anions and that processing and integration of imported precursor proteins is impaired within the subsarcolemmal mitochondrial aggregates of ragged-red fibres, whereas protein import and assembly may still be efficient in the intermyofibrillar mitochondria of these fibres.

Decreased expression of ubiquinol-cytochrome c reductase subunits in patients exhibiting mitochondrial myopathy with progressive exercise intolerance.

The expression of mitochondrial proteins of two patients suffering from myopathy with progressive exercise intolerance and exhibiting a deficiency in the enzymatic activity of complex III (ubiquinol-cytochrome c reductase) has been analyzed by immunological titration. In both patients, the Fe-S protein, the cytochrome b and the 9.5 kDa protein were decreased while the expression of the other complex III subunits were close to normal values. This data indicates that, in some mitochondrial myopathies, proteins of the respiratory chain complexes can be accumulated in mitochondria without being integrated into a functional complex. This may be explained either by a lack of control of the coordination between the synthesis of subunits of mitochondrial and nuclear origin or by a difference in the degradation rate of the various subunits which are not properly assembled.

[Electrophysiologic data on myasthenic syndromes of the Lambert-Eaton type. A series of 23 cases]. / Données électrophysiologiques dans les syndromes myasthéniformes de type Lambert-Eaton. A propos d'une série de 23 cas.

The electrophysiological data of 23 adult patients with Lambert-Eaton myasthenic syndrome (LEMS) have been reviewed. Lung carcinoma was disclosed in 17. In six cases with an EMG follow-up ranging between one and 17 years no carcinoma was detected. The results of repetitive nerve stimulation test (RNS) were not statistically different between the 2 groups. Low CMAP ulnar amplitude was present in all patients (mean: 1.7 mV). Decremental response at low rate of stimulation (3 Hz) was present in 17/20 (means: 30%). An abnormal incremental response at high rate of stimulation was present in all cases (mean: 826%). The authors emphasize the interest of a 50 Hz stimulation for 4 s. Increase of the 'F-wave' amplitude was noticed in some cases. Electrophysiological changes suggestive of an associated mild neuropathy were noticed in eight patients but H-reflex was present in 3/3 cases. SFEMG abnormalities were found in 6/6 cases. In one case, stimulated SFEMG showed more blockings and an increased jitter with low rate of stimulation. In one case the electrical pattern of RNS could be misinterpreted as myasthenia gravis in one tested muscle only. The author's results suggest that CMAP amplitude and RNS test could be used to appreciate the short-term improvement of LEMS with treatment and in some cases for the long-term follow-up.

[Indications for the use of an epidural blood patch in the treatment of spontaneous intracranial hypotension]. / Indication du blood-patch dans le traitement de l'hypotension intracrânienne spontanée.

We report a patient with spontaneous intracranial hypotension treated with an epidural blood patch (EBP) and discuss the indication of this procedure in the treatment of this syndrome. Once diagnosis has been established (symptoms, CSF pressure, MRI), we propose to wait no more than a week, when simple treatment options have failed, before proceeding to an EBP. We inject a minimum of 20 ml, until the appearance of pain while injecting, of autologous blood in the L3-L4 epidural space. If this technique is unsuccessful, T6 would appear to be the best level to perform an EBP because it is by far the most common location of dural leakage (cervico-dorsal junction) and because this choice is also in accordance with the fact the blood may spread over 10 vertebral segments on each side of the injection level. Spinal MRI should at best be done before the procedure but is absolutely required when the patient fails to respond to the EBP conducted in the conditions we propose.

[Voluntary methanol poisoning. Severe regressive encephalopathy with anomalies on x-ray computed tomography]. / Intoxication volontaire par le méthanol. Encéphalopathie sévère et régressive avec anomalies au scanner X.

A 32 year-old woman after voluntary intoxication with methanol presented, after coma with metabolic acidosis, a prolonged parkinsonian syndrome improved by L-dopa. Initial findings were areflexia and electromyographic signs of neurogenic atrophy of lower limbs. A moderate decrease in visual acuity was associated with altered visual evoked potentials indicating a global lesion of the optic tracts. CT scan during the first few days showed symmetrical low densities areas in the putaminal regions, which unchanged 18 months later. This case can be compared with the rare reported cases with extrapyramidal signs, EMG tracing evidence of peripheral neuropathy and analogous images on CT scanning. The clinical sequelae and CT scan imaging anomalies are the consequence of the characteristic lesions of methanol intoxication, in which the role played by circulatory disorders and that related to the action of the toxic substance itself on nerve cells remains a debatable subject.

[Polyradiculoneuritis following lumbosacral zona with adrenal cortex adenoma]. / Polyradiculonévrite secondaire à un zona lombo-sacré avec adénome cortico-surrénalien.

We report a case of severe polyradiculoneuritis consecutive to a lumbosacral herpes zoster and concomitant with an adrenal adenoma with hypercorticism. The patient improved after surgery and plasmaphereses.

[Cerebral Erdheim-Chester disease]. / Maladie d'Erdheim-Chester cérébrale.

We report the case of a 26-old-year man hospitalized for first partial complex epileptic seizure. Brain MRI showed an asymptomatic pseudo-tumor lesion in the brainstem. Diabetes insipidus, hypophyseal gonadotropic deficiency and osteosclerosis of long bones strongly suggested Erdheim-Chester disease, a rare histiocytosis, confirmed after tibial biopsy. Six months later, the patient remained stable. A persistent, and even increased, enhancement with Gd-DTPA on brain MR images was noted as previously described. The review of the literature collected 64 cases, and only 7 cases of cerebral "tumor".

[Limbic encephalitis and SIADH revealing small-cell anaplastic lung cancer: MRI and immunologic findings]. / Encéphalite limbique et SIADH révélant un cancer pulmonaire anaplasique à petites cellules. Aspects IRM et immunologiques.

Paraneoplastic limbic encephalitis (PLE) is a manifestation of clinico pathological entity encephalo-myelo-neuropathy associated with anti-neuronal antibodies type 1 (ANNA-1 also called anti-Hu). Isolated PLE is rare. We reported a case of PLE in a 61-year-old heavy smoker man. An inappropriate antidiuretic hormone secretion syndrome was associated. Cranial MRI showed hyperintensity in amygdalo-hippocampic regions on T2 weighted sequences which appeared hypointense on T1-weighted sequences without gadolinium enhancement. Anti-Hu antibodies were absent in serum and in CSF. Despite chemotherapy, he died 18 months after disease onset. Our patient presented PLE without myelonouropathy and without ANNA-1 which suggests a different immunopathology.

[Alexander's disease in adults and diffuse cerebral gliomatosis in 2 members of the same family]. / Maladie d'Alexander de l'adulte et gliomatose cérébrale diffuse chez deux membres d'une famille.

A 31-year old woman died after 10 years of progressive dysautonomia and cerebellar and pyramidal symptoms. CT scan showed pontine, bulbar and cerebellar atrophy. Post-mortem examination revealed Rosenthal's fibers widespread throughout the CNS, but especially in the subependymal and perivascular regions. White matter cavitations involving peri-ventricular regions, hilum of dentate nuclei and pons were observed, leading to a diagnosis of adult form of Alexander's disease. At the age of 5, the patient had been operated upon for a chiasmatic tumor. Microscopic examination revealed a pilocytic astrocytoma without Rosenthal's fibers. No complementary radiotherapy had been done. Her mother has been operated upon in 1972, for a high-grade glioma and is still alive 20 years later. This suggests diffuse cerebral gliomatosis. This family history may suggest a relation between these different diseases. They might be the result of a transmissible astrocytic abnormality with varying expression.

[Superficial siderosis of the central nervous system]. / Hémosidérose du système nerveux central.

We report five cases of superficial siderosis of the central nervous system. All patients developed progressive deafness and cerebellar ataxia associated with pyramidal tract signs or mental deterioration. The cerebrospinal fluid examinations usually revealed an elevated protein level, without other abnormalities. Magnetic resonance imaging typically showed a hypointense rim around the cerebral and cerebellar hemispheres, the brainstem and the spinal cord on T2-weighted images. A definite source of bleeding was only found in two patients. The literature on superficial siderosis is reviewed. The etiologies and the pathogenesis are discussed.

[Hodgkin's disease manifesting as paraneoplastic limbic encephalitis]. / Encéphalite limbique révélatrice d'une maladie de Hodgkin.

INTRODUCTION: Malignancy is a possible cause of unexplained encephalitis. EXEGESIS: We describe a 59-years-old woman with limbic encephalitis, not explained by other causes, preceding diagnosis of Hodgkin's disease. Successful treatment of Hodgkin's disease was effective against neurological disturbance. CONCLUSION: This case provides evidence that Hodgkin's disease can be uncovered by paraneoplastic limbic encephalitis.

[Polyradicular lesion revealing ankylosing spondylarthritis]. / Atteinte pluriradiculaire révélatrice d'une spondylarthrite ankylosante.

INTRODUCTION: Radicular manifestations of ankylosing spondylitis are rare and observed in the course of long-term ankylosing spondylitis. EXEGESIS: The case of a young man who presented with bilateral and multiple radicular involvement is reported. Neurological symptoms occur a few weeks before ankylosing spondylitis was diagnosed. CONCLUSION: This suggests that nerve root lesions might take place during initial stages of the disease. The role of inflammatory changes in the region of the intervertebral foramina is discussed. Disease evolution is marked by relief of neurological disorders in response to anti-inflammatory treatment.

[Mitochondrial cytopathies]. / Les cytopathies mitochondriales.

Mitochondrial cytopathies are diseases due to a defect of mitochondrial respiratory chain and are characterized by the presence of morphological abnormalities of mitochondria (ragged red fibers). The clinical manifestations can be polymorphic as various organs may be involved; but in general, these disorders affect either muscle alone (progressive external ophtalmoplegia, myopathy with weakness) or muscle and brain (encephalomyopathies). Some of these diseases may be due to alterations of the mitochondrial genome. Diagnosis can be achieved through the following steps: measurement of lactate, pyruvate and ketone bodies after fasting followed by a carbohydrate rich meal and/or exercise; histological examination and biochemical investigation of a skeletal muscle biopsy; study of possible mitochondrial genome alterations.

[Disulfiram (Esperal) toxicity. Apropos of 3 original cases]. / Toxicité du disulfirame (Esperal). A propos de trois observations originales.

Prescribed since 1948 to control chronic alcoholism, disulfiram may cause severe toxicity as report in three cases of acute motive axonal polyneuritis. Disulfiram toxicity may present different clinical aspects: 1) Cytolytic hepatitis with fatal evolution in 30% of cases (fulminant hepatitis), and full recovery for the other 70%. The onset of the symptoms usually occurs as early as 15 days to a maximum of 6 months (most within 2 months) after initiation of treatment. 2) Severe optic neuritis with full recovery in 2 months. 3) Peripheral neuropathy usually dose dependent, with different clinical presentations: polyneuritis with sensory, motor, or both deficits, and few cases of tetraplegia. 4) Encephalopathy frequently associated with one of the precedent symptoms, having a favorable outcome (probably resulting in inhibition of dopamine-beta-hydroxylase by disulfiram). The mechanism of toxicity (direct or idiosyncractic) remain unclear. Disulfiram has been used safely in millions of people since 1948, and we have only few cases reports of severe toxicity. From a practical point of view, treated patients should benefit by a neurological examination once a month, ophtalmological examination every 2 months, and hepatic enzymes monitored twice a month during the 2 first months. This is the price to prevent and to detect side effects of disulfiram therapy.

[Diagnosis of muscular intolerance during exercise caused by enzyme deficiency in adults]. / Diagnostic d'une intolérance musculaire à l'exercice par déficit enzymatique chez l'adulte.

The exertional muscle pain syndrome in adults is expressed by recurrent episodes of pain and/or episodes of acute rhabdomyolysis, sometimes with transient myoglobinuria or even acute renal failure. The enzymatic origin of this syndrome is suggested by a familial history, by its usual onset during childhood or adolescence, and by its recurrent attacks usually, but not exclusively, caused by exercise. The aetiological diagnosis, suggested by the clinical context and some dynamic tests, is almost always based on good histological and biochemical examinations of the muscle biopsy.

[Analysis of 12 cases of McArdle's disease diagnosed after 30 years]. / Maladie de MacArdle diagnostiquée après 30 ans : à propos de 12 cas.

PURPOSE: McArdle's disease (MAD) or glycogen storage disease type V, usually starts in childhood or adolescence. Generally diagnosis is made before the early adulthood because patients present well defined syndrome and are constrained. METHOD: We retrospectively investigated all MAD cases diagnosed in the biochemical laboratory from Debrousse Hospital in Lyon, during 40 years (1962-2002). We then selected patients whose diagnosis had been made after 30 years. RESULTS: Fifteen patients answered our criteria but only 11 files could be analysed. A twelfth patient (service of internal medicine--Royan) supplemented the series. We sought the reasons of a late diagnosis: early age of beginning but few symptoms (7 cases), age of beginning higher than 20 years (5 cases including 3 after 45 years). The principal symptoms were muscular deficit and muscular pains (8 cases) and second wind phenomenon (7 cases). Creatinine phosphokinase level was constantly high. Ischemic effort test when it was carried out was constantly abnormal. Conversely electromyogram was often normal (5 cases). Several biopsies were necessary in a third of the cases to evoke the diagnosis, particularly among the patients with late onset symptoms. CONCLUSION: Diagnosis of metabolic MAD is generally easy if the interrogation finds inaugural symptoms in childhood or adolescence even if the patient consults very late in the life. The diagnosis can become much more difficult if it begins late in life (atypical symptoms, need for several muscular biopsy).

[Susac syndrome or microangiopathy of the cochlea, brain and retina]. / Syndrome de Susac ou microangiopathie cochléo-encéphalo-rétinienne.

OBJECTIVES: Susac syndrome, also called SICRET syndrome (small infarction of cochlear, retinal, and encephalic tissue) is a rare condition difficult to diagnose. Sudden deafness may be the inaugural sign. MATERIAL AND METHODS: A female patient developed subacute encephalopathy, bilateral sensorineural hearing loss, and ischemic retinopathy. The patient was given cyclophosphamid and methylprednisolone for six months, followed by prednisone for eight months. RESULTS: Signs of encephalopathy had totally regressed by month 14 and retinal arteries were free of obstruction. Deafness remained unchanged. CONCLUSION: Diagnosis of this microangiopathy involving the inner ear, the brain, and the retina is suggested by the clinical triad and established on the basis of tonal audiometry, fundus examination, fluorescein angiography, examination of the cerebrospinal fluid, magnetic resonance imaging. Multiple sclerosis is the main differential diagnosis. The pathogenesis remains unknown. We observed transient-evoked otoacoustic emissions. There is no consensus concerning treatment. Many advocate combining corticosteroids and immunosuppressors. Otolaryngologists should be aware that an ophthalmological examination is required for patients with central or visual disorders associated with hearing loss.