The value of clinical characteristics from a standard questionnaire to discriminate cardiac from neurological syncope in the elderly.

OBJECTIVES: To identify signs and symptoms to differentiate cardiac from neurological syncope in patients over 70 using a standardized questionnaire. DESIGN: Prospective cohort study. SETTING: Five short-stay units in a French university hospital. PARTICIPANTS: One hundred thirty-one in-patients with syncope aged 70 and older. MEASUREMENTS: Patients were interviewed about the signs and symptoms that had been present before, during or after syncope. When possible, a witness who had been present during syncope was also interviewed to compare theirs and the patients' answers. The sensitivity and specificity of 35 questions were calculated among 3 groups defined according to the cause of syncope: cardiac (n = 58), neurological (n = 31) and syncope of unknown origin (n = 42). Statistical analyses were performed to determine discriminating signs and symptoms among the causes and crude agreement was calculated for answers from patients and witnesses. RESULTS: Only 8 and 3 of 35 questions had a sensitivity of at least 0.5 for cardiac and neurological causes respectively. A feeling of impending syncope, thoracic oppression, recall of events preceding syncope and a history of arrhythmia were independently and significantly discriminant among groups. Recall of events preceding syncope (Odds Ratio (OR) = 7.5; 95% confidence interval (CI) = 2.2-25.3) and a personal history of arrhythmia (OR = 4.8; 95% CI = 1.6-14.2) were discriminant between cardiac and neurological causes suggesting mostly a cardiac cause. Agreement between patients and witnesses was only found for questions on the patient's medical history or the circumstances surrounding the onset of syncope. CONCLUSIONS: Recall of events preceding syncope and a history of arrhythmia are strongly suggestive of a cardiac rather than a neurological cause of syncope. Interviews of witnesses are not helpful in suggesting a cause for syncope.

[Bickerstaff's brainstem encephalitis]. / La rhombencéphalite postinfectieuse: le syndrome de Bickerstaff.

INTRODUCTION: Bickerstaff's brainstem encephalitis represents a differential diagnostic that must be discuss with meningoencephalitis with ophtalmoplegia, ataxia and confusion. EXEGESE: A 26 year-old woman presented a Bickerstaff syndrome. A severe disturbance of consciousness had lead to admission in intensive care unit with mechanical ventilation. Electrophysiological tests and the brain magnetic resonance were normal. We have not observed systemic anti-G1Qb antibody in our patient. Nevertheless, 66% of patients with Bickerstaff syndrome have anti-GQ1b antibody during the acute phase period. We observed a beneficial effect of IV Ig treatment although no significant improvement was observed with corticosteroids. CONCLUSION: Controlled clinical trials are needed to established the efficacy of IV Ig or plasmapheresis as a specific therapy for this pathology.

Durable remission of a relapsing primary central nervous system lymphoma after autologous bone marrow transplantation.

A 30-year-old patient with a relapsing primary central nervous system lymphoma (PCNSL), was successfully treated with salvage chemotherapy and high-dose therapy including drugs that cross the blood-brain barrier followed by ABMT. Cerebrospinal irradiation was administered after hematological recovery. Six years after transplantation the patient is alive without evidence of recurrent disease with a good neuropsychological status. This result could justify further studies of aggressive management of PCNSL in relapse.

An isochromosome 6p in a primary meningeal malignant melanoma.

The rearrangement of chromosome 6, particularly the deletion of 6q, has been observed in human malignant melanoma with or without brain metastases. The isochromosome 6p has also been described. In this study, we report the cytogenetic analysis of a primary malignant melanoma of the central nervous system. Its dominating karyotype was 47,XX,+i(6)(p10). Fluorescence in situ hybridization (FISH), using a 6p chromosome arm probe, confirmed the structure of the isochromosome. To our knowledge, this is the first report of this type of chromosomal aberration in an uncommon neoplasm of leptomeningeal melanocytic origin.

Neurologic disorders due to brain manganese deposition in a jaundiced patient receiving long-term parenteral nutrition.

BACKGROUND: Neurologic and radiologic disorders have been reported in patients receiving long-term parenteral nutrition (PN). On the basis of elevated serum manganese levels, some of these abnormalities have been attributed to manganese intoxication. Alterations of the basal ganglia signal intensity on T1-weighted magnetic resonance images (MRIs) have been previously reported, but the precise nature of these alterations remains controversial although the deposition of manganese has been suggested in patients with chronic hepatic encephalopathy due to liver failure. METHODS: We report the case of a patient who was receiving PN and exhibited a chronic cholestasis. Neurologic disorders appeared after several months of PN, when a hypersignal in the basal ganglia and white matter was found on T1-weighted MRIs of the brain in association with elevated serum and manganese levels. RESULTS: Elevated autopsic concentrations of manganese were found in the radiologic abnormal cerebral areas. CONCLUSIONS: Our observation is the first demonstration of a relationship between high intracerebral manganese levels, radiologic abnormalities, and neurologic disorders during long-term PN. Moreover, serum manganese levels are not a good indicator of cerebral levels. In fact, in our patient, serum manganese levels returned to normal, whereas those of cerebral manganese remained increased.

Vagal nerve stimulation in tuberous sclerosis complex patients.

This is an open-label, retrospective, multicenter study to determine the outcome of intermittent stimulation of the left vagal nerve in children with tuberous sclerosis complex and medically refractory epilepsy. The records of all children treated with vagal nerve stimulation were reviewed in five pediatric epilepsy centers to locate those with tuberous sclerosis complex who had been treated with vagal nerve stimulation for at least 6 months. These patients were compared with (1) a series of patients obtained from the literature, (2) 10 similar control patients with epilepsy obtained from a registry of patients receiving vagal nerve stimulation, and (3) four published series of tuberous sclerosis complex patients whose epilepsy was surgically managed. Ten tuberous sclerosis complex patients with medically refractory epilepsy treated with vagal nerve stimulation were found. Nine experienced at least a 50% reduction in seizure frequency, and half had a 90% or greater reduction in seizure frequency. No adverse events were encountered. Comparison with published and registry patients revealed improved seizure control in the tuberous sclerosis complex patients. Comparison with the group undergoing seizure surgery demonstrated improved outcomes after surgery. Vagal nerve stimulation appears to be an effective and well-tolerated adjunctive therapy in patients with tuberous sclerosis complex and seizures refractory to medical therapy. Resective surgery has a better prospect for improved seizure control.

[Malignant degeneration of epidermal cyst of the posterior cranial fossa. Diagnostic value of magnetic resonance imaging]. / Dégénérescence maligne d'un kyste épidermoïde de la fosse postérieure. Intérêt diagnostique de l'imagerie par résonance magnétique.

A 72 year-old woman presented with a rapidly evolving left cerebellopontine angle syndrome secondary to a squamous cell carcinoma developed in an epidermoid cyst. The value and the results of magnetic resonance imaging are discussed.

[Marchiafava-Bignami disease with favorable development]. / Maladie de Marchiafava-Bignami d'évolution favorable.

A 35 year-old right-handed woman with a history of alcoholism presented signs of interhemispheric disconnection. CT of the brain revealed a low-density areas in the corpus callosum. Two months later, clinical examination was normal, but the CT showed cystic area in the genu and splenium. MRI was also consistent with a lesion of the corpus callosum. CT and MRI did not show lesions in the hemispheric white matter.

[Continuous partial epilepsy disclosing diabetes mellitus]. / Epilepsie partielle continue révélatrice d'un diabète sucré.

Continuous partial epilepsy (CPE) is characterized by isolated, subintrant clonus focalized to a limited territory with critical focal electroencephalography in a concordant territory. CPE is observed in various cortical lesions but also in disorders of metabolism and notably decompensated diabetes mellitus. We report a case of CPE without focal lesion at MRI which revealed hyperglycaemia without ketosis. The 54-year old female patient was hospitalised for C.P.E.. Early CT and later MRI gave normal results. Biochemistry showed hyperglycaemia without kenoturia, acidosis or hyperosmolality. Insulin therapy rapidly brought glycaemia down to its normal level and the clonsism disappeared. Five months later, the patient had no other seizure and the EEG was normal. Epileptic seizures are frequent in hyperglycaemia without ketosis (25% of the cases) where they are mainly partial and motor (75 to 86% of the cases), rarely associated with a focal lesion (15% of the cases with CT scan). They are rare in patients with ketoacidosis. This apparent protective effect of ketoacidosis may be attributed to an increase of GABA bioavailability consecutive to acidosis. CPE is resistant to antiepileptic treatments. In CPE induced by hyperglycaemia without ketosis normalization of blood glucose level with insulin therapy is concomitant with a rapid cure of epilepsy. Thus glycaemia should be measured in all patients presenting with CPE, the aim being to diagnose hyperglycaemia without ketosis rapidly to avoid hyperosmolality and to prescribe an adequate treatment based exclusively on insulin and rehydration.

[Antineoplastic chemotherapy and Wernicke's encephalopathy]. / Chimiothérapie anticancéreuse et encéphalopathie de Gayet-Wernicke.

BACKGROUND: Wernicke's encephalopathy is usually seen in alcoholic adults. It is rare in childhood and usually discovered in a context of several pathological events. We report here a typical case of a teenager. CASE REPORT: A 15-year-old girl with acute leukemia was given chemotherapy that resulted in profound aplasia and serious infection. She exhibited abnormal eye movements, ataxia, lethargy, enuresis and amnesia. MR examination showed T2-weighted images with increased signal in the thalami (pulvinar) and periaqueducal region. The symptoms improved dramatically with thiamine therapy. Omission of the usual vitamin supplementation between the courses of chemotherapy was responsible for this encephalopathy. CONCLUSION: Thiamine deficiency can lead to death or amnesia. Rapid efficacy of vitamin supplementation helps diagnosis and prevents sequelae.

[Pituitary adenoma treated with a somatostatin analog]. / Adénome thyréotrope traité par un analogue de la somatostatine.

We studied for over 7 months the use of SMS 201-995, in 1 woman with pituitary adenoma. Eight years after surgical and irradiation therapy, adenoma relapsed with hyperthyroidism (T4 L = 71pmol/l, TSH = 5.3 mU/l, subunit: 309 micrograms/l) and was complicated by meningitis. It was invasive and removed only partly. SMS therapy, 100 micrograms SC q 8 h, allowed partial reduction of clinical hyperthyroidism and decreased TSH and alpha subunit of 47%. Increased dosage and changing route of administration did not increase efficacy. A modest decrease of adenoma was probably caused by several reasons. In summary in an adenoma unusual by the extremely high value of alpha subunit in contrast with modest value of TSH, SMS therapy allowed partial control only.

[Causes of first convulsive seizures in adults. According to age and sex]. / Causes des premières crises convulsives de l'adulte. En fonction de l'âge et du sexe.

In the absence of epidemiological studies, we thought it might be of interest to investigate the relative frequency of first seizures in adults according to age and sex and in comparison with a control population. Our study of 317 patients admitted to hospital a few hours at most after the seizures demonstrated that alcoholism in young adults and vascular pathology in the elderly play an important role in triggering the first seizures, which accounts for the male predominance in epilepsy of the adult. It also showed that the occurrence of first seizures increases with age, especially after 60 years. We found that premonitory symptoms were present in almost one-third of the patients, even when the seizures seemed to be generalized from the start, and that there was a risk of one or several attacks during the hours that followed the first seizures.

Primary thrombocythemia in a patient with cerebellar infarction.

A patient suffering from essential thrombocythemia presented manifestations of digital thromboses and two cerebral ischemic strokes. Anomalies of platelet function are discussed in relation to published data and the efficacy of antiaggregant treatment is stressed.

Benign solitary neurinomas of the sciatic popliteal nerves CT study.

Two cases of solitary benign neurinomas of the sciatic popliteal nerves are reported. Computed tomography (CT) confirmed the clinically suspected diagnosis of a tumor of nervous origin, and provided accurate preoperative location of these tumors.

Anorectal manometric anomalies in seven patients with frontal lobe brain damage.

We studied the anorectal manometric and urodynamic pattern of seven patients who had right and/or left frontal lobe injury to investigate the possibility that the frontal lobe area plays an important role in control of anorectal motility as it does for the urinary bladder. We found that the disturbances of anorectal manometric recordings were similar to those of urodynamic recordings: (1) lack of urinary bladder filling sensation (two cases), increased perception threshold of rectal distension (two cases); (2) uninhibited detrusor contractions during filling (five cases), spontaneous rectal contractions during the resting step (four cases); (3) absence of micturition (one case), absence of rectoanal inhibitory reflex (one case). Furthermore, in all but one patient, we observed a decrease in the amplitude and duration of the voluntary contraction of the anal canal (six cases). However, in two of the seven patients we found hypertonia in the anal canal (upper part: one case, lower part: one case), but never in the urethral canal. Our results suggest that frontal lobe may be involved in the nervous control of anorectal motility as it is for urinary bladder function. However, the lack of correlation between urinary and anorectal anomalies in individual cases suggests that these functions depend on distinct areas of the frontal lobe.