[Clinical presentation suggesting Bickerstaff encephalitis and intracranial hypertension]. / Tableau d'encéphalite de Bickerstaff et hypertension intracrânienne.

A 20-year-old man had progressive headache, neck pain and visual loss after upper airway infection. After 3 weeks, he developed ophtalmoplegia, ataxia, areflexia, autonomic failure, four limbs paresis with impaired consciousness. Brain and cervical MRI were normal. Ophthalmological examination confirmed bilateral papilledema. Cerebro-spinal fluid pressure was high, cell count was normal and proteins were mildly elevated. Electromyography showed presence of both proximal and distal demyelination. Electroencephalogram was slowed, with diffuse delta and theta waves. Anti-GM1 and GQ1b antibodies were negative. The patient was treated with intravenous immunoglobulins (0.4 g/kg/day) for 5 days, associated with high doses of acetazolamide and corticosteroids for papilledema. His neurological condition improved for gait, strength, pain, ophtalmoplegia and ataxia. He kept severe visual loss with optic atrophy. Diagnosis is discussed: Bickerstaff encephalitis with intracranial hypertension or malignant pseudotumor cerebri?

[Treating writer's cramp: 14 years' experience with botulinum toxin]. / Prise en charge de la crampe de l'écrivain: 14 ans d'expérience de la toxine botulique.

INTRODUCTION: Writer's cramp is a focal dystonia; treatment remains disappointing. We report our 14-year experience with a population of 119 patients aged between 18 and 85 years (average age 43 years). METHODS: Treatment was based on botulinum toxin injections (Dysport) and physiotherapy. Patients were reviewed every four to six months with clinical and video evaluation by three different observers and subjective analysis of the treatment efficiency by the patient (score of 1 to 3). The post-injection deficit, if present, was also quantified. RESULTS: In the group treated with toxin and physiotherapy, cramps improved (score 2 and 3) in 61.6% of patients; a majority of patients (n=14) reported they were moderately satisfied (score 2). In the group treated with toxin alone, 37.9% of patients were improved (score 2 and 3) with a majority (n=18) very satisfied reporting normal writing (score 3). Age was not a predictor of therapeutic response. Good results were obtained with injections of the flexor carpi radialis followed by flexor digitorum profundus II and III and the flexor pollicis longus. Seventy-one per cent of injections caused moderate muscle weakness, minimally disabling compared to the benefit of injections. Twenty-seven patients were followed for more than two years and three patients, who had achieved score 3 with excellent response, were followed for 14 years with very efficient repeated injections. If the injections were not effective the first time, we re-assessed the situation and adjusted the injections; we considered that toxin treatment was unsuccessful after three injections without benefit. CONCLUSION: The choice treatment for writer's cramp remains well-targeted injections of botulinum toxin. Physiotherapy is useful when the toxin injections are ineffective in completely improving writing. This requires close cooperation between the injector, the physiotherapist and the patient.

[The ictal bradycardia syndrome: a case report and review of the literature]. / Le syndrome de bradycardie comitiale: cas clinique et revue de la littérature.

Epilepsy is a rare cause of sinus node dysfunction which should be recognised. The authors report the case of a 55 year old woman with refractory epilepsy who had sinus arrest during her epileptic fits. After excluding a possible interaction by long-term Carbamazepine treatment, the diagnosis of the Ictal bradycardia syndrome was made in view of the simultaneous occurrence of severe bradycardia and epileptic activity recorded on electro-encephalography. Sudden death being more common in epilepsy, effective treatment of conductive cardiac abnormalities is essential. The refractory nature of the epileptic fits led to the implantation of a permanent pacemaker in this case.

Epileptic nystagmus: electroclinical study of a case.

Epileptic nystagmus (EN) is a rare form of nystagmus that occurs only during epileptic seizures. We report a case in which EN was first noted in an 8 year-old boy. Neuro-imaging was normal. Sharp waves from the left occipital lobe characterised the interictal EEG. Ictal video-EEG showed the aspects of electric seizures during clinical manifestations e.g. nystagmus. Ambulatory EEG displayed numerous diurnal and nocturnal seizures, but exclusively in REM sleep. After two weeks of sodium valproate treatment, the seizures, EEG focus spikes and nystagmus, as well as the squint, disappeared. During a two-year follow-up the child had no further seizures, EEG was normal and the school performance was unaffected. This case has the main features of benign epilepsy, although there are unusual features such as epileptic nystagmus, permanent squint, reduction of EEG paroxysmal abnormalities during NREM, and the presence of seizures during REM sleep. The observation and the significance of EN are discussed with reference to the literature.

[Hypoxic-ischemic encephalopathy in the full-term newborn. Contribution of electroencephalography and MRI or computed tomography to its prognostic evaluation. Apropos of 26 cases]. / Encéphalopathie hypoxo-ischémique du nouveau-né à terme. Apport de l'électroencéphalogramme et de l'IRM ou de la TDM à l'évaluation pronostique. A propos de 26 observations.

OBJECTIVES: Perinatal asphyxia complicated by hypoxic ischemic brain injury still remains the source of neurological lesions often serious and definitive. A major aim of neonatologists is to appreciate the severity of the hypoxic ischemic brain injury in the first days of life and to evaluate the forecast. The purpose of this work is to establish a relation between clinical signs, EEG, neuroimaging (MRI and CTS) and neuro-development. MATERIALS AND METHODS: 26 neonates from paediatric resuscitation unit (Hospital North, Marseille) were enrolled in a retrospective study since February 1994 to December 1997. All the newborns had at least one anamnestic criteria of perinatal asphyxia, an early electroencephalogram in the first two days of life and another between the third and the seventh day of life, and neuroimaging in the first 15 days of life: CTS in five cases and MRI in 21 cases. RESULTS: There was a good electro-clinic (P: 0.01) and prognostic (P: 0.03) correlation in patients within stage 3 of the "Sarnat classification". In the stage 2, the EEG did not provide valuable information about severity of the injury, and neuroimaging (especially MRI) allowed better prognosis. Diffuse brain injury or lesions of basal ganglia in MRI were associated, independently of clinical settings and EEG tracings, with a poor neurodevelopmental outcome (P: 0.02). The MRI was diagnostic in four cases of congenital encephalopathy complicated with neonatal suffering. CONCLUSION: Our study confirms the interest of the association of clinical settings, EEG tracings and MRI in the diagnosis and the prognostic of the hypoxic-ischemic encephalopathy in term neonate.

Activation of respiratory afferents by resistive loaded breathing modifies somatosensory evoked potentials to median nerve stimulation in humans.

The cortical projections of respiratory afferents (vagus and respiratory muscle nerves) are well documented in humans. It is also shown that their activation during loaded breathing modifies the perception of tactile sensation as well as the motor drive to skeletal muscles. The effects of expiratory or inspiratory loaded breathing on somatosensory evoked potentials (SEPs) elicited by median nerve stimulation were studied in eight healthy subjects. No significant changes occurred in latencies of N20, N30 and P40 throughout the expiratory loading period, except for a significant lengthening in P1 latency compared with unloaded breathing. However, inspiratory loading induced a significant increase in peak latency of N20, N30 and P40 components. We suggest that projections of inspiratory afferents from the diaphragm and the intercostal muscles, activated by inspiratory loading, could be responsible for the lengthened latency of median nerve SEP components. Thus, respiratory afferents very likely interact with pathways of the somatosensory system.

[Epileptic seizures and treatment with ifosfamide-mesna]. / Crises épileptiques et traitement par ifosfamide-mesna.

Generalized and partial seizures with secondary generalization were observed during ifosfamide-mesna (IFO) treatment in a patient with lung epidermoid carcinoma. Seizures appeared in a stereotyped manner on the 3rd of the 4th and 6th day of treatment with IFO. Partially resolutive confusion was observed after the last cure. Brain CTS were normal. The responsibility of IFO is considered in the development of these neurological toxic manifestations.

[Evoked potentials in HIV infection]. / Aspects des potentiels évoqués dans l'infection par le VIH.

The study of the literature data on the multimodal evoked potentials in HIV infected patients shows many abnormalities as well in asymptomatic subjects without AIDS as in AIDS subjects with or without neurological signs. Visual evoked potentials (VEPs) reveal prolonged P100 wave latency in 22% of HIV asymptomatic subjects and in 26% of HIV symptomatic subjects; brainstem auditory evoked potentials (BAEPs) reveal an increase of the interpeak latency I-V in 16% of asymptomatic subjects and in 32% of symptomatic subjects; somatosensory evoked potentials (SEPs) by median nerve stimulation reveal prolonged central conduction time in 6% of asymptomatic subjects and in 11% of symptomatic subjects; somatosensory evoked potentials (SEPs) by tibial nerve stimulation reveal prolonged central conduction time in 4% of asymptomatic subjects and in 45% of symptomatic subjects; motor evoked potentials (MEPs) by magnetic stimulation reveal prolonged central motor conduction time in 46% of asymptomatic subjects.

[Trigeminal neurotrophic ulcer and vascular disorders of the brain stem. A clinico-electrophysiological study]. / Ulcération neurotrophique trigéminée et accidents vasculaires du tronc cérébral. Etude clinique et électrophysiologique.

Torpid facial ulcerations may occur as a result of lesions involving the trigeminal fibers. These neurotrophic ulcerations have usually been observed after alcohol injection in the trigeminal ganglion. We report two cases associated with brainstem infarction. In both patients, the blink reflex was studied. The masseter inhibitory reflex was studied in one case. Analysis of these electrophysiological recordings can be of particular value in localizing the site of the lesion along the trigeminal pathway.

[Hemichorea-hemiballismus and toxoplasmosis in AIDS]. / Hémichorée-hémiballisme et toxoplasmose au cours du SIDA.

The authors report two cases of hemichorea-hemiballism revealing a toxoplasmic abscess in the subthalamic nucleus during AIDS. Despite the great frequency of this opportunistic infection such cases are exceptional. This rarity seems to be explained by the frequent association of other lesions of the basal ganglia which may prevent the movement disorders. Nevertheless an hemichorea-hemiballism in young people must suggest a cerebral toxoplasmosis. Finally a symptomatic treatment is often necessary; in these cases the use of sodium valproate must be considered, this drug being able to induce a clinical improvement.

Multimodal evoked potentials in HIV infected patients.

We studied 126 HIV seropositive patients (106 men, 20 women; mean age: 32 years): 45 had neurological signs, 81 had none. Multimodal evoked potential (MEP) assessment included: visual EPs by flash and reversal checkerboard; brain-stem auditory EPs; somatosensory EPs by stimulation of the median nerve. Evaluation also included: electroencephalography, electromyography with measurement of conduction velocities, neuroimaging (CT scan and MRI). We found abnormal MEPs for all modalities. The prevalence of abnormal results was high in neurologically symptomatic patients; in non-neurological subjects, the changes were more dramatic as HIV infection progressed. Whatever the stage of the disease, the modalities were equally affected. MEPs were abnormal in 54.7% of patients: in 41.8% of those without neurological signs vs. 85.7% of those with these signs. Comparison of MEPs and other electrophysiological procedures and neuroimaging techniques showed the high sensitivity of MEPs at all stages of the disease. EMG was sensitive and complementary to MEPs. EEG and neuroimaging showed abnormalities principally at the neurological symptomatic stage. Our results agree with those found in the literature. Abnormal MEPs may: (1) indicate latent neurological involvement of the visual, auditory and somatosensory pathways, (2) help diagnose an encephalitis suspected on neuropsychological, non-quantifiable testing.

[Multimodal evoked potentials in human immunodeficiency virus infection]. / Potentiels évoqués multimodaux dans l'infection par le virus de l'immunodéficience humaine.

We have studied 95 HIV seropositive patients (77 males and 18 females; mean age: 31 years): 67 had no neurological symptoms or signs, 28 had various neurological symptoms and signs. This study included a full multimodal evoked potentials (MEP) assessment: visual evoked potentials by flash and reversal checkerboard; brainstem auditory evoked potentials; somatosensory evoked potentials obtained by stimulation of the median nerve. Patient evaluation further included: electroencephalography, electromyography with measurement of conduction velocities and neuroimaging (brain CT scan and/or MRI). We found abnormal MEP for all modalities. The prevalence of abnormal results was high in neurological symptomatic patients; in non neurological ones, the changes tended to be more frequent with the progression of the HIV infection. Whatever the stage of the disease, the various modes were equally affected. MEP were abnormal in 54.7 p. 100 of the cases: in 41.8 p. 100 (28/67) of patient without neurological signs (in 4/12 of fully asymptomatic subjects, 11/34 ARC patients and 13/21 AIDS patients) vs 85.7 p. 100 of neurological symptomatic patients. In neurological asymptomatic patients, a similar proportion of abnormal MEP was found in asymptomatic and ARC patients, while the evolution into AIDS was associated with a higher prevalence of abnormal MEP. In the latter group, MEP changes were nearly as frequent as in neurological symptomatic patients. Comparison between MEP and other electrophysiological procedures (EEG, EMG) and with neuroimaging techniques (CT Scan, MRI) showed the high sensitivity of the MEP technique at all stages of the disease. EMG was a sensitive method and complementary to MEP. The EEG and neuroimaging techniques showed abnormalities principally at the neurological symptomatic stage. Previous studies could not be properly compared.(ABSTRACT TRUNCATED AT 250 WORDS)

Chronic progressive spinobulbar spasticity. A rare form of primary lateral sclerosis.

Although it was first described over a century ago (by Charcot in 1865; by Erb in 1875), the concept of primary lateral sclerosis (PLS) is still not universally accepted. Despite this skepticism, several well-documented cases of isolated degeneration with varying degrees of involvement of corticospinal pyramidal pathways have been reported in the literature. The clinical manifestations in these cases can take one of two forms, ie, isolated spasmodic paraplegia or tetraplegia on the one hand or spasmodic tetraplegia associated with a pseudobulbar syndrome featuring severe spastic dysarthria (chronic progressive bilateral spinobulbar spasticity) on the other hand. Obviously, without firm pathologic data, PLS is a hazardous diagnosis for isolated paraplegia or tetraplegia. Conversely, for bilateral spinobulbar spasticity, it would appear to be the only diagnosis possible once investigate findings have eliminated the other possibilities, such as a pyramidal form of amyotrophic lateral sclerosis or a spinal form of multiple sclerosis. To underscore this point, in this report, five cases of chronic progressive bilateral spinobulbar spasticity developed over 5, 10, 12, 10, and 28 years, respectively, for which the only possible diagnosis was PLS. It was concluded that there are three forms of degenerative diseases of the principal motor pathways: one involving both central and peripheral neurons, ie, amyotrophic lateral sclerosis; one involving only peripheral neurons, ie, spinal amyotrophy; and one involving only central motor neurons, ie, PLS.