[Guillain-Barré syndrome and anti-hepatitis A vaccination]. / Syndrome de Guillain-Barré et vaccination contre l'hépatite A.

The Guillain-Barré syndrome (GBS) is an acute inflammatory polyradiculoneuritis. The pathophysiology remains unknown but the existence of triggering factors such as external antigens is regularly suspected. We report the case of a 30-year-old patient with a past medical history of Graves disease, who presented with GBS within the month after receiving an anti-hepatitis A vaccination. GBS rarely happens after a hepatitis A vaccination. However, the responsibility of this vaccine should be considered in the clinical presentation of an acute polyradiculoneuritis.

[Anticonvulsant hypersensitivity syndrome and lamotrigine-associated anticonvulsant hypersensitivity syndrome]. / Syndrome d'hypersensibilité aux antiépileptiques. Cas particulier de la lamotrigine.

Anticonvulsant hypersensitivity syndrome (AHS) is defined by the association of high fever, cutaneous rash and multiorgan-system abnormalities (incidence, one in 1000 to one in 10,000 exposures). Fatal complications are described in 10%. This reaction usually develops 1 to 12 weeks after initiation of an aromatic anticonvulsant. Drug rash with eosinophilia and systemic symptoms (DRESS) can be discussed as differential diagnosis. Several hypotheses have been put forward to explain the pathogenesis of AHS. These include accumulation of toxic metabolites, antibody production and viral infection. The one based on toxic metabolites has found the greatest acceptance due to the fact that it can be proven by an in vitro test, the lymphocyte toxicity assay. In vivo, skin biopsies show characteristic findings of erythema multiform or typical leucocytoclastic angitis. The patch-test is positive in 80% of the cases. Lamotrigine-associated anticonvulsant hypersensitivity syndrome (LASH) is rare and was described in 1998. We report two new cases demonstrating the two particular configurations of apparition of LASH found in the 14 cases from the review of literature (Pubmed: anticonvulsant hypersensitivity syndrome - lamotrigine): high doses of lamotrigine (or lamotrigine in very young or old patients), and lamotrigine associated with another anti-epileptic (phenobarbital or sodium valproate). We discuss the links between DRESS after lamotrigine and LASH as illustrated in a new case.

[Idiopathic intracranial hypertension as a side effect of doxycycline]. / Hypertension intracrânienne bénigne secondaire à la prise de doxycycline.

Idiopathic intracranial hypertension is a rare disorder characterized by elevated intracranial pressure without hydrocephaly or intracranial process. Its mechanism is poorly understood. Most cases of benign intracranial hypertension are presumed to be idiopathic but some of them may be related to some treatment. We report a 26-year-old female with benign intracranial hypertension due to tetracycline, revealed by headaches and gradual visual loss. Standard investigations were unremarkable and favourable outcome after therapeutic lumbar puncture confirmed the diagnosis.

[Interferon-beta retinopathy]. / Rétinopathie liée à l'interféron-beta 1a sous-cutané

INTRODUCTION: Interferon-alpha associated retinopathy is an ocular complication of hepatitis C treatment well established in the literature. But, there are far fewer reports on multiple sclerosis related interferon-beta retinopathy. CASE REPORT: A 58-year-old male while receiving subcutaneous interferon-beta 1a 44microg thrice a week since 2001 for multiple sclerosis developed blurred vision. Visual acuity remained stable throughout the course of surveillance. Cotton wool spots were found on fundus exam. The retinopathy disappeared without specific therapy 2 months after discontinuing interferon injections. The diagnosis of interferon-beta 1a retinopathy was retained due to the lack of any other etiology. CONCLUSION: An ophthalmological examination including a fundus examination to search for a retinopathy should be undertaken when new ocular symptoms develop in a multiple sclerosis patient receiving interferon. An adverse event linked to interferon can be discussed and favored if the retinopathy resolves after interferon withdrawal.

[Neurological damage of brain tumor therapy]. / Complications neurologiques des traitements des tumeurs cérébrales.

Damage to the central nervous system induced by treatment of brain tumors is common and impairs the patient quality-of-life. Neurotoxicity is induced by synergistic effects of different cytotoxic treatments such as radiotherapy and chemotherapies administered concurrently or sequentially. Recent progress in the management of brain tumors has led to new neurotoxicities. The growing concern about the neuropsychological performance of patients has disclosed another type of brain damage which has been largely neglected to date. Neurological toxicity can be acute, requiring dose adaptation or a change of drugs. But it also often occurs late and can be irreversible. To date, treatments have been ineffective. The early diagnosis of neurotoxicity is thus a major challenge. Numerous clinical studies suggest an individual sensitivity which is not only related to age or vascular status, but also to genetic predisposition that remains to be detailed. Understanding the mechanisms of personal susceptibilities would be helpful in designing more tailored treatments. In this review we address the question of adverse effects of brain radiation as well as those of chemotherapy protocols which are particularly toxic for the central nervous system that is, methotrexate, platin and aracytin.

[Spontaneous low cerebospinal fluid pressure syndrome. A case report and literature review]. / Syndrome d'hypotension spontanée du liquide cérébrospinal. Revue de la littérature à propos d'un cas.

INTRODUCTION: Spontaneous low cerebrospinal fluid pressure syndrome is a spontaneous intracranial hypotension pressure due to a cerebrospinal fluid leak without any known dural effraction. It is clinically characterised by postural headaches relieved by supine position. We report a 38-year-old patient with this syndrome and review the literature. EXEGESIS: The diagnosis is sometimes difficult in atypical presentation of the syndrome and can lead to incapacitating chronic headache and rarely to complications. Cerebral magnetic resonance imaging has dramatically improved identification, diagnosis and management of this syndrome. Treatment is mainly based on blood patch realisation. Cerebrospinal fluid leak probably due to a spontaneous defect in the dural mater is suspected to be the main mechanism of this syndrome without any history of lumbar puncture or penetrating trauma. CONCLUSION: Early diagnosis, often easy on the basis of clinical characteristics of the headache may avoid complications.

[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.

[Eulenburg's paramyotonia congenita]. / Paramyotonie congénitale d'Eulenburg.

INTRODUCTION: Paramyotonia congenita is an autosomal dominant sodium channelopathy, caused by mutations in gene coding for muscle voltage-gated sodium channel alpha subunit. CASE REPORT: We report the case of a 38-year-old man who described since childhood muscle stiffness with attacks ok weakness induced by two provocative stimuli: cold exposure and exercise. It primarily concerned eyelids and hands, occasionally limbs. Family history suggested an autosomal dominant mode of transmission. Clinical examination revealed myotonia at the thenar eminence percussion. Generalized myotonic discharges were observed on electromyography. Molecular diagnosis reported an Arg1448Cys mutation in exon 24 in gene coding for muscle voltage-gated sodium channel alpha subunit (SCN4A) in chromosome 17. CONCLUSION: Paramyotonia congenita is not evolutive. Treatment is essentially preventive. Some medications could be proposed: membrane stabilizing agents like antiarrhythmic drugs (mexiletine, tocainide), or the carbonic anhydrase inhibitor (acetazolamide). Precautions may be taken during general anaesthesia because of diaphragm myotonia risk.

[Cerebral monitoring with somatosensory evoked potentials in carotid surgery. A review of 141 carotids]. / Monitorage cérébral par potentiels évoqués somesthésiques en chirurgie carotidienne. A propos d'une série de 141 carotides opérées.

OBJECTIVE: To evaluate postoperative and mid-term results of carotid surgery (CS) with somatosensory evoked potentials (SEP) monitoring. METHODS: Between 1998 and 2006, 141 CS in 124 patients were performed under general anesthesia. Selective shunting was based on SEP abnormality. Shunting criteria were: reduction up to 50% of the amplitude or latency increasing up to 10%. Early results and follow-up data are analyzed retrospectively. RESULTS: Shunting rate was 6%, 3 strokes (two transient strokes) occurred and one patient died of perioperative myocardial ischemia. The cumulative stroke and death rate at 30 days was 1.4%. CONCLUSIONS: Intra-operative SEP monitoring with selective shunting may be safely performed in carotid surgery.

[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.

[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".

[Intrinsic and co-intrinsic vowel characteristics in cerebellar and parkinsonian dysarthrias]. / Caractéristiques vocaliques intrinsèques et co-intrinsèques dans les dysarthries cérébelleuse et parkinsonienne.

Few studies were made on the intrinsic and co-intrinsic vowel characteristics in neurologic dysarthrias. This work evaluates these parameters in a group of 4 subjects with cerebellar dysarthria suffering from a cerebellar degenerative syndrome and a group of 4 subjects with Parkinson disease suffering from parkinsonian dysarthria. These subjects were compared with 10 control subjects. An intensive speech treatment inspired from the Lee Silverman voice treatment (LSVT) was applied to one of the parkinsonian subjects of the study, with assessment before and after rehabilitation. The acoustic measures assessed the duration and the frequency of the vowels [a, i, ul indifferent contexts as well as their formant frequency and sentence duration. The results show different impairments according to the type of the dysarthria, and some forms of contrast transposition. Speech therapy restores subnormal contrasts.

[Pulsating exophtalmos and contralateral carotid-cavernous fistula]. / Exophtalmie pulsatile contralatérale à une fistule carotido-caverneuse résolutive après traitement médical.

A rare case of spontaneous carotid-cavernous fistula with contralateral clinical signs (ocular discomfort, pulsating exophthalmos, chemosis, ptosis) is reported. It was associated with partial thrombosis of the ipsilateral cavernous sinus. It was cured with conservative treatment after six months. Anatomical knowledge of the cavernous sinus remains essential for better understanding of this original clinical presentation.

[Acute polyradiculoneuropathy after Chlamydia pneumoniae infection]. / Polyradiculonévrite aiguë secondaire à une infection à Chlamydia pneumoniae.

A 36-year-old woman presented acute polyradiculoneuropathy following Chlamydia pneumoniae infection. Although electrophysiologic studies were normal, clinical features were typical of Guillain-Barré syndrome (GBS). Anti-ganglioside GM1 antibodies were positive. Two other cases of GBS following Chlamydia pneumoniae infection have been reported, but no specific feature emerges. Outcome was good in our patient after intravenous globulin then antibiotic therapy. Our case supports the notion that Chlamydia pneumoniae infection can induce GBS. The association is probably underestimated.

[Radicular pain revealing Hodgkin's disease]. / Sciatalgie révélatrice d'une maladie de Hodgkin.

We report on a case of Hodgkin's disease, revealed in a 52 year-old woman by isolated neurological signs. Sciatica is an uncommon pattern of discovery that is particularly misleading. It may lead to a delayed diagnosis all the more prejudicial since it discloses an advanced stage of the disease. The neurological involvement is always associated with a bone lesion. This latter can be discovered at an early stage through magnetic resonance imaging.

[Acute transverse cervical myelitis following hepatitis B vaccination. Evolution of anti-HBs antibodies]. / Myélite transverse aiguë cervicale après vaccination contre le virus de l'hépatite B. Evolution sérologique des anticorps anti-HBs.

BACKGROUND: The cause and effect relationship between anti-HBV immunization using recombinant vaccine and the development of a neurological event, including flare-ups of multiple sclerosis, is a widely debated issue. CASE REPORT: A previously asymptomatic 16-year-old girl was a hyper-responder to anti-HBV vaccine. Subsequent to a booster shot of anti-HBV recombinant vaccine, she developed regressive acute cervical transverse myelitis with intrathecal oligochonal IgG secretion and a hypersignal on the MRI T2 sequences of the cord. DISCUSSION: The distinction between a first episode of multiple sclerosis or post-vaccinal acute myelitis in this case will depend upon subsequent course, but this observation points out the very high level of persistent post-vaccinal immunization which can be acquired by a hyper-responder.

[Mercury pulmonary embolism. Two case reports]. / Embolies pulmonaires mercuriques. A propos de deux observations.

Mercury pulmonary embolism following intentional or accidental injection of metallic mercury are uncommon. Generally, there are few clinical signs (acute pneumopathy in 50% of the cases). Chest x-ray shows multiple and bilateral point opacities with a metallic density. We present two cases of mercury pulmonary embolism after intentional intravenous mercury injection (one attempted suicide and one HIV+ drug addict).

[Pleuropneumopathy caused by bromocriptine in a patient with Parkinson disease. Review of the literature apropos of a new case]. / Pleuropneumopathie à la bromocriptine chez un parkinsonien. Revue de la littérature à propos d'une nouvelle observation.

Bromocriptine, a derivate of ergot of rye, is employed in high doses for the treatment of Parkinson's disease, and may induct pleuropulmonary affections. We report the case of a 75-years-old patient, on bromocriptin for 5 years, who presented a progressive dyspnea, due to a pleural thickening, and moderate interstitial infiltrate, associated with an inflammatory syndrome. The outcome was favorable with 3 months of bromocriptin cessation. These complications are rarely described, probably underestimated, and justify a respiratory follow-up for these patients, who are difficult to stabilize in their neurologic treatment.