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We report the case of a 55-year-old man without significant medical history admitted to the ICU for a progressive paralysis mimicking life-threatening tetanus. Evolution with classical tetanus treatment was negative, with the need for ventilator support and worsening condition being life threatening. Uncommon evolution revealed a rare glycin antibody-associated hyperekplexia (progressive encephalomyelitis with rigidity syndrome). Patient dramatically improved with immunosuppressive therapy including plasmatic exchanges, cyclophasmid and high dose corticoid infusions. Intensivists should be aware of this very rare syndrome whose treatment is the opposite of tetanus while presentation is very close. Optimal and treatment could lead to prolonged survival.

Pernicious anemia is uncommon in Africa. The purpose of this report is to describe a case of pernicious anemia observed in Madagascar. The revealing manifestation was encephalomyelitis with combined medullar sclerosis that responded favorably to vitamin B12 replacement therapy. Clinical symptoms included paresthesia associated with allodynia of all four extremities and with tetrapyramidal syndrome, medullar ataxia and minor cognitive disturbances ongoing for 5 months. Hemogram testing revealed macrocytic anemia. Serum cobalamin level was low. Anti-intrinsic factor antibody was detected. Spinal cord magnetic resonance imaging showed diffuse high-signal intensity along the posterior spinal cord extending from C1 to C4. Vitamin B12 replacement therapy led to full regression of clinical signs after six weeks. Association of central nervous system involvement with macrocytic anemia suggests vitamin B12 deficiency and pernicious anemia should be suspected. This disease can be considered as a curable form of myelitis in Africa and Madagascar.

Neurological deficit in experimental autoimmune encephalomyelitis (EAE) and multiple sclerosis is widely considered to be a consequence of synergistic T and B cell responses to central nervous system (CNS) antigens. We show that mice immunized with encephalitogenic myelin oligodendrocyte glycoprotein (MOG(35-55)) peptide develop significant serum levels of anti-MOG antibodies in parallel with disease progression. Furthermore, EAE mice developed antibodies against DNA and RNA, a serological hallmark observed in autoimmune diseases such as systemic lupus erythematosus. The presence of anti-nucleic responsive B cells and antibodies during EAE may highlight a previously unappreciated mechanism in the pathogenesis of CNS autoimmunity.

A 20-year old man was hospitalized for acute urinary retention, headaches and mild fever. Neurological examination revealed a meningeal syndrome and a bilateral pyramidal syndrome of lower extremities with a C7 sensory level. The level of antibodies against Mycoplasma pneumoniae (Igm and IgG) suggested a recent unrecognized infection. Cerebral and medullar MRI showed T2-hyperintense lesions in the right thalamus and at the level of C1-C2 and C6. Evoked potentials were normal but EEG showed some bilateral theta waves. The cerebrospinal fluid analysis demonstrated 170 white cells/mm3 with 66% lymphocytes and proteins at 73 mg/dl. A few weeks after treatment with intravenous immunoglobulins at 0.4 g/kg/day for 5 days, the patient showed complete clinical recovery. MRI and urinary flowrate normalized after nine weeks. The authors are discussing the probable diagnosis of acute disseminated encephalomyelitis occurring after a mycoplasma unrecognized infection.

Sensory ganglionopathies have a frequent association with neoplastic disorders (paraneoplastic subacute sensory neuronopathy, or SSN) or dysimmune disorders, with drugs, such as cisplatin or pyridoxine, and with inherited disorders with degeneration of dorsal root ganglion cells. Unsteady gait and pseudoathetoid movements of the hand are the distinctive signs encountered in these disorders. The chronic disorders are characterized by non-length-dependent abnormalities of sensory nerve action potentials (SNAPs) and differ from other sensory neuropathies in showing a global, rather than distal, decrease in SNAP amplitudes. This review focuses on recent advances in defining the mechanisms involved in sensory ganglionopathies, and describes the differential diagnosis including the rarely encountered hereditary neuronopathies and the infectious causes.

The neurological and psychiatric manifestations of Borrelia burgdorferi sensu lato are so numerous that Borrelia is also called the "new great imitator". Thus knowing about the multiple clinical aspects of neuroborreliosis is necessary for the clinician. We reviewed literature for "classical" neuroborreliosis such as acute meningoradiculitis or chronicle encephalomyelitis, but also for encephalitis, myelitis, polyneuritis, radiculitis and more controversial disorders such as chronic neurological disorders, ischemic and hemorrhagic stroke, and motor neuron disease. We specified every time on which basis each disorder was attributed to Lyme disease, particularly if European or American criteria were met. Every part of the nervous system can be involved: from central to peripheral nervous system, and even muscles. In endemic areas, Lyme serology must be assessed in case of unexplained neurological or psychiatric disorder. In case of positive serology, CSF assessment with intrathecal anti-Borrelia antibody index will be more efficient to prove the diagnosis.

If meningoencephalitis with or without mass lesion (granuloma or abscess) is the most common pattern of CNS cryptococcal infection, intramedullary involvement is very uncommon.The authors report an 70-year-old male with Hodgkin's disease treated by chemotherapy then corticosteroids because of pulmonary fibrosis who was presenting for eight days ago, an ataxia, pyramidal syndrome, and bradypsychy. Spinal MRI revealed a gadolinium T1 weighted homogeneous enhancing T4 level intramedullary lesion. CSF had showed 190 GB/mm3 of lymphomonocytes, increased protein level (2.28 g/l), decreased glucose level (1.5 mmol/l) and positivity for crytococcal antigen. Treatment by amphotericine B and flucytosine then fluconazole for six months was instituted and symptoms gradually improved.A cryptococcus infection must be searched by antigen in CSF in case of myelopathy isolated or associated with meningoradiculoencephalomyelopathy, specially in patients with a cellular immunodeficience. Antimycotic agents must be firstly used, surgery would be restricted to decompression if aggravation of disease and compressive effect on the adjacent structures radiologically (MRI) became evident. Prolonged treatment is necessary in case of immunodeficience.

We describe the case of a patient with a particular form of presumably immune-mediated encephalomyelitis associated with a monoclonal cold agglutin gammapathy. Systematic autopsy showed predominantly demyelinating lesions of the brain and spinal cord. The lesions were assumed to be the immune-mediated consequences of the underlying hematologic condition. Similarity with certain paraneoplastic syndromes is underlined.

Avian encephalomyelitis cases have been detected in chicks (future laying hens) in November 1997. It was the first time that the disease was diagnosed in Senegal. Typical lesions of the disease were revealed during the histological diagnosis and confirmed its presence. Vaccination against encephalomyelitis was not mandatory and some hatcheries did not vaccinate their breeding animals. The presence of the disease seems to require from now on a mandatory vaccination of all breeding animals in Senegal. In addition, there is the problem of vaccinating pullets, i.e. future laying hens. Actually, the egg drop syndrome is currently affecting the country, and the laboratory must first differentiate between encephalomyelitis, Newcastle disease and infectious bronchitis before making precise recommendations. In this respect, the ELISA technique will have to be used. Avian encephalomyelitis cases have been detected in chicks (future laying hens) in November 1997. It was the first time that the disease was diagnosed in Senegal. Typical lesions of the disease were revealed during the histological diagnosis and confirmed its presence. Vaccination against encephalomyelitis was not mandatory and some hatcheries did not vaccinate their breeding animals. The presence of the disease seems to require from now on a mandatory vaccination of all breeding animals in Senegal. In addition, there is the problem of vaccinating pullets, i.e. future laying hens. Actually, the egg drop syndrome is currently affecting the country, and the laboratory must first differentiate between encephalomyelitis, Newcastle disease and infectious bronchitis before making precise recommendations. In this respect, the ELISA technique will have to be used. Normal 0 21 false false false FR X-NONE X-NONE /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Tableau Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-parent:""; mso-padding-alt:0cm 5.4pt 0cm 5.4pt; mso-para-margin-top:0cm; mso-para-margin-right:0cm; mso-para-margin-bottom:10.0pt; mso-para-margin-left:0cm; line-height:115%; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri",sans-serif; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-fareast-language:EN-US;} table.MsoTableGrid {mso-style-name:"Grille du tableau"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-unhide:no; border:solid black 1.0pt; mso-border-alt:solid black .5pt; mso-padding-alt:0cm 5.4pt 0cm 5.4pt; mso-border-insideh:.5pt solid black; mso-border-insidev:.5pt solid black; mso-para-margin:0cm; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:10.0pt; font-family:"Times New Roman",serif;}

Eosinophilic meningitis caused by Angiostrongylus cantonensis is widespread in Southeast Asia and Pacific islands. Adults develop a transient meningitis with a benign course but severe or fatal disease may occur in pediatric patients.Case 1. A 11-month-old boy living in Mayotte island was hospitalized a few days with fever and skin rash following by seizure, coma, flaccid quadraplegia, absence of deep tendon reflexes, urinary retention and anal incontinence. Eosinophilia was observed in peripheral blood and cerebrospinal fluid. He further developed a triventricular hydrocephalus treated by ventriculoperitoneal shunt. The child died 3 weeks later. A serodiagnosis of angiostrongylus infestation was restrospectively established. Case reports 2 and 3.-Two infants, 10 and 11-month-old-boys, living in Reunion island, developed fever and vomitings, irritability and, for one of them, unilateral sixth cranial nerve palsy. There was eosinophilia in the peripheral blood and in the cerebrospinal fluid. All symptoms progressively disappeared with complete recovery. The suspected diagnosis of angiostrongylus infestation was confirmed by the serology.We report the first case of Angiostrongylus cantonensis infection in the French island of Mayone (Comoro Islands) and we confirm the presence of this disease in Reunion island. In this Indian Ocean area, eosinophilic meningitis occurs most of the time in infants with sometimes severe radiculomyeloencephalitic forms.

We report the case of a 62-year-old man affected by anti-Hu-associated paraneoplastic encephalomyelitis. The underlying tumor was a neuroendocrine cancer of the rectum expressing Hu antigen. The neurologic presentation was limited to moderate sensitive neuropathy associated with two complex partial seizures (dreamy state) without any further signs of limbic encephalopathy. A paraneoplastic etiology should be considered in patients with moderate symptomatology. Paraneoplastic encephalomyelitis with anti-Hu antibodies is not always associated with small-cell lung cancer.

Eosinophilic meningitis caused by Angiostrongylus cantonensis is widespread in Southeast Asia and Pacific islands. Adults develop a transient meningitis with a benign course but severe or fatal disease may occur in pediatric patients. CASE REPORT 1: A 11 months old boy living in Mayotte island was hospitalized some days after fever and skin rash with seizure status, coma, flaccid quadriplegia, absence of deep tendon reflexes, urinary retention and anal incontinence. Eosinophilia was observed in peripheral blood and in the cerebrospinal fluid. Secondary, he developed a triventricular hydrocephalus treated by a ventriculoperitoneal shunt. After 3 weeks, the child died. Retrospectively, the diagnosis of angiostrongylus infection was established with the help of serology. CASE REPORTS 2 AND 3: Two infants, 10 and 11 months old boys, living in Reunion island, developed fever and vomitings, irritability and, for one of them, a unilateral sixth cranial nerve palsy. There was eosinophilia in the peripheral blood and in the cerebrospinal fluid. All symptoms progressively disappeared with complete recovery. The suspected diagnosis of angiostrongylus infection was confirmed by the serology.We report the first case of Angiostrongylus cantonensis infection in the French island of Mayotte (Comoro Islands) and we confirm the presence of this disease in Reunion island. In this Indian Ocean area, eosinophilic meningitis occurs most of the time in infants with sometimes severe radiculomyeloencephalitic forms. The origin of these occasionally massive infections is the giant African snail Achatina fulica. For a child with meningitis living in Reunion or Mayotte, or coming back from these 2 islands, Angiostrongylus cantonensis infection must be evoked, especially if there is a blood eosinophilia. Since efficiency of antiparasitic treatment is nowadays not proved, information must be given to people living in exposed areas in view to limit incidence of this disease.

Neurological lesions are frequent complications of human immunodeficiency virus (HIV) infections. Organs involved include the brain, peripheral nerves and muscles. Since the widespread use of immunodepressive agents, spinal cord complications have also appeared although poorly documented in the literature. We observed six cases of spinal cord involvement which help indicate the modalities of practical management. In the first case, a 45-year old HIV1 + male presented dysesthesia evolving progressively over the T10 to L2 zones leading to the diagnosis of spinal cord toxoplasmosis. A gait disorder was the first sign in the second case, a 60-year old HIV1 + male. Neurological involvement progressed and the patient developed paraparesia, decreased muscular force with hypoesthesia and impaired proprioception of the lower limbs. Further complications led to coma and death and on autopsy, the patient was found to have cytomegalovirus myeloencephalitis. A 21 HIV1 + haemophiliac was our third case. Here paraplegia resulted from epidural compression due to Burkitt malignant lymphocytosis. The aggravation of paresthesia of the lower limbs, complicated by painful dysesthesia and proximal motor deficiency led to the suspected diagnosis of HIV-related myelitis in a particularly complicated case in a 52-year old seropositive male. In the fifth case, HIV infection led to major demelinization of the cervical and dorsal spinal cord due to toxoplasmosis and vacuolar myelopathy. In the sixth case, acute myelitis in an HIV2 positive male regressed spontaneously in 15 days. In clinical practice, spinal cord complications would appear to be frequent but less so than brain involvement. In the future, a better understanding of these complications should lead to specific identification of spinal cord signs in the neurological symptomatology of patients with HIV infection and allow adapted specific management.

In the last decade, anti-neurons autoantibodies have been found in serum and cerebro spinal fluid of patients suffering from neurological paraneoplastic syndrome. This discovery has made possible to improve the knowledge of these syndromes as well as to characterize some proteins specific to the nervous system, unknown until now. Paraneoplastic encephalomyelitis can manifest together with an ataxic sensitive neuropathy, a limbic encephalitis, a brainstem or cerebellum syndrome. This encephalomyelitis is almost always associated with a small cells lung cancer and auto-antibodies, called anti-Hu, which recognize all the neurons of the nervous system. Patients with paraneoplastic cerebellar degeneration present an antibody, called anti-Yo, directed against Purkinje cells. In this case, the tumor is a gynecologic cancer. Patients presenting with an opso-myoclonus and a breast cancer have an antineurons anti-body, called anti-Ri, which is absent when the opso-myoclonus is associated with a lung cancer or a neuroblastoma. These three antibodies are the most frequently found and the best studied, but others, rarer, have been described. The genes coding for the proteins recognized by these three antibodies have been cloned. These proteins seem to play a major role in the neuronal maturation and homeostasis. These antibodies prove to be irreplaceable tools to study the phenomenons subtending the neuronal degeneration and the cellular proliferation.

A generalized epileptic seizure revealed a subactute encephalopathy which was attributed to Hashimoto's thyroiditis. Primary biliary cirrhosis was also discovered. The encephalopathy rapidly followed a favourable course under corticosteroid therapy, which confirms that it was an immune disease.

The authors report the clinico-pathological study of an encephalomyelitis with necrotic cavitation of the white matter occurring in a 61-year-old female. The disease of sudden onset progressed slowly to the death, 20 months after the onset of the first symptoms. They stress the rarity of the entity and the difficulties to establish the diagnosis clinically. The nosological situation of the condition is discussed and compared with the previously reported cases.

Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating condition of the central nervous system, usually developing after a viral infection or vaccination. We report a case of ADEM predominantly affecting the spinal cord in an 8-year old boy evaluated by MRI. The radiographic picture consisted of multiple focal lesions of the spinal cord, a left posterior thalamic lesion and a subcortical right posterior parietal lesion. These lesions regressed several weeks after corticosteroid treatment. The clinical presentation, the laboratory results and the radiological findings suggest the diagnosis of ADEM secondary to viral infection by Coxsackie B.

Antibodies directed against the central nervous system were looked for by indirect immunohistochemistry in the sera of 8 patients with paraneoplastic neurological syndrome (group 1), 21 cancer patients without neurological signs, 23 patients with miscellaneous neurological diseases and 63 normal subjects (groups 2 to 4). Four patients in group 1 had very high titres of antibodies. In 2 patients with small-cell lung carcinoma associated with sensory neuropathy the antibody recognized the cytoplasm and nucleus of all neurons. A 37 Kd protein was recognized by Western blot. A woman with cancer of the ovary and cerebellar syndrome exhibited an antibody against Purkinje's cell cytoplasm with a band of about 50-55 Kd at Western blot. In a woman with chronic uveitis and cerebellar atrophy with disappearance of Purkinje's cells the antibody (in blood and CSF) recognized certain layers of the retina as well as glial cells and cells present in the subependymal areas of the brain. Two bands of 46 and 59 Kd were revealed by Western blot. Immunoglobulins were detected in the cytoplasm of white matter cells in the cerebellum and brain stem. Among the other groups, one patient with lung cancer had a moderate titre of neuronal antinuclear antibody. The Western blot test was negative. The relevance of these antibodies for the diagnosis and treatment is discussed.

We report the case of a 29 year-old man who died from rabies in France, following a dog-bite during a trip in Mexico. Although it was clinically suspected, the diagnosis was uncertain until he died because of digestive, cardiac and psychiatric misleading symptoms associated to the neurologic disorders. Post mortem diagnosis was based upon virological study in immunofluorescence on cerebral smears, viral isolation on cell-culture, and ELISA. It was confirmed by light microscopy examination which showed numerous Negri bodies, and ultrastructural study of the rhabdovirus in the central nervous system. Extranervous lesions, especially myocarditis and pancreatitis, were observed and their meaning is discussed. The physician is exceptionally confronted to the diagnosis of human rabies in France. Nevertheless, the lack of compulsory antirabic vaccination and the increase of touring in enzootic countries increase the risk of infection. As an intra vitam diagnosis in frequently lacking, the diagnosis of rabies infection needs a complete post mortem virological study as well as an histological and ultrastructural examination of the central nervous system.

A 70 year-old woman presented with a progressive supranuclear ophthalmoplegia, with "apraxia" of eyelid opening, axial akinesia and dementia. CT scan showed a mild cortico-subcortical atrophy and there was a high level of immunoglobulins, with an oligoclonal pattern, without cell reaction in the CSF. The patient died two years after the onset. Post-mortem examination, limited to CNS, showed subacute encephalitis confined to the tectal, pretectal, subthalamic areas and to Ammon's horns. These changes and their location were strongly suggestive of polioencephalomyelitis with or without cancer, in which such a prevalent midbrain involvement has been exceptionally described.

A case of isolated central nervous system involvement in Lyme disease is described. A 13 year-old boy developed progressive spastic quadraparesis, chronic lymphocytic meningitis with a low CSF glucose concentration and demyelinating lesions of the white matter on MRI. The diagnosis was proved serologically by high antibody titers against Borrelia burgdorferi (BB) in the serum (1:5, 120) and CSF (1:1,280). There was evidence of specific intrathecal immune response against the BB antigen. The patient was treated with penicillin G and then ceftriaxone. The CSF abnormalities quickly improved but improvement of the neurologic symptoms was gradual and to date still incomplete.

A 68 year-old woman contracted West Nile fever after a stay of one month in Israel. Mild encephalitis and severe myelitis, resembling the "polio syndrome", developed, with important sequelae. Specific antibodies against West Nile fever virus progressively increased in the patient's serum. Epidemiological and clinical data about neurologic aspects of this infection are reviewed. Meningoencephalitis is not unusual, but only one previous case of acute anterior myelitis has been described in humans although this pathology is well known in experimental and veterinary diseases. Very few studies concern interferon and arbovirus infections in humans. Interferon assays in our patient's serum and cerebrospinal fluid showed its presence and persistence: this is in relation with the replication of the virus in the central nervous system, as it has been demonstrated in other primary viral encephalitis.

A 38-year-old woman with encephalitis presented no signs of infectious disease or exanthema. After a remission lasting 10 months, she died during a second attack after demonstrating signs of brain stem lesions. Histological examination revealed lesions, mainly in the brain stem and characterized by intense necrosis, moderate degrees of inflammation, and localized foci of gliosis. In spite of the necrotic lesions, the neurones were relatively spared. An old infarct of the left caudate nucleus was present. Taking other demyelinating diseases into account, it is suggested that this is a clinical form of multiple sclerosis, this enabling a relationship to be established between multiple sclerosis and post-infections perivenous encephalitis or acute disseminated encephalomyelitis.

We report two patients with chronic encephalomyelitis due to Borrelia burgdorferi in whom the definite diagnosis was delayed because of atypical clinical features. The first patient presented with chronic spastic paraparesis, slight ataxia and nystagmus of several years' duration. A tentative diagnosis of multiple sclerosis was made in spite of important abnormalities of the CSF biological characteristics. The second patient presented with an acute aphasia and a bilateral Babinski's sign. He was thought to suffer from benign herpetic meningoencephalitis. Several months later, as the patient experienced relapses with cerebellar and spinal cord involvement, falsely positive tests for syphilis were found and an antibiotic treatment was given. High protein content, low glucose levels, pleocytosis and oligoclonal bands were observed in all CSF samples, but the definite diagnosis was based on the detection of serum and CSF antibodies against B. burgdorferi.

Within the group of acute encephalitis, H.A.H.L. is individualized mostly by its post mortem pathology. But several cases of recovery were recently published, the diagnostic being based on biopsy material. In 4 of our 8 cases, a comparison between the biopsy and post mortem examinations was possible : in/the diagnostic of H.A.H.L. was unequivocally confirmed, in 2 H.A.H.L. is nearly certain, in 1 it is doubtful. These considerations do not contradict the necessity of an emergency brain decompression in many instances of acute encephalitic states.

Diffuse and chronic muscular pains, without any noticeable underlying anomaly, are a frequent cause for consultation. The origin of the pain was, at the beginning of the century, attributed to an inflammation of fascia, tendons, ligaments, subcutaneous tissues and even the muscle itself. Although this inflammatory etiology is not based on proven anatomical facts, some authors advocate an organic pathology of unknown origin and the terms of fibrositis or primary fibromuscular pain are used in the literature, especially the anglo-saxon literature, to describe such symptomatology. Clinical diagnostic criteria have been recently proposed in order to differentiate primary fibromuscular pain from functional diffuse muscular pains. The authors discuss the legitimacy of such distinction.

Dominant symptoms in 24 patients with neurological manifestations of Behçet's disease were meningo-encephalitis (13 cases), febrile meningeal syndrome (3 cases), intracranial hypertension (6 cases), polyneuritis of the lower limbs (1 case), and isolated retrobulbar ocular neuritis (1 case). Several classical notions concerning the "Neuro-Behçet" meningo-encephalitis were confirmed: the frequency of central motor lesions (33 p. cent), cerebrospinal fluid alterations (anomalies in 100 p. 100 of cases in the initial stage), and the often unfavorable course (40 p. cent). An unusual finding in this series was the frequency of cerebral veins thrombosis (4 certain and 2 probable). These could be differentiated from meningo-encephalitis by their symptomatology, predominantly that of intracranial hypertension, and by their course: often the first disorder to appear in the course of Behçet's disease, they followed a favorable neurological course but were constantly complicated by other vascular manifestations, usually phlebitis. It is suggested that a long-term anticoagulant treatment should be associated with corticoid therapy in these forms.