Devic-like syndrome in the course of pulmonary tuberculosis.

INTRODUCTION: Optic neuromyelitis or Devic's syndrome is a very rare disease affecting the optic tracts and the spinal cord. Its association with evolving pulmonary tuberculosis has been reported in a handful of case reports. CASE REPORT: The authors report two cases of Devic's syndrome associated pulmonary tuberculosis (48 and 43 years old men). The First patient was experiencing evolving pulmonary tuberculosis. The two patients were admitted for bilateral blindness followed by paraplegia and sphincter disturbance. Clinical examination and investigations excluded direct tuberculous involvement of the nervous system or a reaction to antituberculous therapy and Devic's syndrome was diagnosed, based on Wingerchurk's criteria. Following treatment with corticosteroids and antituberculous chemotherapy, we noted partial recovery of motor symptoms and sphincter control but the patients remained completely blind. CONCLUSIONS: Throughout this case report, the authors emphasize the rarity of this association and discuss the pathophysiological mechanism, which appears to be an immune dysfunction triggered by mycobacterium infection.

[Methylprednisolone pulse in treatment of childhood chronic inflammatory demyelinating polyneuropathy]. / Place des bolus de méthylprednisolone dans le traitement des polyradiculonévrites chroniques de l'enfant.

Chronic inflammatory demyelinating polyneuropathy (CIDP) in children is rare and treatment is based primarily on intravenous immunoglobulins or oral corticosteroids. Boluses of methylprednisolone (MP) are a possible alternative. We report 3 cases of CIDP in children with good outcome after MP pulse therapy. One male (7 years of age) and 2 females (4 and 5 years of age) presented with recurring episodes of functional impotence of both lower limbs and walking impairment, partially reversible without treatment. Clinical and electrophysiological data and the analysis of the cerebrospinal fluid were compatible with CIDP. MP pulses were administered: the total number of pulses varied from 5 to 8, very satisfactory progression on the clinical and electrophysiological pattern was noted, without recurrence in the 3 cases. Childhood CIDP presents clinical, electrophysiological outcome, and prognostic particularities, recurring readily, and the outcome is good. Boluses of MP are an alternative for treatment of these neuropathies in childhood.

[Cobalamin deficiency: neurological aspects in 27 cases]. / Carences en cobalamine: aspects neurologiques chez 27 patients.

INTRODUCTION: Combined medullar sclerosis, together with peripheral sensory neuropathies, is the most common neurological manifestation observed in cobalamin deficiency. Biermer's disease is the predominant cause. Other clinical and etiological aspects are nevertheless frequent, although underestimated. METHODS: This retrospective study included patients with neurological symptoms and cobalamin (B12 vitamin) deficiency confirmed by laboratory tests collected over a period of 11 years. RESULTS: Twenty-seven cases were analyzed. Mean age was 47 years and there were 11 women and 16 men. Distribution of the neurological syndromes was: combined medullar sclerosis in 18 patients (67%), sensory neuropathies in 30% of cases and sensory-motor neuropathies in 15%. One patient had fronto-subcortical dementia with good improvement after vitamin replacement. In addition, autonomic dysfunction was noted in six patients (orthostatic symptomatic hypotension and/or urinary dysfunction and/or erectile failure). Dysautonomia revealed cobalamin deficiency in three patients with a good and fast response to the cobalamin therapy in all cases. Biermer's disease was diagnosed in 17 patients (63%) and a likely syndrome of nondissociation of cobalamin in two patients. One patient had Crohn's disease and no etiology was found in seven patients. In five patients (19%), nitrous oxide undoubtedly induced decompensation of latent cobalamin deficiency; four after a general anesthesia and one by chronic professional exposure. Outcome was very good in 46% of patients after vitamin replacement, particularly if treatment was started rapidly. DISCUSSION: The findings in this series highlight the frequency of autonomic dysfunction sometimes revealing cobalamin deficiency with a fast and good response to vitamin replacement and the frequency of neurological disorders following decompensation triggered by general anesthesia using nitrous oxide in patients with latent cobalamin deficiency.

[Cauda equina syndrome revealing neuroblastoma]. / Syndrome de la queue de cheval révélant un neuroblastome.

INTRODUCTION: Neuroblastoma is the most common solid tumor of childhood. Neurological involvement is rare resulting from cord or nerve compression. CASE REPORT: A 7-year-old child was hospitalized for cauda equina syndrome. MRI showed retroperitoneal lesion with dumbbell intradural spreading. Blood and urinary catecholamine level were elevated confirming the diagnosis of neuroblastoma. She was treated with chemotherapy alone with partial neurological recovery. DISCUSSION AND CONCLUSION: Cauda equina compression revealing neuroblastoma is exceptional especially at an early phase in the youngest patients. Treatment is based on surgical resection and/or chemotherapy and/or radiation therapy. Long-term prognosis is sometimes poor with neurological sequels.

[POEMS syndrome with plasmocytoma lytic bone lesion]. / POEMS syndrome avec plasmocytome ostéolytique.

INTRODUCTION: Crow-Fukase or Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin changes syndrome (POEMS) is a rare multisystemic affection with incompletely elucidated etiopathogenesis. CASE REPORT: We report a case of POEMS syndrome in a 48-year-old adult revealed four months before admission by areflexic flask tetraparesis prevalent on the lower limbs in connection with demyelinating and axonal CIDP "like" sensoriomotor neuropathy of the four limbs electroneuromyographically. The patient presented elevated protein level in the CSF with monoclonal standard IgG gammapathy associated with a narrow band lambda, suggesting POEMS syndrome. Further explorations revealed skin lesions with glomeruloid angiomas, edematous vasomotor disorders as well as erythrocyanose, hypogonadism, papillar edema and a lytic bone lesion of the left scapula. Radiotherapy was associated with corticosteroids and plasma exchanges. Outcome was good with resolution of the symptoms and stabilization of the neuropathy. DISCUSSION: POEMS syndrome is rare; the diagnosis is based on necessary criteria, the presence of a demyelinating and axonal polyneuropathy associated with an IgA or IgG monoclonal gammapathy, the light chain being almost entirely lambda, associated to other characteristic elements, in particular glomeruloid angiomas, endocrinopathy, sclerosing plasmocytoma which must be carefully required. Treatment is based on surgical cure or radiotherapy for bone lesion and non specific treatments such as corticosteroid therapy, plasma exchanges and IVIG.

[Subacute myelopathy revealing systemic sarcoidosis]. / Myélopathies subaiguës révélatrices de sarcoïdose systémique.

INTRODUCTION: The spinal localization is rare for neurosarcoidosis (0.43 percent of cases) but can be the inaugural manifestation of the disease. We report two cases of spinal neurosarcoidosis in a 57-year-old man and a 43-year*old woman with uneventful past medical histories. Both presented progressive myelopathic features. METHODS: Magnetic resonance imaging (MRI) of the spine demonstrated intramedullary lesions, dorsal in the first case, and cervical in the second case. Serum angiotensin converting enzyme was elevated. Radiographs of the chest revealed bilateral symmetric hilar mediastinal lymphadenopathy in the first patient, and bronchial biopsy demonstrated non caseating granulomas. In the second patient the diagnosis was made on pathological examination of a minor salivary gland biopsy. RESULTS: The patients received corticosteroid therapy with good response in the second patient. CONCLUSION: The diagnosis of intramedullary sarcoidosis is difficult without a previous diagnosis of systemic sarcoidosis or other apparent symptom(s). Extraneurologic biopsies may be suggestive. We reviewed the literature on the diagnosis and treatment of intramedullary sarcoidosis.

[Peripheral neuropathy in systemic lupus erythematosus with epineural vasculitis and antiphospholipid antibodies]. / Neuropathie périphérique au cours du lupus érythémateux disséminé avec vascularite épineurale et anticorps antiphospholipides.

Neurological manifestations of systemic lupus erythematosus are frequent and polymorphic. Their frequency varies according to authors (24-75p.cent). Central nervous system complications predominate; peripheral features are rare, classically symmetrical polyneuropathy, multiple mononeuropathies or cranial nerve involvement. We report a case of a 48-year-old woman presenting a histologically documented sensitivo-motor polyneuropathy with severe motor involvement complicating lupus associated with antiphospholipides antibodies. Outcome was good after cyclophosphamid pulse. We discuss the frequency of peripheral involvement in systemic lupus erythematosus, pathogenic mechanisms, therapeutic possibilities and outcome of this complication.

[Reccurent stroke revealing catastrophic antiphospholipid syndrome with hepatitis C viral infection]. / Accidents vasculaires cérébraux révélant un syndrome catastrophique des anti-phospholipides associé à une hépatite virale C.

INTRODUCTION: Very little cases of antiphospholipid syndrome (APLS) have been described among patients having chronic hepatitis C virus infection (HCV). CASE REPORT: We report the observation of a 43-year-old woman who presented APLS diagnosed following recurrent strokes. Etiological investigations concluded on an association of APLS with HCV infection. DISCUSSION: Besides being exceptional, this association raises certain etiopathogenic problems. Indeed while antiphospholipid antibodies (aPL) are frequently noted during chronic hepatitis C, they rarely generate thromboembolic complications.

[The presentation of viral hepatitis C infection without cryoglobulinemia with a peripheral neuropathy. Five case reports]. / Neuropathies périphériques sans cryoglobulinémie révélatrices de l'hépatite virale C. A propos de cinq observations.

INTRODUCTION: Peripheral neuropathies are the most common neurological complication of viral hepatitis C infection with mixed cryoglobulinemia. CASES REPORT: We report five cases (three men, two women) of peripheral neuropathies revealing viral hepatitis C infection without cryoglobulinemia; the patients' mean age was 56 years. Paresthesias were the most frequent symptom. Electroneuromyographic examination found one case of polyneuropathy and four cases of multiplex mononeuropathies; the complement level was normal in all patients and the rheumatoid factor positive in two cases. Etiological investigations for peripheral neuropathy remained negative. Treatment and outcome were variable. DISCUSSION: Negative cryoglobulinemia in cases of VHC infection with neurological features has been described in the last few years, suggesting the possibility of other mechanisms such as direct action of the virus on the nervous system. There is no consensus on the treatment and outcome is variable. CONCLUSION: Peripheral neuropathy may reveal VHC infection, underscoring the need for VHC serology testing in etiological investigations for peripheral neuropathies.

[Devic's neuromyelitis optica in Morocco. A study of 9-cases]. / La neuromyélite optique au Maroc. Etude de neuf cas.

INTRODUCTION: Optic neuromyelitis or Devic's syndrome associates optic neuritis and myelitis. It can have a monophasic or relapsing course. The limits of this entity remain controversial. METHODS: We analyzed the results of a retrospective series of Moroccan patients with Devic's syndrome defined on the basis of Wingerchuk's criteria. RESULTS: Six women and three men were retained for study; average age was 39 years. Three patients presented with a monophasic form and six with relapsing disease. CSF analysis was abnormal in five. Brain MRI was normal in eight. Signal abnormalities extended over more than three vertebrae in eight patients. Infections were associated with neurological features in half of the patients. DISCUSSION: Our results are in agreement with those of large series in the literature regarding the clinical, imaging, and laboratory findings and disease course. We also noted that infections were more frequently associated with Devic's syndrome in our patients. CONCLUSION: Our study reports the results of the first North African series of Devic's syndrome patients. We suggest that this syndrome is particular in our context due to the high frequency and diversity of associated infections.

[Aseptic meningoencephalitis in primary Gougerot-Sjögren's syndrome]. / Méningoencéphalite aseptique au cours d'un syndrome de Gougerot-Sjögren primitif.

INTRODUCTION: Central nervous system involvement is rarely observed in primary Sjogren's syndrome (PSS) and aseptic meningoencephalitis occurs exceptionally. OBSERVATION: We report the case of a 25-year-old woman who presented with weakness of the four limbs, vision loss and concentration deficit. Neurological examination revealed a mental confusion, cerebellar involvement and spastic tetra paresis. Cerebrospinal fluid analysis revealed elevation of cell counts (162/mm3) and protein content (3 g/l). MRI showed increased T2 signals in the white matter. No evidence for an infectious etiology, especially tuberculosis, was obtained in this patient. PSS was diagnosed on the basis of ocular dryness, lachrymal hyposecretion, aspects of labial gland biopsy witch revealed destruction of the ducts with periductal lymphocytic infiltration and positive anti-SSB antibodies. Oral corticosteroids (1 mg/kg/day) allowed an improvement of the neurological manifestations. CONCLUSION: Aseptic meningoencephalitis can be the first manifestation of primary Sjogren's syndrome. Infectious causes, particularly tuberculosis which is frequent in our context must be ruled out.

[Devic's optic neuromyelitis and pulmonary tuberculosis]. / Neuromyélite optique de Devic associée à une tuberculose pulmonaire.

INTRODUCTION: Optic neuromyelitis or Devic's syndrome is a very rare disease affecting the optic tracts and the spinal cord. Its association with evolving pulmonary tuberculosis has been noted in a handful of case reports. CASE REPORT: The authors reportthe case of a 40 year old patient with evolving pulmonary tuberculosis who experienced bilateral blindness followed by paraplegia and sphincter disturbance. Clinical examination and investigations excluded direct tuberculous involvement of the nervous system or a reaction to antituberculous therapy and Devic's syndrome was diagnosed, based on Wingerchurk's criteria. Following treatment with corticosteroids and antituberculous chemotherapy, the patient recovered normal mobility and sphincter control but remained completely blind. CONCLUSIONS: Throughout this case report, the authors emphasise the rarity of this association and discuss the pathophysiological mechanism, which appears to be an immune dysfunction triggered by mycobacterial infection.