[Striatopallidodentate calcinosis, hypoparathyroidism and neurological features: a case series study]. / Calcinose striatopallidodentelée, hypoparathyroïdie et atteinte neurologique: étude de série.

INTRODUCTION: The respective roles of hypocalcemia and intracerebral calcifications in the occurrence of various neurological manifestations in hypoparathyroidism is not entirely clear. Nevertheless, therapeutic and prognostic implications are important. OBJECTIVES: We analyze the neurological clinical aspects observed in hypoparathyroidism and correlate them to the biological calcium abnormality and radiological CT scan findings. We also compare these results with data reported in the idiopathic form of striatopallidodentate calcinosis. PATIENTS: The neurological clinical, CT scan findings and outcome have been retrospectively studied in patients recruited during 13 years (2000-2012) for neurological features associated with hypoparathyroidism or pseudohypoparathyroidism. RESULTS: Twelve patients with primary hypoparathyroidism (n=5), secondary to thyroidectomy (n=4) and pseudohypoparathyroidism (n=3) were studied. The sex-ratio was 1 and mean age was 39 years. All patients had a tetany, 60% had epilepsy, associated in one patient with "benign" intracranial hypertension; 50% had behavioral changes. Response to calcium therapy was excellent for all these events. Moderate cognitive deficit was noted in three patients (25%), parkinsonism in two patients and hyperkinetic movement disorders in one other. These events were not responsive to calcium therapy and were more common in cases of extensive brain calcifications and in patients who had pseudohypoparathroidism. COMMENTS: This study suggests that, in patients with hypoparathyroidism, epilepsy and psychiatric disorders are induced by hypocalcemia and reversible after its correction. Cognitive and extrapyramidal impairment seem to be related to the progressive extension of intracerebral calcification, particularly in patients with a late diagnosis. In patients with pseudohypoparathyroidism, this finding is different because of the contribution of other factors, specific to this disease.

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.

[Sjögren-Larsson syndrome: a novel mutation in a Moroccan child]. / Syndrome de Sjögren-Larsson en rapport avec une nouvelle mutation chez un enfant marocain.

Sjögren-Larsson syndrome is a very rare inherited neurocutaneous disorder caused by a deficiency of microsomal enzyme fatty aldehyde dehydrogenase (FALDH). The authors report a case of typical Sjögren-Larsson syndrome in a 7-year-old Moroccan child who presented with classical symptoms (congenital ichthyosis, mental retardation, and spastic paraparesis) and epilepsy. The genetic study revealed a new mutation in the FALDH gene mapped to chromosome 17, consisting in a G109A substitution in exon 2.

[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.

[Acute disseminated encephalomyelitis associated with hepatitis A virus infection]. / Encéphalomyélite aiguë disséminée associée à une infection au virus de l'hépatite virale A chez l'adulte.

INTRODUCTION: Hepatitis virus A (HVA) infection is usually a benign infection, but it can lead to severe manifestations and neurological symptoms. CASE REPORT: We report the case of a 44-year-old man who was admitted for pyramidal tetraparesis, loss of proprioceptive sensitivity and cranial nerve involvement. He had developed concomitally jaundice and fatigue. Brain MRI and cerebrospinal fluid examination were normal. Blood tests revealed elevated serum transaminase and anti-hepatitis A virus (IgM and IgG) levels. Acute disseminated encephalomyelitis (ADEM) was diagnosed and the patient was treated with high dose intravenous then oral corticosteroid therapy. The clinical condition continued to deteriorate and the patient died at eight months. DISCUSSION: ADEM is exceptionally associated with HVA infection or after vaccination for hepatitis A. Other neurological complications, including either peripheral or central nervous system, are reported. The clinical presentation and the outcome of our patient are atypical.

[Manic-depressive psychosis as prevalent manifestation of multiple sclerosis]. / La psychose maniacodépressive comme manifestation prévalente d'une sclérose en plaques.

INTRODUCTION: Manic-depressive psychosis (MDP) and multiple sclerosis (MS) coexistence is unusual but well-proven. OBSERVATIONS: We report two cases observed in two women aged 30 and 31, who were followed up for neurological episodes associated with concomitant or deferred manic or depressive fits. Brain magnetic resonance imaging revealed multiple zones of high intensity signals in the white matter. Biological balance was normal. The diagnosis of multiple sclerosis (MS) was established. Given to treat acute episodes, high-dose corticosteroids enabled regression of the psychological fits. Similarly, long-term treatment in one patient enabled significant regression of fits, which became less frequent and less severe. DISCUSSION/CONCLUSION: The MDP-MS association may be due to local MS-related brain damage or to common genetic susceptibility. The positive effect of corticosteroids against psychological fits is another finding favouring an organic cause of these disorders.

[Sturge--Weber-Klippel--Trenaunay syndrome (case report)]. / Syndrome de Sturge-Weber--Klippel-Trenaunay (une observation).

INTRODUCTION: Sturge-Weber syndrome is characterized by capillary malformations in the face, associated with leptomeningeal and choroidal venous malformations. Klippel-Trenaunay syndrome consists of the triad: capillary malformation of one leg, ipsilateral hypertrophy and varicose veins. OBSERVATION: We report the case of a 23-year-old male patient who presented a complex congenital neurocutaneous syndrome with vascular malformations involving the brain, face and limbs and associated with epilepsia and leptomeningeal calcifications. The patient fulfilled the diagnostic criteria of both vascular phacomatosis syndromes. DISCUSSION AND CONCLUSION: Similar descriptions of dual or overlapping syndromes have been published. Many show that there is no clear distinction between Klippel-Trenaunay syndrome and Sturge-Weber syndrome. There is a clinical and biological overlap. The complexity of the disease phenotypes shows that a classification based on an eponymous category does not enable resolution of the nosological problems. Some authors suggest that these vascular malformations are best described in anatomical/histological or functional terms. We report a new observation that illustrates these difficulties.

[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.

[Electrophysiological aspects of intermediate syndrome in organophosphate poisonings]. / Aspects électrophysiologiques du syndrome intermédiaire au cours des intoxications aux organophosphorés.

Acute organophosphate poisoning, whether accidental or suicidal, is frequent in developing countries and produces significant morbidity and mortality. Organophosphates inhibit cholinesterase activity at the neuromuscular junction and cause extensive muscle paralysis, particularly for respiratory function. Poisonings evolve in three stages: cholinesterase crisis, intermediate syndrome and delayed neuropathy. Electrophysiological aspects have been poorly studied. We report the case of a 25-year-old woman admitted to intensive care unit with muscarinic signs and respiratory failure after attempted suicidal organophosphate poisoning. Cholinesterase activity was low and the electrophysiological study disclosed the characteristic aspect of intermediate syndrome. The patient died due to septic complications. This syndrome is exceptional with a poorly understood pathophysiology. The electrophysiological study is essential for diagnosis.

[Clinical and electrophysiological aspects of acute organophosphate intoxication]. / Aspects cliniques et électrophysiologiques de l'intoxication aiguë aux organophosphorés.

INTRODUCTION: Acute organophosphate (OP) intoxications, accidental or voluntary, are responsible for a high mortality. They cause extensive muscular paralysis by acetyl cholinesterase activity inhibition at the neuromuscular junction level. AIM: To underline the rarity and the characteristic electrophysiological pattern during cholinergic crisis. OBSERVATION: A 28-year-old woman was admitted to the medical intensive care unit for Malathion acute intoxication with signs of glandular hypersecretion, complicated tetraparesis, and respiratory distress. The cholinesterase activity was 17%. The electroneuromyography showed multiple motor responses to the same stimulation, which is characteristic of the cholinergic crisis. Other electrophysiological parameters, in particular low-frequency repetitive stimulations, were normal. The evolution was favourable after symptomatic treatment and respiratory assistance. DISCUSSION AND CONCLUSIONS: Organophosphate intoxications evolve in three phases: acute cholinergic crisis, intermediate syndrome, and delayed neuropathy. While the electrophysiological aspects of delayed neuropathy are best characterized, those of crisis and intermediate syndrome remain very little studied. The persistence of acetylcholine in the synaptic slit would explain the multiple motor responses to single stimulation during the crisis.

[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.

[Parry-Romberg syndrome with dysphonia]. / Syndrome de Parry-Romberg avec dysphonie.

INTRODUCTION: Parry-Romberg syndrome is a clinical entity consisting of progressive hemifacial atrophy appearing at a young age. This syndrome has features of linear scleroderma 'en coup de sabre'. The aetiology of this affection is poorly understood. A number of neurologic disorders associations have been reported particularly seizures. CASE REPORT: We report case of Parry Romberg syndrome in 29 years old men associated with dysphonia. Neurological examination and Brain CT scan were normal. DISCUSSION AND CONCLUSION: Different mechanisms have been discussed, immunological disorders, sympathic dysfunction or infectious origin. Management consists on reconstructive surgery, symptomatic treatment, with psychological issues.

[Chronic inflammatory demyelinating polyradiculoneuropathy in childhood: outcomes after methylprednisolone pulse therapy]. / Polyradiculonévrite chronique idiopathique de l'enfant: évolution sous bolus de méthylprednisolone.

INTRODUCTION: Chronic inflammatory demyelinating polyneuropathy (CIDP) in children is relatively rare and treatment is based primarily on intravenous immunoglobulins or oral corticosteroids. Boluses of methylprednisolone (MP) are a seldom used alternative. CASE REPORT: We report the case of an 8-year-old child, first presented at the age of 3 years, with recurring episodes of functional impotence of both lower limbs and walking impairment, partially reversible without treatment. Clinical, progressive, and electrophysiological data and the analysis of the cerebrospinal fluid were compatible with CIDP. MP boluses were administered: after a total eight monthly boluses, very satisfactory progression on the clinical and electrophysiological fronts was noted after 24 months. CONCLUSION: Childhood CIDP presents clinical, electrophysiological, progressive, and prognostic particularities, they recur readily and the outcome is good. Boluses of methylprednisolone are an alternative to the treatment of these neuropathies in childhood.

[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.