[Primary Sjögren's syndrome and neuromyelitis optica]. / Syndrome de Goujerot-Sjögren primitif et neuromyélite optique de Devic.

Neuromyelitis optica (NMO) is characterized by the association of optic neuritis and myelitis without any other neurological signs. In 30 % of the cases, NMO is associated with a systemic disorders. We report two cases of NMO associated with primary Sjögren's syndrome that was diagnosed during an acute flare of NMO. The relationship between NMO and Sjögren's syndrome has not been clearly identified. This association likely reflects the coexistence of two autoimmune disorders. The prognosis of NMO appears to be more severe in patients having coexisting Sjögren's syndrome, which renders useful to search for this association in patients with neuromyelitis optica.

[Celiac disease and ischemic stroke]. / Maladie coeliaque et accidents vasculaires cérébraux ischémiques.

INTRODUCTION: Neurological manifestations of celiac disease are various. An association with ischemic stroke is not common and has not been well documented. We report two cases. OBSERVATIONS: The first patient had experienced several transient ischemic strokes in the past 2 years and then had an acute ischemic stroke involving the territory of the right posterior cerebral artery. Investigations revealed celiac disease with no other recognizable etiology. The clinical course was marked by persistent visual aftereffects, but no new vascular event. The second patient had been followed since 1998 for celiac disease confirmed by pathology and serology tests. She was on a gluten-free diet. The patient had an ischemic stroke involving the territory of the left middle cerebral artery. Apart from a positive serology for celiac disease and iron deficiency anemia, the etiological work-up was negative. DISCUSSION: The mechanisms of vascular involvement in celiac disease are controversial. The most widely incriminated factor is autoimmune central nervous system vasculitis, in which tissue transglutaminase, the main auto-antigen contributing to maintaining the integrity of endothelium tissue, plays a major role. Other mechanisms are still debated, mainly vitamin deficiency. CONCLUSION: Being a potentially treatable cause of ischemic stroke, celiac disease must be considered as a potential etiology of stroke of unknown cause, particularly in young patients, and even without gastrointestinal manifestations.

[Neurological manifestations of chronic inflammatory bowel disease]. / Manifestations neurologiques des maladies inflammatoires chroniques de l'intestin.

INTRODUCTION: Several extra-intestinal manifestations of inflammatory bowel disease have been reported, although reports of neurologic disorders are rare. CASE REPORTS: We report two cases of peripheral and central neurological manifestations of inflammatory bowel disease. The first case is a 33-year-old woman who developed acute inflammatory demyelinating motor polyneuropathy in association with Crohn's disease. Her symptoms improved with immunomodulatory therapies. Our second case is a 46-year-old woman, previously diagnosed with ulcerative colitis that subsequently developed ischemic stroke concomitantly to severe exacerbation of digestive symptoms. Her radial and humeral pulses were unequal bilaterally. Associated Takayashu's arteritis was suspected. The cardiovascular and immunological assessments were negative. Complementary investigations were foreseen but the patient died early of acute abundant rectal bleeding. CONCLUSION: This paper focuses essentially on the probable manifestations of inflammatory bowel disease in the nervous system, particularly peripheral neuropathies and stroke. We discuss different pathophysiologic mechanisms incriminated, notably thromboembolism and immunological abnormalities.

[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.

[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.

[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.

[Symptomatic periodic paralysis secondary to primary Sjogren's syndrome]. / Paralysie périodique symptomatique d'un syndrome de Gougerot-Sjögren primitif.

INTRODUCTION: Hypokalaemic periodic paralysis can be primitive or secondary to potassium deficiency which can arise from several causes. Primary Sjogren's syndrome is a rare cause related to kidney involvement. CASE REPORT: A 50-year-old woman has been admitted for hypotonic tetraparesis which had appeared a few days earlier. History taking revealed three previous similar episodes with a notion of oral and lacrimal dryness. Laboratory tests revealed severe hypokalaemia, hyperchloremia, alkaline urinary pH and a minima 24h proteinuria. Additional investigations led to the diagnosis of a primary Sjogren's syndrome defined on the basis of international criteria. Kidney biopsy revealed tubular-interstitial nephritis. Oral corticosteroïd therapy and potassium supplementation led to symptom improvement. A recurrent episode also responded to treatment. Additional urinary alkalinisation has prevented further relapse. DISCUSSION: Primary Sjogren's syndrome is an exocrine disease causing systemic disorders. Tubular-interstitial nephropathy may occur in 25 percent of patients leading to distal tubular acidosis defined by the association of hypokalaemia, hyperchloremia and alkaline urinary pH. When hypokalaemia is severe, periodic paralysis may occur. CONCLUSION: Primary Sjogren's syndrome can lead to nephropathy and subsequent hypokalaemic periodic paralysis. Urinary alkalinisation is essential to prevent this catastrophic presentation from recurring.