[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.

[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.

[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.

[Crohn's disease presenting with recurrent acute polyradiculoneuropathy]. / Polyradiculonévrite à rechutes révélant une maladie de Crohn.

INTRODUCTION: The neurological manifestations of Crohn's disease are rare, dominated by multiple mononeuropathies and the abnormalities of the white matter. Polyradiculoneurities remain exceptional. OBSERVATIONS: We report the case of a 33-year-old patient admitted for an ascending weakness of all four limbs. Eight years earlier he had presented a similar episode which had regressed spontaneously. The neurological examination revealed a tetraparesis with areflexia and hypotonia. These manifestations were concomitant with chronic diarrhea which had been neglected to date. The electrophysiological aspect was compatible with an acute polyradiculoneuritis. The analysis of the cerebrospinal fluid showed an albumino-cytological dissociation. The existence of the diarrhea directed the investigations towards an inflammatory enteropathy, which was attested later on by the endoscopic, radiologic and histological data leading to the diagnosis of active Crohn's disease. The diagnosis of a relapsing polyradiculoneuritis associated with Crohn's disease was retained. The patient was treated by salazopyrine-budesonide with improvement in the digestive and neurological manifestations after 3 years. CONCLUSION: The frequency of neurological features in Crohn's disease is not well documented. The incriminated mechanisms are either directly related to the disease (deficit in B12 vitamin or folic acid and/or by the means of an auto-immune vascularitis) or secondary to long-term treatment with metronidazole. The course of neurological manifestations is largely dependent on the course of the inflammatory disease.

[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.

[Postoperative dementia: toxicity of nitrous oxide]. / Démence postopératoire: toxicité du protoxyde d'azote.

INTRODUCTION: Post-operative neuropsychiatric manifestations represent a frequent situation and may be due to several aetiologies. The responsibility of vitamin B12 deficiency must be evoked, especially in case of anaesthesia with a currently used substance: nitrous oxide. CASE REPORT: A 65 year-old man with no medical history, presented problems walking and memory loss 16 days after surgery for femoral prosthesis. Neurological examination revealed paraplegia with syndrome of combined degeneration of the spinal cord. The exploration of cognitive functions showed disorientation in time with memory disorders and disturbance of executive functioning. There was no apraxia, aphasia or agnosia. There were neither psychotic symptoms nor mood changes. MMS was at 18/30. Red blood count revealed an anaemia with macrocytosis (MGV=120 3). Vitamin B12 rate was very low (less than 30 g/l). Folate blood level was normal. Brain MRI showed moderate cerebral atrophy. Other investigations led to the diagnosis of Biermer's disease (fundic atrophy at biopsy with presence in the serum of antibodies to intrinsic factor). The diagnosis of neurological attack related to a vitamin B12 deficiency secondary to Biermer's disease was established, but the appearance of disorders in the post-operative period suggested the existence of an added factor. The recovery of informations revealed that anaesthesia was maintained by nitrous oxide during two hours and the patient exhibited pre-operative anaemia with macrocytosis. The hypothesis of decompensation of latent vitamin B12 deficiency by nitrous oxide was evoked. Replacement therapy by vitamin B12 induced real improvement of the cognitive impairment. MMS increased to 25/30. DISCUSSION: Cognitive impairment due to vitamin B12 deficiency is rarely dominated by isolated memory disorders. An authentic dementia is exceptional. Our patient had a dementia diagnosed on the basis of DSM IV criteria including memory disorders, disturbance of executive functioning and significant impairment in social and occupational functioning, associated with a combined degeneration of the spinal cord, common in vitamin B12 deficiency. Furthermore, he had an unknown Biermer's disease responsible for pre-operative deficiency which was clinically latent (there was only macrocytosis anaemia). The appearance of problems in the post-operative period was due to an acute decompensation of the latent deficiency induced undoubtedly by nitrous oxide used in anaesthesia. According to Christensen, nitrous oxide causes irreversible oxidation of vitamin B12 cobalt's atom responsible for its inactivation and the appearance of clinical manifestations. Evolution under vitamin B12 replacement therapy depends on the rapidity of its founding. In our case, it led to an improvement, notably in cognitive functions. CONCLUSION: Through this observation, the authors underline the necessity to search for vitamin B12 deficiency in the case of cognitive features following general anaesthesia.

[Bilateral idiopathic orbital myositis]. / Myosite orbitaire idiopathique bilatérale.

INTRODUCTION: Orbital myositis is an idiopathic inflammation of the extraoccular muscles. It is often included under the broad description of orbital pseudotumor. It is commonly a unilateral affection. CASE REPORT: We report one case of bilateral orbital myositis in a 45-year-old woman. The first symptoms were diplopia and acute bilateral orbital pain exacerbated by eye movements. Physical examination especially neurological was normal. Serum creatine kinase and biological thyroid function were normal. CT scan found a bilateral enlargement of rectus oculi medialis and lateralis. Clinical improvement was obtained a few days after oral corticosteroid treatment with remission of all signs. CONCLUSION: Orbital myositis is a rare entity. 90-95p.cent of cases are unilateral. Bilateral forms are exceptional and in this case require search for specific etiologies particularly thyroid ophthalmopathy. There is controversy concerning treatment options but corticosteroids are still the most common first choice therapy with good outcome.

[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.

[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.

[Systemic lupus erythematosus presenting with a choreo-athetosic syndrome associated with antiphospholipid antibodies]. / Syndrome choréo-athétosique révélant une maladie lupique associée à des anticorps antiphospholipides.

INTRODUCTION: Chorea is a rare manifestation of systemic lupus erythematosus (1-4 percent), commonly affecting young woman. Chorea is revealing lupic disease in 50 percent, in the other cases it occurs early in the course of the disease. OBSERVATION: A 33-year-old woman was hospitalized for choreo-athetosic movements prevailing on the left leg and arm accompanied by behavioral and general state deterioration. The biological assessment consolidated the diagnosis of lupic disease associated antiphospholipides antibody (aPL). Cerebral magnetic resonance imaging (MRI) was normal except for cortical and subcortical atrophy. The patient was treated by corticosteroids (1mg/kg/day) and then was lost to follow-up. CONCLUSION: We review data in the literature on the pathophysiological mechanisms of lupic chorea focusing particularly on role of aPL.

[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 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.

[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.

[Severe neonatal myasthenia with arthrogryposis]. / Myasthénie néonatale sévère avec arthrogrypose: à propos d'un cas et revue de la littérature.

Maternal myasthenia gravis has been associated with the presence of neonatal myasthania and sometimes fetal congenital anomalies. The purpose of this paper is to present an infant with multiple deformations born to a mother with myasthenia gravis. The infant presented with arthrogryposis multiplex and pulmonary hypoplasia. The new born died within the first day of life. Twenty-seven other cases of neonatal myasthenia with arthrogryposis have been reported. Twenty-two of them were stillborn or died. The surviving children needed ventilatory assistance for a long period.

[Fahr syndrome associated to hypoparathyroidy, revealed by dermatologic manifestation]. / Syndrome de Fahr secondaire à une hypoparathyroïdie à révélation dermatologique.

BACKGROUND: Fahr syndrome is defined by the presence at the brain base of strio-pallido-notched non-arteriosclerotic bilateral and symmetric calcifications. We report an observation of Fahr syndrome revealed by a growing pyodermitis in direct relation with hypocalcemia. OBSERVATION: A 17 year-old girl was admitted to the Dermatology Department for a growing pyodermitis of the large folds. The patient was known to be epileptic and had undergone surgery for a left eye cataract a year before. Microbiologic and mycologic examinations of the pus and of the cutaneous fragments were negative. During hospitalization, the patient had an attack of tetany, a laryngeal spasm and a generalized erythema thrust studded with pustules. The phosphocalcic assessment showed severe hypocalcemia and hyperphosphoremia. Parathormone serum rate was decreased. The brain tomodensitometry conveyed bilateral calcifications of the central gray nucleus. The diagnosis of a growing amicrobial pustulosis secondary to an idiopathic hypoparathyroidism and responsible for the Fahr syndrome was maintained. Treatment was composed of calcium (2 g/day) and vitamin D3. The evolution was favorable with a relapse at 2 years. DISCUSSION: We report an idiopathic hypoparathyroidism associated with Fahr syndrome conveyed by growing amicrobial pustulosis. We discuss the dermatologic manifestations of Fahr syndrome associated with hypoparathyroidism and the physiopathologic mechanisms of the dermatologic lesions during hypocalcemia.