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.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.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.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.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.

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. (C) 2013 Elsevier Masson SAS. All rights reserved.

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 (1312 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. (C) 2008 Elsevier Masson SAS.

Journal de Gynecologie Obstetrique et Biologie de la Reproduction - Vol. 32 - N° 7 - p. 660-662

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.

La vascularite nécrosante restreinte au système nerveux périphérique est une entité nosologique rare définit par une atteinte localisée au système nerveux périphérique sans signes systémiques. Nous rapportons l’observation d’un patient de 65 ans qui a présenté un déficit progressif flasque aréflexique des quatre membres avec atteinte des nerfs crâniens. L’électroneuromyogramme a conclu à une polyneuropathie sensitivomotrice démyélinisante. L’étude du LCR montrait une discrète hyperprotéinorachie. La biopsie du nerf sural objectivait un infiltrat inflammatoire polymorphe avec nécrose fibrinoïde. Le bilan d’extension et le bilan immunologique (notamment les ANCA) ont été négatifs. La corticothérapie orale a été sans effet. Le patient est décédé peu de temps après l’introduction des immunosuppresseurs. Les atteintes nerveuses périphériques au cours des vascularites nécrosantes sont retrouvées dans 50–75 % des cas. Elles sont représentées essentiellement par les mononeuropathies multiples de nature axonale et peuvent être révélatrices. Des cas de polyneuropathies démyélinisantes avec parfois des blocs de conduction ont été également rapportés. Cette association pose des problèmes d’ordre physiopathologique. Non systemic vasculitis neuropathy (NSVN) is a rare type of vasculitic neuropathy that evolves without manifestations of vasculitis in other organs and in the absence of serological abnormalities. We report the case of a 65-year-old man with demyelinating sensory motor polyneuropathy presented as distal and proximal weakness of the four limbs with paresthesia, areflexia and cranial nerve involvement. Sural nerve biopsy revealed marked loss of myelinated fibbers and vascular fibrinoid necrosis with polymorphic inflammatory cell infiltrate. The cerebrospinal fluid analysis showed raised protein (0.60 g/l) with normal cell counts. Both the extension and immunological investigations were normal. Oral corticosteroids have been started with no improvement. Lesions of the peripheral nervous system are observed in 50–75% of cases of necrotizing vasculites. Usually, they consist in axonal multiple mononeuritis but some cases of demyelinating polyneuropathy with conduction blocks have been reported. These findings pose pathophysiology problems.

Nous rapportons l’observation d’un patient ayant une maladie de Biermer méconnue qui a présenté, au 15e jour après une intervention chirurgicale, une atteinte neurologique associant un syndrome démentiel et un syndrome de sclérose combinée de la moelle. Les manifestations neurologiques étaient secondaires à une inactivation aiguë de la vitamine B12 vraisemblablement induite par l’anesthésie générale utilisant le protoxyde d’azote. L’évolution sous vitaminothérapie B12 était très favorable notamment sur les manifestations cognitives. À travers cette observation, les auteurs soulignent la nécessité d’évoquer un déficit en vitamine B 12 devant des manifestations cognitives survenant après une anesthésie générale.

La coexistence sclérose en plaques (SEP) et psychose maniaco-dépressive (PMD) est inhabituelle mais établie. Les liens de causalité restent très discutés. Trois patientes âgées respectivement de 25 ans, 31 ans et 53 ans, suivies pour SEP établie, ont présenté au cours de l'évolution des poussées maniaques et/ou dépressives. Dans un cas, les poussées de la SEP étaient essentiellement psychiatriques, dans les deux autres cas, elles alternaient avec les poussées neurologiques. L'évolution des manifestations psychiatriques était favorable chez les trois patientes sous psychotropes associés aux bolus de corticoïdes, Le traitement de fond à base de Bétaferon chez une patiente, et de cyclophosphamide chez une autre a permis de réduire significativement la fréquence et la sévérité de ces poussées. La PMD est plus fréquente dans la SEP que dans la population générale. Sa survenue est variablement expliquée : manifestation de certaines localisations lésionnelles notamment frontales, susceptibilité génétique commune aux deux affections. Contrairement à ce qui se passe dans la PMD primitive, les poussées psychiatriques répondent mieux si on associe des bolus de corticoïdes, élément en faveur de l'organicité. Les troubles de la personnalité sont classiques dans la SEP, la PMD l'est moins, néanmoins elle n'est pas exceptionnelle. Sa connaissance permet d'adapter le traitement et de l'entreprendre précocement.

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.

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.

Avec l'allongement de l'esperance de vie, le praticien se trouve de plus en plus confronte aux pathologies du sujet âge. L'accident vasculaire cerebral ischemique (AVCI) en constitue une des plus frequentes, aux consequences parfois dramatiques. Dans cette mise au point, les auteurs proposent une conduite a tenir globale face a l'AVCI, en abordant toutes ses facettes : cliniques, paracliniques, therapeutiques et pronostiques.

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.

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.

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.

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.