[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.

[Subarachnoid hemorrhage induced by cerebral venous thrombosis]. / Thrombose veineuse cérébrale révélée par une hémorragie sous-arachnoïdienne.

Nontraumatic subarachnoid hemorrhage is a relatively rare disease, typically secondary to a ruptured aneurysm. We report the case of a 23-year-old patient who developed a subarachnoid hemorrhage caused by extensive cerebral venous thrombosis due to a factor V Leiden mutation. Cerebral venous thrombosis is an uncommon etiology of subarachnoid hemorrhage. This raises diagnostic difficulties and a therapeutic dilemma regarding the use of anticoagulants.

[Recurrent gelastic syncopes]. / Syncopes gélastiques récurrentes.

INTRODUCTION: Laughter-induced syncope or gelastic syncope is a rare and unrecognized phenomenon. We report an additional case. CASE REPORT: We report a 65-year-old man with no personal past medical history, particularly diabetes or heart disease, was admitted to investigate recent four episodes of loss of consciousness exclusively induced by laugh. The first episode had occurred 8 months earlier after reading a funny story. There were no other symptoms and physical examination, particularly neurological and cardiac was normal. All paraclinical investigations were also unremarkable: laboratory tests (glucose, thyroid function test and blood cobalamin level), cardiac and neurological investigations (electrocardiographic monitoring, echocardiography, electroencephalography and brain MRI). Treatment with propanolol prevented subsequent attacks. CONCLUSION: Sustained laugh is accompanied by repetitive bursts of forced expiration, equivalent to short repetition of Valsalva maneuvers. Laughter-induced syncope is thought to be a subtype of the vagal mediated syncopal attacks. Differential diagnosis should rule out especially gelastic atonic seizures and cataplexy. Propanolol is an effective therapy to prevent recurrence.

Chorea-ballismus in acute non-ketotic hyperglycaemia.

Diabetic patients during hyperglycaemic crises may present a rare syndrome characterised by a typical triad: unilateral involuntary movements (hemichoreahemiballism), radiological contralateral striatal abnormality, and rapid resolution of symptoms after glycae - mic correction. This study reports a series of patients showing less usual aspects and also discusses the pathophysiology of this clinical-radiological syndrome. We included in this study four patients presenting choreic or ballic involuntary movements and in whom aetiological assessment revealed frank non-ketotic hyperglycaemia, without other abnormalities that could explain the movement disorder. All the patients underwent CT or MR brain imaging. The typical triad was present in only one case. Less classical aspects were more frequently found: movement disorders revealed diabetes in two patients and one patient had generalised chorea and strictly normal neuroimaging. Correction of blood glucose was not sufficient to improve symptoms in two cases. In one, abnormal movements persisted despite treatment with tetrabenazine. The clinical, radiological and outcome spectrum of the syndrome of chorea-ballismus induced by non-ketotic hyperglycaemia is heterogeneous and not restricted to a typical triad.

[Painful legs and moving toes syndrome associated with lumbar radiculopathy]. / Syndrome de jambe douloureuse et orteils instables associé à une radiculopathie lombaire.

INTRODUCTION: Painful legs and moving toes (PLMT) is a rare syndrome characterized by spontaneous neuropathic pain in the lower limbs associated with peculiar involuntary movements of the toes. It has been associated with a variety of peripheral and central nervous system diseases. Pathophysiology is unclear and treatment approaches remain largely empirical. CLINICAL CASE: We report a case of a 42-year-old women with typical presentation of PLMT syndrome, associated with lumbar (L5) disc prolapse. Oxcarbazepine gave a partial improvement. CONCLUSION: Clinical presentations and etiological aspects of the PLMT syndrome are described and pathophysiological mechanisms and therapeutic possibilities discussed.

[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.

[l-arginine efficiency in MELAS syndrome. A case report]. / La l-arginine, une thérapeutique symptomatique efficace au cours d'un syndrome MELAS.

INTRODUCTION: Mitochondrial encephalomyopathy lactic acidosis and stoke-like episodes (MELAS) is a rare neurodegenerative disease caused by mutations of mitochondrial DNA. CASE REPORT: We report the case of a 12-year-old child with MELAS syndrome who presented with recurrent migraine-like headache and sudden blindness suggesting stroke-like episodes. Furthermore, he developed progressive muscular impairment with bilateral hearing loss. Serum lactate and pyruvate levels were elevated and the muscle biopsy showed an aspect of red-ragged fibers with Gomori trichrome. Brain imaging showed calcifications of basal ganglia on the CT scan and a parieto-occipital high signal on diffusion-weighted MRI. A genetic analysis was not performed but the presence of hearing loss in the patient's mother was suggestive of maternal transmission. Stroke-like episodes in the form of migraine-like headache and blindness were the patient's major complaint and did not improve despite analgesic drugs. After oral administration of l-arginine at the dose of 0.4mg/kg per day, stroke-like symptoms totally and rapidly disappeared. DISCUSSION: The efficiency of l-arginine in stroke-like episodes was initially reported then confirmed in a controlled study. The pathophysiology of stoke-like episodes and the mechanisms underlying the action of l-arginine are discussed.

[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.

[Severe apathy following head injury: improvement with Selegiline treatment]. / Apathie sévère après un traumatisme crânien: évolution favorable sous Sélégiline.

Apathy is defined as reduced goal-directed behavior due to lack of motivation. Traumatic brain injury is a frequent cause. Drugs activating the dopaminergic system provide variable benefit. A 30-year-old patient was the victim of a severe head injury with frontal bruise at the age of 15. At the request of his family, he consulted for a 7-year history that included a lack of initiative and the inability to generate behavior spontaneously, contrasting with the ability to execute behaviors on command. He also presented indifference, major emotional disruption without sadness, pessimism, and other depressive signs. The examination found a severe apathetic syndrome confirmed by specific scales with a mild impairment of executive functions and without depressive syndrome. Encephalic MRI showed atrophy of the whole prefrontal cerebral cortex. The patient was treated with bromocriptine, which he did not tolerate, then with Selegiline at 15 mg per day, which dramatically improved his symptoms. Apathy occurs frequently after traumatic brain injury, in 23-71% of patients according to the authors. The pathophysiology of apathy has been described in anatomical terms as related to disruption of frontal-subcortical pathways. The biochemical hypothesis postulates a disruption in dopaminergic activity. The use of dopaminergic agents usually improves cases similar to our patient. Apathy is frequent following head injury, warranting a search for systematic causes. Since it increases dopaminergic activity, Selegiline is well worth trying in these patients.

[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.

[Familial trigeminal neuralgia]. / Névralgie trigéminale familiale.

Trigeminal neuralgia in its classic form is usually an isolated disease that occurs in sporadic fashion, but familial cases have been described. We report the case of a 45-year-old man who presented with left V2 trigeminal neuralgia. The neurological examination was normal and imaging and laboratory investigations were non-contributive to the etiological work-up. Carbamazepine gave excellent symptomatic relief. During patient evaluation, we became aware of a clustering of trigeminal neuralgia in four other family members over three generations. Familial trigeminal neuralgia has been expounded on in fewer than 30 reports in the literature. Our cases and the literature review suggest an autosomal dominant transmission. The clinical features of familial trigeminal neuralgia are described and pathophysiological implications of this genetic clustering discussed.

[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.

[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.

[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.

[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.