Paget's disease of bone presenting with multiple cranial nerve palsies: A case report.

Paget's disease of bone (PDB) is a progressive monostotic or polyostotic osteopathy with unknown cause. It is associated with the involvement of the nervous system. The cranial nerves, spinal roots, cauda equina, spinal cord, and brain can be affected in PDB due to their close anatomical relation to bone. Hearing loss occurs in 12%-50% of patients with PDB. The optic nerve can be affected at the optic canal. The diagnosis of PDB is radiological by highlighting characteristic lesions like thickening of the cortical bone, hypertrophic and fibrillary bones. Progressive or chronic neurological deficits should be treated with bisphosphonates. We present a rare case of multiple cranial nerve palsies as the first manifestation of PDB.

[Chronic demyelinating polyradiculoneuropathy revealing a solitary plasmacytoma of the right iliac bone]. / Polyradiculonévrite chronique révélant un plasmocytome solitaire de l'os iliaque droit.

Solitary bone plasmacytoma (pso) is a rare tumor, made of malignant plasma cells observed in a single bone, and without systemic proliferation. We report the case of a 33 year-old man who was admitted with chronic demyelinating sensorimotor polyneuropathy. Immunoelectrophoresis with immunofixation of serum proteins showed a monoclonal peak igg with lambda light chain and the workup was in favour of right hipbone secreting solitary plasmacytoma. The patient was treated with radiotherapy and steroids, and the evolution has been marked by a partial recovery of the motor deficit. An acute demyelinating sensorimotor polyneuropathy has often been described in association with pso. However, subacute and chronic demyelinating polyradiculoneuropathies have rarely been described in such patients with pso.the diagnosis should differentiate pso, poems and multiple myeloma.

Le plasmocytome solitaire osseux (pso) est une tumeur rare, faite de plasmocytes malins qu'on observe dans un seul os, sans atteinte plasmocytaire proliférative systémique. Nous rapportons l'observation clinique d'un patient de 33 ans qui a été admis pour une polyradiculonévrite chronique sensitivo-motrice démyélinisante. L'immunoélectrophorèse avec immunofixation des protéines sériques a révélé un pic monoclonal igg à chaîne légère de type lambda et le bilan étiologique a mis en évidence un plasmocytome solitaire sécrétant de l'os iliaque droit. Le patient a été traité par radiothérapie et corticothérapie, et l'évolution a été marquée par une récupération partielle du déficit moteur. C'est la polyneuropathie sensitivo-motrice démyélinisante aiguë qui a été le plus souvent décrite en association avec le pso alors que les polyradiculoneuropathies démyélinisantes subaiguës ou chroniques ont été rarement rapportées. Le diagnostic différentiel, parfois difficile, doit se faire entre un pso, un poems et un myélome multiple.

[Contribution of CSF/serum immunofixation to the diagnosis of inflammatory diseases of the central nervous system]. / Intérêt de l'immunofixation du couple LCR/sérum dans le diagnostic des maladies inflammatoires du système nerveux central.

INTRODUCTION: Multiple sclerosis and other inflammatory diseases of the central nervous system produce various and nonspecific symptoms. The diagnosis of these diseases is ultimately a clinical decision, although examination of cerebrospinal fluid (CSF) and other complementary tests such magnetic resonance imaging (MRI) and evoked potentials can be contributive. One important aspect of these diseases is intrathecal synthesis of immunoglobulins. PATIENTS AND METHODS: In order to determine the contribution of CSF/serum immunofixation to the diagnosis of inflammatory diseases of the central nervous system, we conducted a retrospective study in the biochemistry laboratory of the military instruction hospital Mohammed V. 363 CSF/serum samples were collected over a period of four years. RESULTS: Immunofixation was less sensitive than MRI for the diagnosis of inflammatory neurological disease (44% vs 87%), but was much more specific than MRI (97% for immunofixation vs 38%). The positive predictive value was higher that for MRI (85% vs 40%). The negative predictive value (80%) was close to that of MRI (86%). The bivariate analysis showed that immunofixation results could be predicted from clinical findings and complementary test results such as the index of Link, syphilis serology in CSF, and MRI. CONCLUSIONS/DISCUSSION: Semi-automatic Hydrasys immunofixation of CSF IgG is a technique exhibiting excellent diagnostic and analytical performance for the diagnosis of inflammatory neurological diseases.

[Unusual leukoencephalopathy of post-partum]. / Encéphalopathie inhabituelle du post-partum.

Neurologic complications of post-partum are serious and usually secondary to eclampsia or stroke. We here report a 26-year-old female who presented with severe headaches, blurred vision, and repeated generalized seizures secondary to posterior reversible encephalopathy that occurred after a caesarean section for fetal death in utero. Outcome was favourable. Although uncommon, this neurologic complication of the post-partum should be discussed in the presence of any sign of encephalopathy occurring in the context of acute hypertension.

Prevalence and risk factors of osteoporosis in patients with Parkinson's disease.

Parkinson's disease (PD) is the most common cause of disability in the elderly. It is currently recognized as a cause of secondary osteoporosis. To evaluate the prevalence of osteoporosis in PD and detect its risk factors, 52 patients with PD (36 men/16 women) and 52 controls paired for age and sex were recruited. Clinical data including demography, disease duration and disease severity were collected. All subjects had bone mineral density (BMD) measured by dual energy X-ray absorptiometry, dorsal and lumbar spine X-ray, and biological exams (osteocalcin, CTX, parathormon). The mean age of the patients was 60.0 +/- 9.25 years [30-77], and the mean disease duration was 4.9 +/- 4.5 years [0.2-17]. Nine patients (17.3%) were osteoporotic and 28 (53.8%) osteopenic. BMD at the lumbar spine and the hip was lower among patients than controls (spine: 1.031 vs. 1.175 g/cm(2); P < 0.001; hip: 0.968 vs. 1.054; P = 0.02). PD patients with low BMD presented a more severe disease and an insufficient sun exposure and calcium intake. There was a positive statistically significant correlation between patients BMD and body mass index and negative correlation with age, severity of PD, and osteocalcin levels. The prevalence of osteoporosis/osteopenia is high in PD patients and seems related to the severity of the disease, an insufficient sun exposure and calcium intake. This osteoporosis constitutes with falls the major risk factors of fracture in PD patients.

[Meningovascular syphilis: study of five cases]. / Méningovascularite d'origine syphilitique: étude de cinq cas.

INTRODUCTION: Clinical manifestations of syphilis are highly variable, and currently on the rise. We report five cases of ischemic stroke related to neurosyphilis. CASE REPORT: The cases of five men aged 37, 34, 41, 42 and 44 years are reported. A notion of a genital chancre was noted in three. The clinical presentation was typical of ischemic stroke in four patients. One presented with intellectual deterioration. Imaging revealed zones of ischemia in all five patients. Examination of the cerebrospinal fluid revealed lymphocytic meningitis and all five patients exhibited positive syphilis serology. Brain angiography demonstrated signs of arthritis in four patients and carotid dissection in one. Improvement was achieved with penicillin G in four patients. CONCLUSION: When the cause is doubtful, the routine work-up for ischemic stroke in young subjects should include syphilis serology.

[Idiopathic hypereosinophilic syndrome presenting with oculomotor paresis]. / Paralysie oculomotrice révélant un syndrome hyperéosinophilique essentiel.

A 42-year-old patient with a known depressive syndrome developed diplopia. The neurological examination revealed a peripheral neurogenic syndrome with incoordination of the left arm. Biological and radiological findings were in agreement with the diagnosis of idiopathic hypereosinophilic syndrome. Treatment with corticosteroids was effective.

[Neurological feature of Mediterranean spotted fever: a study of four cases]. / Manifestations neurologiques de la fièvre boutonneuse méditerranéenne : à propos de quatre observations.

BACKGROUND: Rickettsia conorii is the etiologic agent of Mediterranean spotted fever that is endemic in Mediterranean. EXEGESIS: We report four cases of serious form of Mediterranean spotted fever with neurological feature. Two of them 56 and 62 years old had meningoencephalitis. Two others had cerebellitis, they are aged 43 and 56. The course has been favourable with ciprofloxacin. CONCLUSION: It is a benign well-known illness in spite of apparition of severe visceral complications, which can drag the death in 2.5% of cases. Neurological feature is more frequent and present in 28% of cases. Precocious treatment prevents this unfavourable evolution.

[Sneddon's syndrome: 4 cases and a review of the literature]. / Le syndrome de Sneddon: 4 cas et revue de la littérature.

Sneddon syndrome is an association of livedo racemosa and cerebrovascular ischemic events generally occurring in young adults. This is an uncommon chronic progressive arterio-occlusive disorder of unknown cause involving small and medium sized vessels. We report four cases. One case was disclosed by cerebral hemorrhage. One pathogenic hypothesis suggests the involvement of an idiopathic progressive inflammatory arteriopathy or secondary thrombotic disorder comparable with antiphospholipid syndrome.

[Neurological manifestations of xeroderma pigmentosum]. / Les manifestations neurologiques du xeroderma pigmentosum.

Xeroderma pigmentosum is a genodermatosis with neurological manifestations in approximately 18p. 100 of cases. Polymorphous and variably associated signs are observed, progressing with the clinical course. The etiology of the neurological breach remains unknown. We report two siblings who had xeroderma pigmentosum with intellectual deficiency, a pyramidal, cerebellar and cordonal syndrome, ophthalmoplegia, and axonal peripheral neuropathy. We discuss the epidemiological, clinical and electrophysiological aspects of the neurological breach in xeroderma pigmentosum.

[Brainstem tuberculomas: two cases]. / Tuberculomes du tronc cérébral.

We report two cases of brainstem tuberculomas with favorable outcome after medical treatment alone. Tuberculomas should be suspected in all cases presenting space-occupying lesions of the brainstem.