ABSTRACT
INTRODUCTION: Neurological involvement in sarcoidosis is rare and highly variable. To date, no consensus was reached about the diagnosis approach. We report a case series of 9 patients with neuosarcoidosis, with favorable outcome under therapy. MATERIALS AND METHODS: We examined a case series of 9 patients with neurosarcoidosis. Clinical, radiological, therapeutic features and outcome were studied. RESULTS: Six of the nine patients were females. Patients' age ranged from 31 to 70 years. Initial neurological symptoms lead to the diagnosis of systemic sarcoidosis in all patients. Central nervous system involvement was found in 77 percent with cranial nerve involvement in 55 percent. Twenty-three percent of patients presented with peripheral neuropathy and 33 percent with meningitis. The diagnosis was definite in 2 patients, probable in one and possible in six others. All patients were given steroid therapy. Total remission was obtained in three and partial remission in three. Three patients remained stable and one died. CONCLUSION: Histological signs are not constant in neurosarcoidosis. The lack of these signs should lead the physician to search for latent extraneurological symptoms which are suggestive of the diagnosis. Nervous biopsy can thus be avoided.
Subject(s)
Brain Diseases/pathology , Sarcoidosis/pathology , Adult , Aged , Anti-Inflammatory Agents/therapeutic use , Brain Diseases/drug therapy , Bronchoalveolar Lavage Fluid/immunology , Electroencephalography , Electromyography , Female , Humans , Hypertrophy/pathology , Lymphocytes/immunology , Magnetic Resonance Imaging , Male , Methylprednisolone/therapeutic use , Middle Aged , Parotid Gland/pathology , Prednisone/therapeutic use , Sarcoidosis/drug therapyABSTRACT
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.
Subject(s)
Hypereosinophilic Syndrome/complications , Oculomotor Nerve Diseases/etiology , Adult , Humans , Hypereosinophilic Syndrome/diagnosis , MaleABSTRACT
INTRODUCTION: Two cases of hyperthyroidism revealed by a cerebral venous thrombosis are reported. EXEGESIS: Clinical symptomatology consisted of intra-cranial hypertension and convulsive crisis associated to thyrotoxicosis. Evolution was favorable with etiologic and symptomatic therapy. CONCLUSION: The cerebral venous thrombosis risk on hyperthyroidism is discussed.
Subject(s)
Hyperthyroidism/complications , Intracranial Thrombosis/etiology , Thrombophlebitis/etiology , Adult , Female , HumansABSTRACT
INTRODUCTION: Deep cerebral vein thrombosis is a specific clinical pattern of neuroBehçet. The clinical features of ischemic stroke by Rosenthal or internal cephalic vein thrombosis predominate. METHOD: A retrospective analysis of twelve cases of Rosenthal vein thrombosis revealing Behçet disease were analyzed. These cases accounted for one-quarter of the NeuroBehçet patients treated in our unit. RESULTS: The typical pattern of Rosenthal vein thrombosis clinical was present in all patients who developed a diencephalo-mesencephalic syndrome. CONCLUSION: The clinical presentation is highly suggestive of diagnosis confirmed by imaging. Outcome is favorable with corticosteroids and anticoagulation.
Subject(s)
Behcet Syndrome/complications , Stroke/complications , Venous Thrombosis/complications , Adrenal Cortex Hormones/therapeutic use , Adult , Anticoagulants/therapeutic use , Behcet Syndrome/drug therapy , Cerebral Angiography , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Retrospective Studies , Venous Thrombosis/drug therapyABSTRACT
A case of cavernous sarcoid granuloma, revealed by a slit sphenoidal syndrome is reported. Brain MRI showed a cavernous pseudo-tumoral lesion. The diagnosis of sarcoidosis was confirmed by the epithelioid and giant-cell granuloma without caseous necrosis found at hepatic biopsy. Outcome was favorable with corticosteroid therapy alone.
Subject(s)
Bone Diseases/diagnosis , Granuloma/diagnosis , Skull Base , Humans , Male , Middle AgedABSTRACT
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.
Subject(s)
Sneddon Syndrome/diagnosis , Adult , Female , Humans , Male , Middle Aged , Sneddon Syndrome/etiologyABSTRACT
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.
Subject(s)
Nervous System Diseases/physiopathology , Xeroderma Pigmentosum/physiopathology , Adult , Brain/diagnostic imaging , Female , Humans , Intellectual Disability/etiology , Intellectual Disability/genetics , Male , Nervous System Diseases/etiology , Nervous System Diseases/genetics , Ophthalmoplegia/etiology , Ophthalmoplegia/genetics , Pedigree , Tomography, X-Ray Computed , Xeroderma Pigmentosum/complications , Xeroderma Pigmentosum/geneticsABSTRACT
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.
Subject(s)
Brain Stem/diagnostic imaging , Brain Stem/pathology , Tuberculosis, Central Nervous System/diagnosis , Adult , Anti-Inflammatory Agents/therapeutic use , Antibiotics, Antitubercular/therapeutic use , Enzyme-Linked Immunosorbent Assay , Humans , Immunoglobulins, Intravenous/therapeutic use , Magnetic Resonance Imaging , Male , Steroids , Tomography, X-Ray Computed , Tuberculosis, Central Nervous System/drug therapyABSTRACT
Three patients aged 32, 30 and 36 years, had chicken pox then developed acute cerebellar ataxia (for two) and acute polyradiculoneuritis. Cerebrospinal fluid (CSF) protein content was increased and varicella virus serology was positive in both blood and CSF. All three patients improved with aciclovir.
Subject(s)
Cerebellar Ataxia/etiology , Chickenpox/complications , Polyradiculopathy/etiology , Acute Disease , Adult , Cerebellar Ataxia/cerebrospinal fluid , Cerebellar Ataxia/virology , Cerebrospinal Fluid Proteins/analysis , Chickenpox/cerebrospinal fluid , Herpesvirus 3, Human/isolation & purification , Humans , Male , Polyradiculopathy/cerebrospinal fluid , Polyradiculopathy/virologyABSTRACT
We report a case of Miller-Fisher syndrome with ophthalmoplegia, ataxia and areflexia. The main characteristics of this case were bilateral optic neuropathy and ponto-mesencephalic abnormalities on MRI. This case confirms the possible central neurologic localisation in this rare syndrome.
Subject(s)
Magnetic Resonance Imaging , Mesencephalon/pathology , Miller Fisher Syndrome/pathology , Optic Neuritis/etiology , Pons/pathology , Adult , Anti-Inflammatory Agents/therapeutic use , Humans , Intracranial Hypertension/etiology , Intracranial Hypertension/pathology , Male , Methylprednisolone/therapeutic use , Miller Fisher Syndrome/complications , Miller Fisher Syndrome/drug therapy , Optic Neuritis/drug therapy , Optic Neuritis/pathologyABSTRACT
We report a clinical association of diffuse scleroderma and amyotrophic lateral sclerosis (ALS) in two patients. Scleroderma was diagnosed on skin, digestive, osteoarticular, pulmonary lesions and inflammatory syndrome. ALS was suspected on the association of diffuse amyotrophy, fasciculations, pyramidal tract involvement and electrophysiological data. Chronic medulla ischemia and or immune abnormalities are proposed as potential pathological mechanisms for ALS but fortuitous association can not be excluded.
Subject(s)
Amyotrophic Lateral Sclerosis/complications , Scleroderma, Systemic/complications , Amyotrophic Lateral Sclerosis/diagnosis , Humans , Male , Middle Aged , Scleroderma, Systemic/diagnosisABSTRACT
It has been previously shown in Tunisian and Algerian families that the locus for SCARMD maps to the proximal part of 13q, and in Algerian families that the disease is associated with deficiency of the 50 kDa dystrophin associated glycoprotein (50DAG). We have tested this linkage in six families from Morocco where this disease is also prevalent. In one family the 50DAG was tested and found to be negative in a muscle biopsy. Our results showed similar linkage in this country, with statistical tests indicating genetic homogeneity between the three Maghreb countries.