RESUMO
INTRODUCTION: Multifocal motor neuropathy with partial conduction block is characterized by being a chronic, demyelinating, autoimmune severely disabling neuropathy. In Cuba three cases were reported by Dr. Estrada et al in 1999. This neuropathy presents clinically in relatively young persons. The arms are predominantly affected and the typical signs are of severe asymmetrical weakness, with atrophy which is less marked than the weakness, fasciculations, cramps and myokymiae of the affected muscle. Neurophysiological study shows partial block of motor nerve conduction. Clinical interest is due to it being potentially curable. Many cases are wrongly diagnosed as motor neurone disease. CLINICAL CASES: We present five patients aged under 55 years with progressive chronic motor neuropathy mainly affecting their arms. Study of nerve conduction showed partial block of the conduction in motor nerves, in segments with no block of sensory neuroconduction. One patient had been diagnosed as having motor neurone disease; another had slight sensory involvement in the distal territory of the radial nerve; in two patients the symptoms affected all four limbs. In three patients good results were obtained with intacglobin, followed by azothroprine and prednisone. Two patients showed no improvement with this treatment so intravenous cyclophosphamide was given for nine months which stopped progression of the disorder. CONCLUSIONS: Multifocal motor neuropathy is potentially treatable. In some cases intacglobin azathioprine and prednisone may be a useful alternative to cyclophosphamide.
Assuntos
Doença dos Neurônios Motores/complicações , Condução Nervosa , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença dos Neurônios Motores/diagnósticoRESUMO
INTRODUCTION: The opsoclonic-myoclonic-ataxic syndrome (OMAS) is that in which there are at least two of the cardinal signs (opsoclonus, myoclonus and ataxia). OBJECTIVES: To report three cases, their evolution and aetiology, and discuss the main physiopathological basis and guidelines for treatment. CLINICAL CASES: We present three cases admitted to the Internal Medicine Department with the diagnosis of OMAS. The investigations included standard biochemical and haematological tests, a full study of the cerebrospinal fluid, computerized axial tomography of the skull and thorax, and magnetic resonance. We describe the clinical presentation, evolution, aetiology and treatment given. The results of the complementary tests are shown in the form of tables. Finally we consider the diagnosis of this condition. Three patients, two men and one woman, aged between 43 and 78 were diagnosed as having OMAS. The causes were: paraneoplasia, acute diffuse encephalomyelitis and probable multiple sclerosis. The patient with lung cancer died whilst the other two showed almost complete recovery on the treatment given. CONCLUSIONS: Our cases show that OMAS may be a feature of a diffuse process affecting the brainstem and cerebellum. The commonest causes found are multiple sclerosis, acute diffuse encephalomyelitis and as a paraneoplastic feature of a tumour. It may improve with treatment, but depending on the aetiology, death may occur. Immunomodulation treatment is vital even though symptomatic treatment may be given.
Assuntos
Ataxia/diagnóstico , Mioclonia/diagnóstico , Transtornos da Motilidade Ocular/diagnóstico , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , SíndromeRESUMO
INTRODUCTION: Thrombosis of the veins and venous sinuses is an uncommon condition. In 1947 it was described in adults by Garcin and Pestel. It is a potentially serious disorder, in which there is severe intracranial hypertension which may be fatal or cause serious neurological sequelae. Diagnosis is made, during life, on the clinical condition and neuro-imaging features. We consider that treatment of the underlying condition is essential to improve prognosis, although this is controversial. In Cuba there are no publications reporting series of patients with this condition. CLINICAL CASES: We present five patients, four of whom were women, with a neurological disorder involving focal neurological deficits, headache and severe intracranial hypertension, of subacute onset and with convulsions in 60%. The results of imaging studies were compatible with thrombosis of veins and/or venous sinuses. The diagnosis was conformed at necropsy in two patients. The commonest risk factor was the puerperium, occurring in four cases. The patients were treated symptomatically. Two patients died. The other patients made good progress. CONCLUSIONS: The puerperium is the commonest risk factor in thrombosis of the venous sinuses and the veins draining into them. Headache, neurological focal signs and level of consciousness are the main symptoms and signs. Coma, haemorrhagic infarct and convulsions are of bad prognosis and followed by unsatisfactory progress of the disorder.