Síndrome de enclaustramiento: presentación de un caso / Locked-in syndrome: case report

El síndrome de enclaustramiento (Locked-in syndrome) es una entidad neurológica altamente discapacitante, producida por lesiones en la porción ventral de la protuberancia; de etiología principalmente vascular. Clínicamente se manifiesta por tetraplejía, anartria, preservación de la conciencia y capacidad de expresarse mediante movimientos oculares. Presentamos el caso de un paciente masculino de 33 años, transferido de otra institución de salud con un cuadro clínico progresivo y poco específico, caracterizado principalmente por deterioro del estado de conciencia, dificultad respiratoria, cefalea de intensidad moderada y vómitos. Mediante pruebas de imágenes se comprobó la presencia de un área de isquemia en la región irrigada por el sistema vertebrobasilar. Se realizó un enfoque diagnóstico y terapéutico invasivo de orden endovascular.

Locked-in syndrome is a highly disabling neurological entity, due to lesions in the ventral portion of the pons, mainly vascular etiology. Clinical features are quiadriplegia, anarthria, preservation of consciousness and the ability to express by ocular movements. We shown a case of a 33 years-old man, who was transfer from another institution with a progressive and poorly specific clinical manifestations, mainly characterized by diminishing of consciousness, breathing difficulty, headache and vomiting, with the presumptive diagnosis of central nervous system infection. We performed image diagnostic tests and they shown and ischemic feature in the region of the vertebrobasilar irrigation. It was perform an endovascular diagnostic therapeutic approach.

Locked-in syndrome and blue toe syndrome caused by cardiopulmonary bypass

Severe inflammation after cardiopulmonary bypass with the vasculitis of the acral extremity and vertebro-basilar arterial system leads to the locked-in syndrome and blue toe syndrome. In broad terms, systemic, idiopathic, and environmental factors provoke syndromes that present with digital discoloration or the blue toe syndrome. Painful digital discoloration, accompanied by ulceration, suggests vasculitis, involving small blood vessels. Definitive diagnosis usually requires histological documentation because vasculitic syndromes have no pathognomonic clinical features or laboratory test results. The case introduced herein is that of a woman who developed the locked-in syndrome in conjunction with quadriplegia, loss of facial movement, speech loss, and loss of horizontal eye movements. She had initially presented with severe mitral stenosis and left atrial clot and undergone mitral valve replacement and clot extraction. The patient expired from multiple organ failure despite prolonged ventilatory support, including tracheotomy, and meticulous nursing care and antibiotic prophylaxis. Given the previously reported partial recovery from this syndrome with the use of steroids, we would advocate the use of such pharmacological agents

Locked-in syndrome: A rare manifestation of pediatric stroke.

Locked-in syndrome is characterized by upper motor neuron quadriplegia, paralysis of lower cranial nerves, bilateral horizontal gaze palsy and anarthria, with preserved consciousness. It is due to a ventral pontine lesion following a basilar artery occlusion. We report the first Indian case report of locked-in syndrome, a 10-year old girl in whom the syndome was preceded by a ‘herald hemiparesis’. Although the exact etiology for the basilar artery occlusion could not be determined, treatment with low molecular weight heparin and warfarin was followed by partial recovery.

Central myelinolysis in a patient with hyponatraemia

We present a case of a 50-year old man who developed mutism and a flaccid quadriparesis within 48 hours of presentation to hospital with severe hyponatraemia. A diagnosis of central pontine myelinolysis was made based on the clinical features and typical appearances on magnetic resonance imaging.

Tetraparesis secondary to cervical ossification of the posterior longitudinal ligament: case report

Ossification of the posterior longitudinal ligament (OPLL) is a rare cause of myelopathy in non-Oriental populations and relatively unrecognized by general practitioners. A case of an Afro-Brazilian 54-years-old woman presenting with tetraparesis due to cervical OPLL is presented. Emphasis is made for the inclusion of OPLL in the differential diagnosis of compressive cervical myelopathy.

Ossificação do ligamento longitudinal posterior (OLLP) é causa rara de mielopatia na população não Oriental e relativamente subdiagnosticada por clínicos gerais. Relata-se um caso de mulher de 54 anos descendência Afro-Brasileira com tetraparesia associada a OLLP cervical. Enfatiza-se a inclusão da OLLP como diagnóstico diferencial de mielopatia cervical compressiva.

Epilepsy in children with cerebral palsy.

This was a prospective analytical study done from October'99 through December 2000 to observe the clinical profile of epilepsy in children with cerebral palsy. Sixty epileptic children with cerebral palsy were studied and their results were compared with the results of epilepsy in 30 developmentally normal children. Ages of all children studied were 1-15 years. Majority were below five years of age and male out numbered female. Spastic quadriplegia (60%) was the commonest type of cerebral palsy. Generalized tonic-clonic seizure (66%) was the commonest seizure type in both groups. Second most common type of seizure was partial (13%) and myoclonic (29%) in developmentally normal and cerebral palsy children respectively. Mental retardation (63%) and speech delay (58%) were the two common associated problems in cerebral palsy patients. Ventriculomegaly (55%) was the commonest computed tomographic findings in cerebral palsy patients. In electro encephalography generalized epileptiform activity was the commonest finding in both groups. Clinical pattern is different in many ways when epilepsy is associated with cerebral palsy.

Quadriparesis in a young female suffering from rheumatoid arthritis.

Cervical spine is involved in a significant proportion of patients suffering from rheumatoid arthritis. Although cervical spine disease may often be 'benign', neurological complications are not uncommon. Patients of rheumatoid arthritis should be screened for cervical spine involvement and appropriately treated with combination of anti-rheumatic drugs. We report a case of quadriparesis secondary to subluxation and disc herniation at C4-C5 level in a young woman with rheumatoid arthritis of short duration.

Avaliação audiológica em paralíticos cerebrais quadriplégicos espásticos / Audiological assessment in individuals with spastic quadriplegic cerebral palsy

O objetivo deste estudo é apresentar a avaliação audiológica de 17 indivíduos paralíticos cerebrais quadriplégicos espásticos, de idade variando de 7 a 14 anos. Foram realizadas as avaliações da audição buscando identificar bloqueios na transmissão dos sons pelo sistema periférico e/ou central, por meio da audiometria tonal liminar e do teste PSI - Pediatric Speech Intelligibility Test - em tarefa dicótica-PSI-MCC e em tarefa monótica-PSI-MCI. A relevância deste estudo está na discussão dos procedimentos de inibição dos padrões patológicos e facilitação de padrões posturas que auxiliaram o paralítico cerebral a expressar seus conhecimentos, realizando as avaliações propostas e sem os quais a avaliação audiológica não seria possível, e apresentar os resultados destas avaliações. Os resultados indicaram perda auditiva por bloqueio no funcionamento das estruturas do sistema auditivo periférico em 59 por cento das orelhas direitas e 53 por cento das orelhas esquerdas dos paralíticos cerebrais avaliados. A configuração da curva audiométrica que predominou foi descendente (90 por cento para a orelha direita e 67 por cento para a orelha esquerda). No teste PSI - MCC, os resultados indicaram normalidade quando os estímulos foram apresentados às orelhas direitas em 8 por cento, e às orelhas esquerdas em 17 por cento. Nenhum quadriplégico espástico conseguiu normalidade para o teste PSI - MCI.

Neurological complications associated with mycoplasma pneumoniae infection. A case report

manifestations may be neurological with variable expression, encephalitis being the most frequent. Meningitis, myelitis or polyradiculoneuritis are also reported. The pathophysiology of neurological manifestations remains poorly understood. Although not isolated from cerebral tissue, MP was reportedly detected in cerebro-spintal fluid [CSF] on several occasions. We report the case of a five-year old girl who presented with fever, then pneumonia, and later developed spastic quadriparesis. MP was identified as the causative agent, evidenced by the presence of specific antibodies in both serum and CSF. The identification of a central nervous syndrome associated with confirmed MP infection extends the list of similar cases documented in individuals younger than 17 years of age

Pure motor spastic quadriparesis in systemic lupus erythematosus.

SLE has numerous neurological manifestations. We report a case of SLE with gradual onset, upper motor neuron type spastic quadriparesis without sensory or bladder/bowel involvement. There was good response to steroids.

Hallazgos tomográficos en el sindrome de encerramiento: reporte de tres casos / Tomographic findings in loocked in syndrome: report of 3 cases

Se reportan tres casos de pacientes con síndrome de "encerramiento" en los cuales las lesiones fueron demostradas por estudio tomográfico. Dos pacientes presentaron hemorragia ventral protuberancial y mesencefálica y el otro un infarto isquémico protuberanciall. Los hallazgos tomográficos en el síndrome de "encerramiento" han sido reportados raramente