Subacute sclerosing panencephalitis: case based review

Subacute sclerosing panencephalitis (SSPE) is a chronic debilitating condition that occurs in children affected with measles. SSPE is broadly distinguished as typical SSPE, the more rampant form, occurring over a period of years following primary measles infection, while the atypical has a more rapidly progressive course over weeks to months. SSPE can present with cognitive, epileptic, autonomic, pyramidal and ophthalmologic manifestations with scholastic decline being the primary feature. The management of SSPE focuses on improvement of quality of life and prolongation of survival which can be achieved with the use of supportive care modalities and immunomodulators respectively. This is a comprehensive review which discusses several parameters of SSPE such as epidemiology, pathophysiology, clinical presentations, and detailed management protocol for this condition. As part of this review, we also discuss a case of rapidly progressive, fulminant and atypical SSPE in a five-year-old male presenting clinically with myoclonic jerks of lower extremities.

Causas no oncológicas del síndrome de Kinsbourne / Non oncologic causes of Kisbourne syndrome

Introducción. Kinsbourne en 1962, enmarca una tríada caracterizada por opsoclonos, mioclonos y ataxia cerebelosa. Su incidencia no está definida y es poco común; ocurre en su mayoría en la edad pediátrica. Se han propuesto diferentes etiologías, dentro de las más frecuentes se relaciona con entidades paraneoplásicas (50% asociado a neuroblastoma). Objetivo: analizar las causas del paciente con síndrome de Kinsbourne (SK) atendidos en el Hospital Infantil de México Federico Gómez en el período comprendido entre 1993-2004. Material y métodos. Se realizó un estudio retrospectivo en pacientes de ambos sexos, menores de 16 años, atendidos en el período comprendido entre 1993 y 2004; estableciéndose la frecuencia de pacientes con SK en dicho período de tiempo estudiado; además de los síntomas más frecuentes y el seguimiento de estos pacientes. Resultados. Se obtuvieron 26 pacientes, la edad de presentación más frecuente fue de 21 meses de edad. El tiempo que transcurrió entre la presentación de los síntomas y el diagnóstico de la enfermedad fue en promedio de 3.8 meses. El signo inicial en 88.5% de los pacientes fue la ataxia. Dentro de las etiologías más frecuentes se encontró en primer lugar las causas infecciosas, seguidos de trauma craneoencefálico leve y procesos inflamatorios. En 27% de los casos no se consignó algún antecedente patológico relacionado al inicio de los síntomas. Sólo se encontró un caso asociado a neuroblastoma. Conclusión. La causa más frecuente encontrada en nuestra población fue la de origen infeccioso, lo cual da pauta a redefinir la búsqueda y los protocolos de estudio en estos pacientes, así como los tratamientos y pronósticos propuestos. Es interesante observar que a cinco años de seguimiento, únicamente se encontró un proceso neoplásico, lo cual también puede redefinir el pronóstico en general de estos pacientes.

Introduction. In 1962 Kinsbourne describes a triad characterized by opsoclonus, myoclonus and ataxia.The incidence is rare; it occurs predominantly in pediatric population. It may express different etiologies; the most frequently cause associated is with paraneoplasia (50% with neuroblastoma).The great variety of diagnosis proposed for the Kinsbourne syndrome (KS) implies a great number of treatments too. Besides KS is rarely seen, the dramatic presentation, sudden appear, characterized triad and torpid evolution that can be in relationship with a paraneoplasia makes the syndrome a entity of great interest for the medical doctors, making of great importance known the different etiologies of each community for establish standardized protocols for the clinical study and follow adequate for these patients. With this revision, we suspect to identify the different etiologies in the KS in the population attended in the Hospital Infantil de Mexico Federico Gomez (HIMFG). Objective: describe and analyze the different causes of KS attended in the HIMFG between 1990-2004. Material and methods. With a retrospective study, we include patients of both sexes, <16 years, seen between 1990 and 2004. We study the frequency of patients seen in that period, the most frequent etiologies and the evolution of these patients. Results. Of 26 patients, the most frequent age of presentation was 21 months of age. The time between the first symptoms and the diagnosis was approx. 3.8 months.The initial symptom in 88.5% was ataxia.The most frequent etiologies related were infections, craneoencephalic trauma and inflammatory process. In 27% of the patients there were no antecedents. Only one of the patients was associated with neuroblastoma. Conclusion. The infection was the most frequent cause in our series that results implies the necessity to make strategies in the search of these patients.

Two Cases with Carbamazepine-induced Unusual Side Effects-Encephalopathy and Myoclonic Jerks

Central nervous system toxicity is the most commonly recognized problem during treatment with carbamazepine (CBZ). The most common side effects of CBZ are drowsiness, incoordination, and vertigo. However, unusual conditions such as movement disorders, seizure aggravation, and encephalopathy have also been attributed to CBZ therapy. In case 1, cognitive dysfunction and exacerbation of preexisting gait disturbance were observed in a 63-year-old female who had frontal lobe epilepsy, schizencephaly, and lissencephaly treated with CBZ. The neurological symptoms were resolved 24 hours after the withdrawal of CBZ and induced with the reintroduction of CBZ. In case 2, myoclonic jerks occurred in a 37-year-old female when CBZ was readministered after a 4-day-withdrawal period of CBZ. The myoclonic jerks disappeared 12 days after CBZ was discontinued. In both cases, plasma CBZ levels were within the therapeutic range. We report two cases with encephalopathy and myoclonic jerks as unusual side effects of CBZ, with the plasma levels of CBZ being within the therapeutic range.