Balo's concentric lesions with concurrent features of Schilder's disease in relapsing multiple sclerosis: neuropathological findings

Atypical inflammatory demyelinating syndromes are rare neurological diseases that differ from multiple sclerosis (MS), owing to unusual clinicoradiological and pathological findings, and poor responses to treatment. The distinction between them and the criteria for their diagnoses are poorly defined due to the lack of advanced research studies. Balo's concentric sclerosis (BCS) and Schilder's disease (SD) are two of these syndromes and can present as monophasic or in association with chronic MS. Both variants are difficult to distinguish when they present in acute stages. We describe an autopsy case of middle-aged female with a chronic history of MS newly relapsed with atypical demyelinating lesions, which showed concurrent features of BCS and SD. We also describe the neuropathological findings, and discuss the overlapping features between these two variants.

Neuropathological spectrum of lesions associated with intractable epilepsies: a 10-year experience with a series of 153 resections.

BACKGROUND: Surgical management of intractable epilepsies is currently an established mode of therapy in various clinical settings. AIMS: To retrospectively evaluate the neuropathological findings in both temporal and extratemporal lobe resections in such patients. MATERIALS AND METHODS: The study included resected specimens from patients with intractable epilepsy managed at a tertiary care hospital of India, during a 10-year period (1995-2004). RESULTS: A total of 153 patients, with mean age of 19.4 years and male predominance (73.2%) were included in the study. Overall, there was a predilection for the temporal lobe (73.2%), while 41cases were extratemporal in location. On histopathology, mesial temporal sclerosis (MTS) (24.8%) was the commonest lesion, followed by tumors (19.6%) and isolated focal cortical dysplasia (FCD - 15.11%). Other less common findings included Rasmussen encephalitis, non-specific gliosis and vascular malformations. In addition, 20.9% (32 cases) had dual lesions, majority of which included FCD with ganglioglioma (15 cases) or with dysembryoplastic neuroepithelial tumor (12 cases). In the temporal lobe, neoplasms and dual lesions formed the majority (apart from MTS), unlike dual lesions followed by neoplasms and FCD, in the extratemporal location. CONCLUSION: This series demonstrates that most patients with chronic intractable epilepsy have significant histopathological findings and highlights the neuropathological spectrum of such lesions, in the Indian context. This was similar to that reported from the West, but different from the single Indian series available in the literature. Further, the overall profile of temporal lobe lesions was not different from the extratemporal ones.

Concentric sclerosis: imaging diagnosis and clinical analysis of 3 cases.

Baló's Concentric sclerosis (BCS) is a rare demyelinating disease considered to be a variant of multiple sclerosis (MS). The typical magnetic resonance imaging (MRI) changes associated with BCS consist of concentric rings or onions' cross-section on T1-weighted (T1W) images. Because MRI reveals pathological changes consistent with autopsy in the focus of BCS, it plays an important role in the before-death diagnosis of BCS. We report three cases of BCS diagnosed antemortem on the basis of the typical concentric rings pattern on MRI and on the basis of clinical findings and cerebrospinal fluid (CSF) examination. BCS often occurs in the prime of life, acutely or subacutely. Then come cerebral multifocal symptoms and signs. We find that BCS is not always an acute and irreversible pathological process as described in the past.

Leucodistrofia cavitaria sudanofílica: forma familiar adulta / Sudanophilic cavitating leukodystrophy: a familiar adult form

La leucodistrofia cavitaria sudanofílica constituye un trastorno raro de la sustancia blanca cerebral, el cual se caracteriza clínicamente por sordera, retardo mental, trastornos de conducta y afectación de tractos largos descendentes. Desde el punto de vista neuropatológico, los rasgos resaltantes son extensa desmielinización central subcordial y profunda, conspicua astrocitosis y macro y microcavitación. Se describe el caso de un paciente masculino de 34 años de edad, con antecedentes personales y familiares, rasgos clínicos y hallazgos neuropatológicos consistentes con leucodistrofia cavitaria sudanofílica. Hasta donde alcanza nuestro conocimiento, en Venezuela no han sido publicados casos de esta inusual entidad nosológica

Comentários sobre dos casos de enfermedad de Pelizaeus-Merzbacher / Pelizaeus-Merzbacher disease

Comentários a dos casos - hermanos- de síndrome de Pelizaeus-Merzbacher sin historia de antecedentes familiares con, entre los dos, una hermana sana. UMa anoxia neonatal grave, y una sintomatomalogía, atípica dificultaran el diagnóstico en el primer caso. Salvo una gran diferencia en cuanto a la afectación mental, la evolución somática fué, luego, bastante paralela. El signo inicial fué un nistagmo pendular sólo. No había acromatopsia. No habia cambio oftalmoscópicos escandalosos y todos de acuerdo con los descritos en esta enfermedad, salvo, en el segundo caso, unas microhemorragias. La analítica solo reveló una plaquetopenia.