MRI changes were universal but diverse and involved almost all the structures of the brain in symptomatic patients. A fair correlation between MRI observations and various clinical features provides an explanation for the protean manifestations of the disease.
Patients with ET showed widespread areas of atrophy in both cerebellum and cerebral GM, which supports the current concept of the progressive and diffuse nature of ET. Patients with additional head tremor may represent a distinct subgroup of ET.
Wilson's disease (WD) is clinically and radiologically a dynamic disorder. However, there is a paucity of studies involving sequential MRI changes in this disease with or without therapy This study looked at serial MRI changes and their clinical correlate in patients with WD The severity of MRI changes using 1.5 T MRI in 50 patients with WD was graded based on alteration in signal intensity of focal lesions and atrophy. Details of clinical manifestations, Schwab and England Activities of daily living (MSEADL) score, Neurological Symptom Score (NSS) and Chu staging were recorded. Clinical severity and disability scores were correlated with MRI scores using SPSS v10 The mean age at onset of illness and diagnosis was 12.8+/-5.6 years and 14.4+/-6.0 years, respectively. At the time of first MRI, patients had been treated for 49.0+/-77.3 months. At a follow-up of 24.2+/-12.2 months, clinically 36 patients had improved, 9 remained the same and 5 had worsened. Serial imaging revealed an improvement in MRI parameters in 35 patients, no significant changes in 10, worsening in 4 and an admixture of resolving and evolving changes in 1. The overall MRI score improved from 8.2+/-5.7 to 5.9+/-6.6. There was an improvement in measures of disability and impairment in all: Chu stage, 11.5+/-0.7 to 1.3+/-0.6; MSEADL score (%), 79.7+/-27.6 to 88.0+/-25.4; NSS, 10.6+/-11.2 to 8.0+/-11.6, with good clinico-radiological correlation. Patients with extensive changes, white-matter involvement and severe diffuse atrophy had a poor prognosis In conclusion, the majority of patients with WD showed variable improvement in clinical and MRI features when treated.
Endoscopic third ventriculostomy (ETV) for obstructive hydrocephalus has a failure rate of 20–50% in various series. The present study analyzes ETV failures in 72 patients over a 2-year period and attempts to outline a management plan. Of the 72 patients who underwent ETV, it failed in 13. Seven of these failures occurred within 1 month, and in 5 others, ETV failed after 1–2 months. Another patient had a delayed failure 2 years after the initial surgery. Upon clinical failure, MRI scans were performed in all patients using either T2 fast spin echo or two-dimensional phase contrast MRI techniques. Of these, no flow could be demonstrated in 12 patients, whereas in 1 patient, good flow was observed. Endoscopic exploration was undertaken in the 12 patients in whom flow could not be demonstrated. Of the 12 who underwent endoscopic exploration, a patent stoma was observed in 7, necessitating insertion of a ventriculoperitoneal shunt (VPS). In the other 5, the stoma had closed by gliosis and a repeat ETV was performed. In 3 of these patients, in addition to the ETV, a VPS was also inserted in accordance with the family’s wishes. VPS insertion was carried out in the patient with suggestion of good flow through the stoma. In failed ETV, MRI with flow studies is essential to identify the possible cause of failure. Endoscopic exploration is indicated for patients with no evidence of flow. A repeat ETV is indicated in patients with a closed stoma. Patients with a patent stoma could require insertion of a cerebrospinal fluid shunt.
Our findings suggest involvement of multiple WM tracts in juvenile OCD. In addition to the widely proposed hypothesis of orbitofrontal-striato-thalamo-cortical circuitry deficits in the development of OCD, our findings suggest involvement of additional brain regions, possibly parietal cortex, lateral prefrontal cortex, and limbic system. The widespread differences in WM among cases and controls also points to the possibility of underlying myelination changes.
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