ABSTRACT
This study described the clinical and paraclinical features of south Indian patients with longitudinally extensive transverse myelitis (LETM) and contrasted the findings betweenaquaporin-4 positive versus negative patients. The subjects were recruited between2010 and 2013.The distinctive features among71 LETM patients were compared and it was observed that 56% of the total subjects were found to be AQP4-Ab positive. The ratio of female tomale was found to be higher in the AQP4-Ab positive group. Magnetic resonance imaging showed holocord involvement more commonly in AQP4-Abnegative than positive group. The presence of hypointense lesions did not correlate with severity. The main distinctive features between AQP4-Abpositive and negative cases include older onset age, higher proportion of female, low frequency of conus involvement and higher prevalence of coexisting autoimmune disorders in AQP4-Ab positive cases. Therewas no difference in attack severity, onset of optic neuritis, and spasms between the two groups. Our results suggest that the clinical and spinal cord neuro-imaging information can aid in distinguishing between the positive and negative group of patients with LETM. The early detection of AQP4-Ab positive status predicts the recurrence of LETM or occurrence of optic neuritis duringthe study period.
ABSTRACT
BACKGROUND: Subacute sclerosing panencephalitis (SSPE) is a delayed and fatal manifestation of measles infection. Fulminant SSPE is a rare presentation in which the disease progresses to death over a period of 6 months. The clinical features are atypical and can be misleading. CASE REPORT: We report herein a teenage boy who presented with acute-onset gait ataxia followed by right hemiparesis that evolved over 1 month, with left-hemispheric, delta-range slowing on the electroencephalogram (EEG). Magnetic resonance imaging disclosed multiple white-matter hyperintensities, suggesting a diagnosis of acute disseminated encephalomyelitis. He received intravenous steroids, and within 4 days of hospital admission he developed unilateral slow myoclonic jerks. Repeat EEG revealed Rademecker complexes, pathognomonic of SSPE, and an elevated titer of IgG antimeasles antibodies was detected in his cerebrospinal fluid. The disease progressed rapidly and the patient succumbed within 15 days of hospitalization. The diagnosis of SSPE was confirmed by autopsy. CONCLUSIONS: This case illustrates the difficulty of recognizing fulminant SSPE when it manifests with asymmetric clinical and EEG abnormalities.