Hippocampal sclerosis and amygdala dysplasia

Case contributed by RMH Neuropathology
Diagnosis certain

Presentation

Long standing temporal lobe epilepsy.

Patient Data

Age: 35
Gender: Female

The left hippocampus is small and has increased T2 and FLAIR signal. The internal architecture is only mildly distorted. No other abnormalities identified. In particular, the amygdala has a normal appearance. Normal grey-white matter differentiation. No intra or extraaxial mass lesion.

MICROSCOPIC DESCRIPTION:

1. The sections of temporal neocortex and white matter show patchy mild gliosis of the sub-pial molecular layer (Chaslin's sclerosis). Cortical laminar architecture is preserved. There are no features of malformation of cortical development. Scattered neurons are noted in white matter. Myelination appears normal. No features of tumor are seen.

2. The section of amygdala shows a disorderly arrangement of enlarged and dysmorphic neurons with a focal back-to-back arrangement. No balloon neurons are seen. No evidence of tumor is seen. The features are of amydgala dysplasia.

3. Serial sections of hippocampus show mild reduction in the numbers of neurons at the interface between CA1 and CA2. There is preservation of neurons in CA3 and CA4. The dentate fasciculus appears unremarkable with preservation of neuronal numbers and no evidence of dispersion. No evidence of tumor is seen.

DIAGNOSIS:

  • "Temporal cortex": Mild Chaslin's sclerosis only.
  • "Amygdala": Amygdala dysplasia
  • "Hippocampus": Mild hippocampal sclerosis (Blumcke Type 2)

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