|Contributed by Zissimos Mourelatos MD1, Michael McGarvey MD2, Jacqueline A. French MD2, and Gregg Wells MD PhD1|
|1Division of Neuropathology, Department of Pathology, and 2Department of Neurology, University of Pennsylvania Medical Center, Philadelphia, PA|
|Published on line in October 2002|
The patient was a 27 year-old woman who was first diagnosed with partial complex seizures at age 7. Despite multiple trials of various anti-epileptic drugs the seizures were difficult to control. At age 20 her seizures changed in character. She was experiencing multiple daily episodes of left-sided face, arm and leg twitching without impairment of consciousness and she also developed a slowly progressive left sided weakness. She had multiple hospitalizations for focal motor status epilepticus.
Her past medical history was remarkable for asthma and depression. She had a normal early childhood with no history of febrile seizures, meningoencephalitis or head trauma. She graduated from high school and worked as a nursing assistant but was forced to quit due to her seizures. Her family history was noncontributory.
Neurological examination upon a recent hospital admission showed episodes of left facial twitching every 5 minutes. Examination of higher mental functions showed dyscalculia and possible left neglect. Her memory, orientation to place, person and time were intact. Cranial nerve examination was significant for left homonymous hemianopia and mild left facial droop. Motor examination revealed a mild left hemiparesis while examination of the sensory systems showed decreased sensation to pinprick on the left. Motor reflexes were normal with down-going toes bilaterally. Motor coordination was poor bilaterally and her gait was very unsteady. Neuroimaging studies were performed and the results compared to previous studies, and showed progressive right hemispheric atrophy and contralateral cerebellar atrophy. A biopsy of the right temporal lobe tip was performed and showed marked gliosis and neuronal loss and focal perivascular and intraparenchymal lymphocytic infiltrates.
The patient was placed on multiple anti-epileptic drugs, with relative control of her seizures, and was scheduled for a surgical procedure. Unfortunately she expired at home during her sleep a week later.
The brain shows a striking pattern of cerebral atrophy confined to the right hemisphere with contralateral (left) cerebellar atrophy; the right temporal pole had been previously surgically removed. Fig 1 shows the basal surface of the brain after removal of the brainstem and cerebellum; fig 2 shows the right lateral aspect with atrophic right cerebral hemisphere and normal right cerebellar hemisphere; fig. 3 shows the left lateral aspect with normal left cerebral hemisphere and atrophic left cerebellar hemisphere. Fig. 4 shows right cerebral cortical and white matter atrophy and severe atrophy of the right caudate nucleus with secondary enlargement of the right lateral ventricle. Fig. 5 shows atrophy of the cerebral cortex and white matter, and atrophy of the right thalamus, subthalamic nucleus, lenticular nucleus, tail of the caudate and hippocampus with secondary enlargement of the right ventricular system. Fig. 6 shows atrophy of the right parieto-occipital lobes with secondary enlargement of the occipital horn of the right ventricle. Fig. 7 shows atrophy of the left cerebellar hemisphere and left dentate nucleus.