Hypermotor seizures in patients with temporal pole lesions.

Hypermotor seizures are considered to be characteristic of frontal lobe epilepsy, with only rare occurrence in temporal lobe epilepsy. After noting hypermotor seizures in several patients with lesions involving the pole of the temporal lobe, we investigated whether temporal pole lesions were associated with hypermotor seizures. We systematically reviewed medical records, MRI images and pathological findings in consecutive patients who underwent epilepsy surgery over the preceding 10 years in our institution and identified eight patients with temporal pole lesions and intractable complex partial seizures. We analyzed all recorded seizures for semiology, classifying seizures as hypermotor or typical "psychomotor." Four patients exhibited hypermotor seizure semiology and four patients manifested typical psychomotor seizure characteristics. In patients with hypermotor seizures, scalp EEG tended to demonstrate lateral anterior temporal ictal onset, with lesser involvement of the sphenoidal electrode, while the patients with psychomotor seizures had initial inferomesial temporal rhythmic theta activity. Two patients with hypermotor seizures had implanted frontal and temporal subdural grids demonstrating orbitofrontal spread before hypermotor behavior. Patients underwent either anterior temporal lobectomy or lesionectomy. All improved considerably, with six patients seizure-free since surgery. We conclude that hypermotor seizures occur frequently in patients with temporal pole lesions. A search for temporal pole pathology is recommended for patients with hypermotor seizures and temporal epileptiform discharges. Modification of the surgical approach to include this region should be considered in patients who exhibit hypermotor seizures.

An 80-year-old man with a ring-enhancing right basal ganglia lesion.

Cerebral phaeohyphomycosis is a rare diagnosis that designates a central nervous system (CNS) infection by dematiaceous fungi. These organisms most commonly cause cutaneous infections in humans, but much less commonly, they cause CNS disease with evidence of neurotropism. We describe here the clinical course and post-mortem findings in a fatal case of cerebral phaeohyphomycosis occurring in an 80-year-old man. He had a long and complex past medical history and approximately 7 weeks prior to his death, he presented to an outside institution with imaging findings reported to be consistent with a cerebrovascular accident. He was treated with thrombolytic therapy and sent to a rehabilitation program. Approximately 2 weeks prior to his death, he was transferred to our institution with worsening chronic heart failure symptoms. Imaging after admission showed a ring-enhancing lesion and the differential diagnosis shifted to include a primary neoplasm vs. an abscess. There was a downward clinical course and neurosurgical biopsy was declined secondary to predicted poor outcome. A full autopsy was performed and confirmed the pre-mortem imaging findings of a cerebral abscess with multiple satellite lesions. The histologic and microbiologic findings were characteristic of cerebral phaeohyphomycosis. Microbiological features and disease characteristics of these organisms as well as incidence and populations affected are also discussed.

Lithium treatment prevents neurocognitive deficit resulting from cranial irradiation.

Curative cancer treatment regimens often require cranial irradiation, resulting in lifelong neurocognitive deficiency in cancer survivors. This deficiency is in part related to radiation-induced apoptosis and decreased neurogenesis in the subgranular zone of the hippocampus. We show that lithium treatment protects irradiated hippocampal neurons from apoptosis and improves cognitive performance of irradiated mice. The molecular mechanism of this effect is mediated through multiple pathways, including Akt/glycogen synthase kinase-3beta (GSK-3beta) and Bcl-2/Bax. Lithium treatment of the cultured mouse hippocampal neurons HT-22 induced activation of Akt (1.5-fold), inhibition of GSK-3beta (2.2-fold), and an increase in Bcl-2 protein expression (2-fold). These effects were sustained when cells were treated with lithium in combination with ionizing radiation. In addition, this combined treatment led to decreased expression (40%) of the apoptotic protein Bax. The additional genes regulated by lithium were identified by microarray, such as decorin and Birc1f. In summary, we propose lithium treatment as a novel therapy for prevention of deleterious neurocognitive consequences of cranial irradiation.

A combined deficiency of vitamins E and C causes severe central nervous system damage in guinea pigs.

A short period of combined deficiency of vitamins E and C causes profound central nervous system (CNS) dysfunction in guinea pigs. For this report, CNS histopathology was studied to define the nature and extent of injury caused by this double deficiency. Weanling guinea pigs were fed a vitamin E-deficient or -replete diet for 14 d. Then vitamin C was withdrawn from the diet of some guinea pigs. Four diet groups were thus formed: replete, vitamin E deficient, vitamin C deficient, and both vitamin E and C deficient. From 5 to 11 d after institution of the doubly deficient diet, 9 of 12 guinea pigs developed paralysis, and 2 more were found dead. The remaining guinea pig in the doubly deficient group and all animals in the other 3 groups survived without clinical impairment until the experiment was terminated at 13-15 d. Brains and spinal cords were serially sectioned and stained for examination. Only the combined deficiency produced damage in the CNS. The damage consisted mainly of nerve cell death, axonal degeneration, vascular injury, and associated glial cell responses. The spinal cord and the ventral pons in the brainstem were most severely affected, often exhibiting asymmetric cystic lesions. Several features of the lesions suggest that the primary damage was to blood vessels. These results indicate that the paralysis and death caused by combined deficiency of vitamins E and C in guinea pigs is caused by severe damage in the brainstem and spinal cord.