A review of neurophysiological testing.

The rapid advances in the technology of, and accumulation of pertinent data in, electrophysiological testing has increased exponentially in the past decade. This is attributable to continued advances in computer technology, biomedical engineering, and now the coregistration of the electrophysiological data with neuroimaging results. Knowledge of normal function and electrophysiological response at rest or on stimulation of the central and peripheral nervous systems is important to the neurosurgeon. Only by a basic understanding of normal and abnormal recordings may diagnoses and localizations be achieved. Intraspinal and intracranial surgical procedures are predicated on nontrauma to the neuraxis. This can be accomplished by performing electrophysiological testing to monitor the function of the spinal and cranial nerves, spinal cord, brainstem, basal ganglia, and cerebrum. If the surgeon cannot delineate critical cortex or pathways, he or she will be unable to avoid these areas in the patient.

Evolution and rebirth of functional stereotaxy in the subthalamus.

The first human stereotactic surgery based on intracerebral landmarks and Cartesian coordinates was performed in 1947. With this followed the publication of a number of stereotactic frames and atlases. The intercommissural line joining the anterior and posterior commissures was to define stereotactic coordinate systems used in movement disorders and other functional neurosurgical procedures. Initially the target for Parkinson disease was the globus pallidus internus (GPi), but many investigators soon turned to the thalamus or parts of the subthalamus, but not the subthalamic nucleus. Microelectrode recording was introduced in 1961. With the apparent clinical efficacy of L-DOPA in 1965 interest in stereotactic surgery for Parkinson disease declined. The failure of prolonged, consistent pharmacologic management of bradykinesia and tremor, the side effects of dyskinesias, and the fading therapeutic success of medical treatment of movement disorders led to a resurgence of interest in the surgical management of movement disorders. With advances in understanding of the functional anatomy of the corticobasal ganglia circuit, advances in brain imaging, more sophisticated electrophysiologic recordings, and the use of deep brain stimulation as a reversible lesion, stereotactic surgery returned as a viable option for the treatment of movement disorders. The posterior medial part of the globus pallidus, ventral intermediate nucleus of the thalamus, and the subthalamus, its nuclei and pathways, are sites for interrupting pathophysiologic circuits. Not only has this been applied to movement disorders, but to epilepsy, chronic pain, and behavioral disorders.

Focal chronic inflammatory epileptic encephalopathy in a patient with malformations of cortical development, with a review of the spectrum of chronic inflammatory epileptic encephalopathy.

PURPOSE: Chronic cellular inflammation closely associated with epilepsy without an active infection is a hallmark of Rasmussen encephalitis (RE). RE has typical and defining features lacking in other rare epilepsy patients who also have neocortical lymphocytes without an identifiable cause. A patient with malformations of cortical development had an abrupt change in frequency and epileptic focus after 22 years of a stable seizure disorder. Functional neurosurgery yielded a specimen showing a mixed cellular meningoencephalitis in the absence of a demonstrable infection. METHODS: Historical, neurologic, electroencephalographic, pathologic, and literature data were correlated. RESULTS: There was a subarachnoid mixed infiltrate including evidence of dendritic cells in our patient and also cytotoxic T lymphocytes adjacent to karyolytic neurons that corresponded to cells previously demonstrated to damage neurons in RE. Literature review disclosed 42 other cases similar to RE but with heterogeneous findings. The course was more protracted and often more benign than in RE. The inflammation that would have markedly decreased or disappeared in RE over that period was generally still well represented. CONCLUSIONS: Our patient has heterogeneous features similar to, yet with differences from, RE. Literature review of chronic cellular inflammatory epileptic encephalopathy cases also similar to RE discloses important differences that may reflect idiosyncratic reactions and pace of the disease rather than a different disease. Comorbidity factors, genetic population traits, and secondary effects of the seizure disorder may lead to an expansion of the initial site of damage by an autoimmune reaction. These cases might best be grouped, probably along with RE, as secondary autoimmune diseases.