Faculty Bibliography

Activated T cells are implicated in the pathogenesis of multiple sclerosis (MS), an autoimmune demyelinating disease of the central nervous system. Serial measurements of T cell activation molecules and T cell subpopulations were performed over 12 months in patients with chronic progressive multiple sclerosis and healthy controls, and correlated with clinical indices of disease progression measured by standardized disability scores. Of the markers studied, the activation molecule CD26 appeared to exhibit a more consistent pattern and to be elevated in MS patients; therefore we concentrated our attention on this marker, especially in view of recent evidence of its role in T cell activation. In this small patient group, the elevation of CD26 in the MS patients did not reach statistical significance, when compared to the level in the controls. Interestingly, the percentage of CD26 but not CD25 or HLA-DR correlated with the MS patients' disability scores. However, the clinical significance of this observation as an indication of disease activity in chronic progressive MS remains to be demonstrated in studies of larger patient populations.

In this article, the crossed syndromes of Millard-Gubler (facial palsy and contralateral hemiparesis), Foville (facial palsy, conjugate gaze paralysis, and contralateral hemiparesis), Weber (oculomotor palsy and contralateral hemiparesis), and Raymond-Cestan (internuclear ophthalmoplegia and contralateral hemiparesis) are detailed from the original reports. These and other related syndromes were instrumental in establishing important principles in brain-stem localization: the occurrence of cranial nerve palsies contralateral to hemibody motor or sensory disturbances, the concepts of the medial longitudinal fasciculus and conjugate gaze, and the corticobulbar innervation of the facial nerve nucleus.

Ten patients with migraine developed persistent positive visual phenomena lasting months to years. The complaints were similar in their simplicity and involvement of the entire visual field and usually consisted of diffuse small particles such as TV static, snow, lines of ants, dots, and rain. Neurologic and ophthalmologic examinations were normal, and EEGs were normal in eight of eight patients tested. MRI was normal in all patients except one who had nonspecific biparietal white matter lesions and another with a small venous angioma. Treatment of this unusual complication of migraine was unsuccessful.

We report a patient who developed conjugate horizontal gaze deviation and ipsipulsion of saccades from a lateral medullary infarction. Recent evidence suggests that the gaze deviation may result from increased inhibition of the ipsilateral vestibular nucleus and ipsipulsion of saccades from decreased excitation of the contralateral ocular premotor areas of the brainstem reticular formation. Interruption of the olivocerebellar pathways may account for both of these ocular motor abnormalities.

Stroke is the third leading cause of death in the United States. Efforts directed at reversing acute cerebral ischemia are promising but are hampered by multiple logistic and physiologic barriers. Prevention of stroke, therefore, remains of critical importance. Primary prevention is accomplished through reduction of risk factors and the appropriate use of warfarin or aspirin in patients with cardiac sources of emboli such as atrial fibrillation. Secondary prevention is designed to reduce the risk of stroke in patients with known stroke precursors, including transient ischemia, reversible ischemic deficits, and completed stroke. Aspirin and ticlopidine are two antiplatelet agents with an established role in secondary stroke prevention. In a major North American clinical trial, ticlopidine demonstrated superior efficacy to aspirin for the prevention of recurrent stroke, particularly in the first year following a stroke. Dipyridamole has not been shown to be useful for stroke prevention. The role of warfarin in the prevention of recurrent noncardiogenic stroke remains controversial and is currently under investigation. Stroke prevention remains an important challenge, and therapy should be individualized to achieve optimal results.

An 11-year-old boy with tuberculous meningitis developed blindness, a rare complication of this disease in the United States. Magnetic resonance imaging demonstrated perichiasmal enhancement, suggesting that arachnoiditis caused the visual loss. Serial neuroimaging over 7 months revealed a persistent inflammatory process in the chiasmatic cistern, hydrocephalus, and progressive cerebral infarctions.

A 21-year-old woman presented with a two year history of progressive loss of vision in the left eye. Brain MRI revealed a supresellar mass felt to be most consistent with a meningioma. However, pathologic examination including special stains disclosed features characteristic of hemangioblastoma. Further evaluation established the diagnosis of von Hippel-Lindau disease by demonstrating retinal capillary hemangiomas, small renal and hepatic cysts, and cervico-medullary masses later confirmed to be hemangioblastomas. To date, no other family members have displayed features of this inherited syndrome.

Critical care neurology is a rapidly developing subspecialty of neurology. The neuro-intensivist is called upon to manage critically ill patients with stroke, neuromuscular disease, traumatic injury, increased intracranial pressure, and other disorders. Therapeutic and technologic advances are rapidly being incorporated into practice and will improve patient care and outcome.