Faculty Bibliography

Paraneoplastic cerebellar degeneration (PCD) is a rare disorder that is associated with lung or gynecological malignancies and Hodgkin lymphoma. Neurologic symptoms are commonly the initial presenting sign leading to the diagnosis of an underlying malignancy. We are presenting an Asian male with progressive lower extremity weakness with EBV-positive nasopharyngeal carcinoma (NPC) and anti-Yo antibodies. Peculiarly, transient diffuse leptomeningeal enhancement is seen on MR imaging. This is the first report of PCD associated with NPC and thus illustrates that PCD embodies a boarder set of disease than previously described.

BACKGROUND AND OBJECTIVES/OBJECTIVE:Hyperekplexia and the stiff-man syndrome (SMS) are both conditions with exaggerated startle suggesting abnormal brainstem function. Investigation of brainstem reflexes may provide insight into disturbed reflex excitation and inhibition underlying these movement disorders. PATIENTS AND METHODS/METHODS:Using four-channel EMG, we examined four trigeminal brainstem reflexes (monosynaptic masseter, masseter inhibitory, glabella, and orbicularis oculi blink reflexes) and their spread into pericranial muscles in five patients with familial hyperekplexia (FH), two with acquired hyperekplexia (AH), 10 with SMS, and 15 healthy control subjects. RESULTS:Both FH/AH and SMS patients had abnormal propagation of brainstem reflexes into pericranial muscles. All patients with hyperekplexia showed an abnormal short-latency (15-20 ms) reflex in the trapezius muscle with a characteristic clinical appearance ("head retraction jerk") evoked by tactile or electrical stimulation of the trigeminal nerve, but normal monosynaptic masseter reflexes. Inhibitory brainstem reflexes were attenuated in some FH/AH patients. Four of 10 patients with SMS had similar short-latency reflexes in the neck muscles and frequently showed widespread enhancement of other excitatory reflexes, reflex spasms, and attenuation of inhibitory brainstem reflexes. CONCLUSION/CONCLUSIONS:Reflex excitation is exaggerated and inhibition is attenuated in both stiff-man syndrome and familial or acquired hyperekplexia, indicating a physiological relationship. Reflex transmission in the brainstem appears biased towards excitation which may imply dysfunction of inhibitory glycinergic or GABAergic interneurons, or both.

BACKGROUND:There are no data describing a relation between the course of blood pressure and outcome in acute ischemic stroke. We found such a link in a chart review, and then used that data to design a pilot study. We report on the chart review and the first 13 patients enrolled. METHODS:We examined charts of patients admitted to our service in 2000 and stratified them by good (Rankin scale < 4 on discharge, meaning functional independence with a device or better) or poor outcome. A significant difference in blood pressure course and outcome was found, and we then designed a pilot study in an attempt to reproduce these findings. RESULTS:Analysis of the chart review data from 54 patients revealed that the course of mean arterial pressure was different in the two groups: the mean arterial pressure in the group with good outcome decreased about 2 mmHg daily while there was no change in the group with poor outcome (p=0.002 for a difference between slopes). In the pilot trial there were no differences between mean arterial pressure parameters or outcome in either the placebo or treatment groups. Nearly all patients with an admission NIH Stroke Scale score of 6 or less were discharged home. CONCLUSIONS:While our retrospective data were suggestive, we were unable to reproduce findings linking gradual control of blood pressure and outcome in acute ischemic stroke. This pilot study may be used to refine future trials.

Intra-arterial (IA) recombinant tissue plasminogen activator (rt-PA) is an investigational treatment for acute stroke. We report a case of IA thrombolysis of a hyperacute middle cerebral artery stroke 5 days after coronary artery bypass graft surgery. Despite a serious extracranial bleeding complication (hemothorax), immediate thrombolysis with IA rt-PA led to near complete resolution of the neurological deficit and a favorable outcome.