Sam U. Ho

Multifocal central nervous system damage caused by toluene abuse.

Four toluene abusers had evidence of severe multifocal central nervous system damage. Impairment of cognitive, cerebellar, brainstem, auditory, and pyramidal tract function, as well as CT evidence of cerebral cortical, cerebellar, and brainstem atrophy, have been noted. In addition, we found opsoclonus, ocular flutter, and ocular dvsmetria. All three patients tested had abnormal brainstem auditory evoked potentials, indicative of brainstem dysfunction. The patient with opsoclonus had CT evidence of brainstem, cerebellar, and cerebral cortical atrophy.

Autologous non-myeloablative haematopoietic stem cell transplantation for refractory systemic vasculitis.

Objective: For patients with systemic vasculitis (SV) refractory to conventional therapy, new treatment strategies aimed at aggressive induction of remission and relapse prevention are being sought. We herein report our single-centre experience in treating four patients with refractory SV employing non-myeloablative autologous haematopoietic stem cell transplantation (HSCT). Methods: Four patients with refractory SV (two with neurovascular Behcet disease, one with neurovascular Sjögren syndrome, and one with Wegener granulomatosis) were involved in an Institutional Review Board (IRB) and US Food and Drug Administration (FDA) approved phase I clinical trial of high dose chemotherapy and autologous HSCT. Peripheral blood stem cells were mobilised with cyclophosphamide (Cy) and granulocyte-colony stimulating factor (G-CSF). Conditioning regimen consisted of Cy 200 mg/kg and rabbit anti-thymocyte globulin 5.5 mg/kg intravenously (iv). Results: All four patients tolerated HSCT well without transplant related mortality or any significant toxicity. At median follow-up of 28 (range 22–36) months all patients were alive. Three patients (one with Behcet disease, one with Sjögren syndrome, and one with Wegener granulomatosis) entered a sustained remission at 6, 6 and 24 months, respectively, after transplant. They had significant decrease in disease activity and disease or treatment related damage, as measured by the Birmingham Vasculitis Activity Score and Vasculitis Damage Index, respectively. All three patients who achieved remission discontinued immunosuppressive therapy at the time of transplant and have not required treatment since. One patient with Behcet disease and positive for human leukocyte antigen (HLA)-B51 has not improved after HSCT. Conclusion: We suggest non-myeloablative autologous HSCT is an alternative therapy for select patients with SV refractory to conventional immunosuppressive therapies.

Multiple Brain Abscesses

A young woman with 12 separate brain abscesses was treated medically after aspiration of one abscess for diagnostic bacteriological examination. She made an excellent recovery with only minimal residual neurological dysfunction. Surgical aspiration for detailed bacteriological studies followed by appropriate antimicrobial therapy is an effective way of treating multiple brain abscesses in the neurologically stable patient.

Cerebellar infarction: A clinical and CT study

Seven patients with computed tomographic (CT) evidence of cerebellar infarction were found during review of all CT records over a 5-year period. CT demonstrated decreased density in 2 distinct anatomic areas, corresponding to the distribution of the posterior inferior cerebellar artery and the superior cerebellar artery. Some patients also had hydrocephalus and fourth ventricular effacement and displacement. The location and extent of infarction do not relate directly to the clinical outcome. Rapid deterioration of the patients sensorium is the best indication for surgical decompression.

CT scan in interhemispheric subdural hematoma Clinical and pathological correlation

In a patient with a large interhemispheric subdural hematoma, the CT scan showed a radiodensity with a straight sagittal and a convex lateral border. This image correlated with the autopsy findings. It differs from CT scan images of intracerebral hematomas.

Sarcoid encephalopathy with diffuse inflammation and focal hydrocephalus shown by sequential CT

Sequential computerized tomography (CT) was performed on a patient with seizures and an organic mental syndrome. Cerebral sarcoidosis was subsequently diagnosed on the basis of nonca-seating granulomas in three organ systems. Cranial nerve palsies, hypothalamic dysfunction, and widespread disease were absent. An unusual neuroradiologic sequence of events indicated focal and asymmetric hydrocephalus, mass effect, ependymitis, diffuse breakdown of the blood- brain barrier, and periventricular white matter destruction. These findings have not been previously described together in a patient with sarcoid. With the advent of noninvasive CT, it is possible to perform serial investigations of encephalopathies of obscure origin. Findings similar to those in the present case should prompt a thorough search for subclinical systemic involvement by sarcoid.

Pulfrich stereo-illusion phenomenon: Poor man's VEP?

he Pulfrich effect reflects the interocular latency difference subjectively. The VEP testing provides quantitative measures of the absolute transmission time in each eye and also detects subtle interocular latency difference better than Pulfrich. However, when the Pulfrich effect is present, VEP is always expected to be abnormal. Moreover, the Pulfrich effect reliably identifies the involved eye. When the Pulfrich effect fails to be abnormal, VEP may still be abnormal.This test can be used as a screening procedure to detect optic nerve disease since it is simple, quick, and less expensive than VEP.