TABLE 1.
Surgical biopsy and autopsy data
| Disease and patient no. | Patient age (yr) | Sex | Tissuea | Postmortem interval (h) |
|---|---|---|---|---|
| Acute MSb | ||||
| 1 | 19 | F | B | |
| 2 | 26 | F | B | |
| Diffuse sclerosisc | ||||
| 3 | 11 | M | A | NAd |
| Chronic MS | ||||
| 4 (CPe) | 66 | M | A | 3 |
| 5 (RRf) | 40 | F | A | 2 |
| 6 (CP) | 42 | M | A | 4 |
| 7 (RR) | 82 | F | A | 2.5 |
| 8 (CP) | 54 | F | A | NA |
A, autopsy specimen; B, biopsy specimen.
The acute-MS patients were diagnosed based on the following findings: acute monophasic lesion(s) involving predominantly cerebral white matter; increased T2-weighted signal pattern on magnetic resonance imaging; exhibition in both patients of a mass effect with internal shift; on patient followup, abnormal visual evoked potential; CSF IgGs showing oligoclonal banding; detection in biopsy specimens of acute demyelinating lesions (of the same age) accompanied by variably prominent perivascular and parenchymal mononuclear infiltrates and reactive astrocytosis; preservation of axons; lack of gray matter involvement.
Rapidly progressive, essentially unremitting demyelinating disease was diagnosed in an 11-year-old African-American male. Clinically, there was predominant cerebral hemispheric white matter involvement. Magnetic resonance imaging exhibited increased signal on T2-weighted images. Optic neuritis was evident. The patient died 18 months after the onset of illness. Postmortem neuropathology included extensive, confluent-to-diffuse demyelinating lesions involving the cerebral hemispheric white matter, bilaterally, and virtually all funiculi of the spinal cord. There was no evidence of dysmyelinating disorder in the context of leukodystrophy. The adrenal glands, visceral organs, peripheral nervous system, and musculoskeletal system were unremarkable, and there was no evidence of mitochondrial cytopathy.
NA, not available.
CP, chronic progressive.
RR, remitting relapsing.