[Baló's concentric sclerosis]

P Castaigne, R Escourolle, F Chain, J F Foncin, F Gray, B Sauron, C Duyckaerts
Revue Neurologique 1984, 140 (8-9): 479-87
A clinico-pathological case of concentric sclerosis (Baló type) is reported. A 30-year-old man experienced dizziness, nausea and vomiting. Twelve days later he developed gait disturbances. Neurological examination showed broad based gait, brisk tendon reflexes, bilateral extensor plantar responses, right hemihypoesthesia, cerebellar dysmetria, and a left lateral gaze palsy. CSF examination showed, 520 mg p. 100 ml protein, 7500 red blood cells, 31 lymphocytes and 9 polymorphonuclear leukocytes/mm3, 18 p. 100 gammaglobulin. Three CT scans were performed and showed a round hypodensity in the parieto-occipital white matter with contrast enhancement on one occasion, and several other hypodensities in the contralateral parieto-occipital white matter and in both frontal lobes. 23 days after the onset of the disease, the patient became comatose. A cerebral biopsy was obtained from one of the frontal lesions. He died from aspiration bronchopneumonia 2 months after the first signs. Neuropathological examination showed numerous concentric zones of demyelination which involved the white matter of both hemispheres, brain stem, and cerebellum. On light microscopy sudanophilic myelin breakdown products were numerous in the bands of demyelinisation. Astrocytic proliferation was marked, with frequent Rosenthal fibers. Edema was noted in some lesions. Myelin-axonal dissociation was obvious, but some axonal swelling were observed. Electron microscopy demonstrated the integrity of oligodendrocytes and of blood vessels and confirmed the prominent alterations of the astrocytes. Fifteen similar cases of the literature have been reviewed. The present case seems to be the first one with CT scan examination and electron microscopic study of a brain biopsy. The nosological situation of Baló's disease among the inflammatory demyelinating diseases of the group of MS is discussed.

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