Loss of Purkinje Cells is Associated with Demyelination in Multiple Sclerosis

Multiple sclerosis (MS) is an inflammatory demyelinating disease of the central nervous system (CNS) previously thought to involve mainly the white matter.In the past 10-15 years, pathology and MRI studies have shown that in addition to white matter lesions, there is significant involvement of the gray matter as well as significant axonal and neuronal loss 1 .Involvement of the cerebellar cortex is not commonly described in MS, although in a recent report Kutzelnigg et al have described extensive demyelination in the cerebellar cortex which was associated with relative axonal and neuronal preservation 2 .Here, we report Purkinje cell loss in two cases of multiple sclerosis, one of the classical Charcot variant and the other of the acute Marburg variant.At age 30, a male patient developed right hemianesthesia and paresthesiae which resolved within a few weeks.Five years later he complained of numbness of both hands and decreased vision in the left eye.At this time, visual evoked responses were abnormal and MRI of the brain was suggestive of MS.Later during the same year, the patient complained of balance problems that made it difficult to jump on and off trains at his vocation as train engineer.By the end of the second year the patient had spastic paraparesis with extensor plantar responses.His gait deteriorated and he developed spastic quadriparesis.Seven years after the onset the patient was in a wheelchair and had significant difficulty standing up or transferring in and out of bed or on and off the toilet.A repeat MRI showed extensive white matter changes in the cerebral hemispheres but no disease in the cerebellum although the right middle cerebellar peduncle was affected.After ten years of MS, the patient had developed pendular nystagmus in both eyes, tremor and dysarthria, severe contractures, multiple and extensive decubitus ulcers.He died with aspiration pneumonia at age 49.A post mortem examination of the nervous system confirmed the diagnosis of MS affecting the cerebral hemispheres and the corpus callosum, the thalamus and hypothalamus, the brain stem, the spinal cord and the cerebellum.In the cerebellum, Purkinje cells loss was associated with focal demyelination in adjacent folia (Figure 1).A 17-year-old girl suddenly lost the vision in her right eye.During the subsequent two weeks she developed numbness of her left arm and leg.Within three months she developed bilateral visual loss, nausea and vertigo, and profound loss of hearing in both ears.Asymmetrical weakness, worse on the right side, and urinary retention quickly developed.Nystagmus of high amplitude was present on conjugate gaze to the left and to the right and hearing was absent in the left ear and reduced in the right.Asymmetrical quadriparesis was present.Bilateral lower limb ataxia was worse on the left side.Cerebral spinal fluid

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