Demographic and clinical summary of the cases described
| Case | Age/sex | Initial symptoms | Paraclinical findings | Follow up |
|---|---|---|---|---|
| CSF, cerebrospinal fluid; F, female; M, male; MRI, magnetic resonance imaging; OCB, oligoclonal bands; INO, internuclear ophthalmoplegia; VER, visual evoked response. | ||||
| 1 | 47/F | 2 year history: progressive amnesic syndrome, emotional lability and depression. | VER: delayed | 3 years; spastic paraplegia, dysarthria, optic atrophy, and urinary incontinence; wheelchair-bound, full time care, severe cognitive impairment |
| CSF: OCB + | ||||
| MRI: diffuse and marked cerebral atrophy | ||||
| Brain biopsy: very mild hypercellularity and moderate diffuse microglial activation with no evidence of vasculitis, leucodystrophy, or progressive multifocal leucoencephalopathy | ||||
| Neuropsychology: impaired retrograde memory, poor verbal fluency, anomia, and dyscalculia | ||||
| 2 | 52/F | 3 year history: depressive illness + progressive amnesic syndrome; difficulty in autobiographical and semantic memory and face recognition | VER: delayed | 4 years; optic atrophy, continued to deteriorate cognitively |
| CSF: OCB+ | ||||
| MRI: periventricular high signal changes | ||||
| SPECT: bilateral (R>L) hypoperfusion of posterior parietal cortices | ||||
| Neuropsychology: dyscalculia, dysgraphia, visuo-constructive and visuo-spatial dysfunction, impaired anterograde and retrograde memory | ||||
| 3 | 60/F | 5 year history: progressive amnesia, inattention, and personality changes | VER: normal | 2 years; cerebellar ataxia, INO, bladder involvement, blindness, and progressive cognitive impairment |
| CSF: OCB− | ||||
| MRI: cortical atrophy with multiple high signal lesions in white matter | ||||
| SPECT: marked hypoperfusion of cortical perfusion particularly in frontal regions | ||||
| Neuropsychology: anterograde and retrograde memory impairment, reduced digit span, reduced verbal fluency, and mild arithmetic and visuospatial difficulty | ||||
| 4 | 38/F | 15 months history: progressive amnesic syndrome and difficulty in writing, reading, spelling, use of words associated with depression, and later ataxia and alien limb phenomenon | VER: delayed | 18 months; severely demented, without verbal communication, blind, spastic quadriparesis, doubly incontinent and required full care |
| CSF: OCB− | ||||
| Brain MRI: marked cortical atrophy with widespread periventricular high signal lesions | ||||
| Brain biopsy: no evidence of vasculitis, leucodystrophy, or progressive multifocal leucoencephalopathy | ||||
| Neuropsychology: severe impairment of retrograde memory, comprehension, attention, working memory, and verbal fluency together with marked executive dysfunction, constructional apraxia, and pervasive psychomotor retardation but normal visual-perceptual skills | ||||
| 5 | 42/F | 4 year history: recurrent depression, before signs and symptoms of cerebellar dysfunction | VER: delayed | 2 years; developed INO, spastic paraparesis, marked cerebellar signs and bladder instability, as well as progressive amnesic syndrome |
| CSF: OCB+ | ||||
| MRI: bilateral high signal changes in the cerebrum, corpus callosum, and cerebellum | ||||
| Neuropsychology: impairment of anterograde and semantic memory and poor abstract reasoning | ||||
| 6 | 57/M | 3 year history: depression, episodes of confusion, disorientation, delusional ideation, memory impairment, preceding an episode of ataxia, double vision and unilateral facial palsy | VER: normal | 3 years; bilateral pyramidal signs with extensor plantar response; severe cognitive deterioration requiring nursing home care |
| CSF: OCB+ | ||||
| MRI: marked cortical atrophy with widespread periventricular high signal lesions | ||||
| SPECT: hypoperfusion of both frontal and temporal regions | ||||
| Neuropsychology: impaired verbal fluency and anterograde memory suggestive of fronto-temporal dysfunction | ||||