Demographic and clinical summary of previously reported cases of cortical multiple sclerosis
| Ref No | Age/sex | Initial presentation | Paraclinical finding | Follow up period after presentation and progress |
|---|---|---|---|---|
| CSF, cerebrospinal fluid; CT, computed tomography; EEG, electroencephalogram; F, female; INO, internuclear ophthalmoplegia; IQ, intelligence quotient; LAEG, lumbar air encephalogram; M, male; MRI, magnetic resonance imaging; OCB, oligoclonal bands; VER, visual evoked response; WM, white matter. | ||||
| 13 | 19/ F | Subacute onset of intermittent confusion plus social withdrawal and epilepsy | VER: delayed; CSF: pleocytosis | 5 years; bilateral optic atrophy, complete deafness, hemi-pyramidal weakness, reduced sensation on T10 to L4 distribution bilaterally |
| 23/F | Subacute episode of depression, leading to mutism and change in personality | VER: delayed; CSF: pleocytosis, increased IgG; EEG: bilateral slow waves | 2 years; ataxia and sphincter involvement responded to steroids with complete recovery within 4 months; developed sensory and visual symptoms plus extensor plantar responses | |
| 24/F | Depression plus disorientation associated with poor memory and lack of insight | VER: delayed; CSF: high protein; CT or MRI (?): cerebral atrophy and low density periventricular flares; EEG: bilateral slow waves | 7 years; impaired verbal and performance IQ, change in personality, recovered over 7 months; developed a subacute amnesia plus epilepsy, dysgraphia, and hemipyramidal weakness | |
| 14 | 30/F | Anxiety, depression, reduced global cognitive function | VER: not done; CSF: increased IgG; LAEG: cortical atrophy; EEG: bilateral slow waves | 4 years; reduced verbal and performance IQ, dysphagia, diplopia and urinary incontinence. Bilateral optic atrophy, nystagmus, dysarthria, bilateral facial palsy, hyper-reflexia and ataxia. |
| 35/M | Anxiety, depression, clumsiness, slurred speech, poor memory, euphoria | VER: not done; CSF: increased IgG; LAEG: cortical atrophy | 1 year; bilateral optic atrophy, vertical nystagmus, long tract signs cerebellar ataxia reduced verbal intelligence, progressive amnesia | |
| 40/M | Episodes of anxiety, depression, perseveration, poor attention, memory deficit, dysgraphia | VER: not done; CSF: increased IgG; LAEG: cortical atrophy; EEG: bilateral slow waves | 12 years; facial palsy, slurred speech and gait ataxia, dizziness, nystagmus, clumsiness, dyscalculia, quadriparetic spasticity | |
| 35/M | Depression, poor memory, inappropriate hunger, aggressive behaviour | VER: not done; CSF: increased IgG and pleocytosis; LAEG: cortical atrophy; EEG: bilateral slow waves | 8 months; optic atrophy, gait ataxia, progressive cognitive impairment , dyscalculia, dysphasia, lack of insight, reduced verbal and performance intelligence, severe memory impairment, nominal dysphasia | |
| 39/M | “Hysterical” type symptoms, progressive psychotic depression, limb trembling | VER: not done; CSF: increased IgG; LAEG: cortical atrophy | 9 years; optic atrophy, dyslexia, limb ataxic, long tract signs, progressive cognitive impairment characterised by impaired memory and non-verbal cognitive skills | |
| 15 | 41/F | Depression | VER: not done; CSF: increased IgG; no imaging | 5 years; nystagmus, cerebellar signs, neuropsychological deficit (dyspraxia and somatognosia) suggesting cortical dysfunction |
| 33/F | Depression, “histrionic” personality | VER: not done; CSF: increased IgG; no imaging | 15 years; optic neuritic, neuropsychological deficit including constructional apraxia suggesting cortical dysfunction | |
| 32/F | Depression, medically “unexplained” symptoms, eg, dizziness, blurred vision, “off balance” with no neurological signs | VER: not done; CSF: increased IgG; no imaging | Nystagmus, cerebellar ataxia, neuropsychological deficit suggesting right temporo-parietal dysfunction | |
| 16 | 37/M | Depression, memory loss (Korsakoff type) | VER: delayed; CSF: OCB−; MRI (10 years after initial presentation): bilateral high signal in temporal lobes and periventricular area | 13 years; cognitive impairment, personality change, INO, ataxia, incontinence, visual loss |
| 17 | 37/F | Depression | VER: delayed; CSF: OCB+; MRI: cerebral atrophy + WM high signal changes | 8 months; impaired visuospatial skills, frontal lobe dysfunction, impaired abstract thinking, concept formation, judgment, apraxis, poor attention, long tract signs, posterior column signs |
| 18 | 41/M | Change in personality, forgetfulness, labile indifference and poor verbal memory | VER: delayed; CSF: OCB+, MRI: WM high signal changes; EEG: diffuse slow activity | 8 years; impaired verbal fluency, impaired verbal and performance IQ, visuospatial deficit, left/right disorientation, dyscalculia, dysgraphia, urinary incontinence, wheelchair bound and incontinent |
| 35/M | Memory decline, poor concentration | VER: delayed; CSF: OCB+; MRI: WM high signal changes; EEG: diffuse slow activity | 4.5 years; personality change, poor verbal memory, dyscalculia, reduced verbal fluency, bilateral optic atrophy, gait ataxia, severe cognitive impairment | |
| 19 | 43/M | Acute onset of psychotic depression leading to attempted suicide | VER: delayed; CSF: OCB+; MRI: WM high signal changes | 3 months; responded to intravenous methylprednisolone, nystagmus, extensor plantar respnses |
| 20 | 43/F | Depression leading to suicidal attempt, impaired remote memory | VER: delayed; CSF: OCB+; MRI: cerebral atrophy + WM changes | 2.5 years; long tract signs, gait ataxia, sphincter involvement |