Table 2

Demographic and clinical summary of previously reported cases of cortical multiple sclerosis

Ref NoAge/sexInitial presentationParaclinical findingFollow 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.
1319/ FSubacute onset of intermittent confusion plus social withdrawal and epilepsyVER: delayed; CSF: pleocytosis5 years; bilateral optic atrophy, complete deafness, hemi-pyramidal weakness, reduced sensation on T10 to L4 distribution bilaterally
23/FSubacute episode of depression, leading to mutism and change in personalityVER: delayed; CSF: pleocytosis, increased IgG; EEG: bilateral slow waves2 years; ataxia and sphincter involvement responded to steroids with complete recovery within 4 months; developed sensory and visual symptoms plus extensor plantar responses
24/FDepression plus disorientation associated with poor memory and lack of insightVER: delayed; CSF: high protein; CT or MRI (?): cerebral atrophy and low density periventricular flares; EEG: bilateral slow waves7 years; impaired verbal and performance IQ, change in personality, recovered over 7 months; developed a subacute amnesia plus epilepsy, dysgraphia, and hemipyramidal weakness
1430/FAnxiety, depression, reduced global cognitive functionVER: not done; CSF: increased IgG; LAEG: cortical atrophy; EEG: bilateral slow waves4 years; reduced verbal and performance IQ, dysphagia, diplopia and urinary incontinence. Bilateral optic atrophy, nystagmus, dysarthria, bilateral facial palsy, hyper-reflexia and ataxia.
35/MAnxiety, depression, clumsiness, slurred speech, poor memory, euphoriaVER: not done; CSF: increased IgG; LAEG: cortical atrophy1 year; bilateral optic atrophy, vertical nystagmus, long tract signs cerebellar ataxia reduced verbal intelligence, progressive amnesia
40/MEpisodes of anxiety, depression, perseveration, poor attention, memory deficit, dysgraphiaVER: not done; CSF: increased IgG; LAEG: cortical atrophy; EEG: bilateral slow waves12 years; facial palsy, slurred speech and gait ataxia, dizziness, nystagmus, clumsiness, dyscalculia, quadriparetic spasticity
35/MDepression, poor memory, inappropriate hunger, aggressive behaviourVER: not done; CSF: increased IgG and pleocytosis; LAEG: cortical atrophy; EEG: bilateral slow waves8 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 tremblingVER: not done; CSF: increased IgG; LAEG: cortical atrophy9 years; optic atrophy, dyslexia, limb ataxic, long tract signs, progressive cognitive impairment characterised by impaired memory and non-verbal cognitive skills
1541/FDepressionVER: not done; CSF: increased IgG; no imaging5 years; nystagmus, cerebellar signs, neuropsychological deficit (dyspraxia and somatognosia) suggesting cortical dysfunction
33/FDepression, “histrionic” personalityVER: not done; CSF: increased IgG; no imaging15 years; optic neuritic, neuropsychological deficit including constructional apraxia suggesting cortical dysfunction
32/FDepression, medically “unexplained” symptoms, eg, dizziness, blurred vision, “off balance” with no neurological signsVER: not done; CSF: increased IgG; no imagingNystagmus, cerebellar ataxia, neuropsychological deficit suggesting right temporo-parietal dysfunction
1637/MDepression, memory loss (Korsakoff type)VER: delayed; CSF: OCB−; MRI (10 years after initial presentation): bilateral high signal in temporal lobes and periventricular area13 years; cognitive impairment, personality change, INO, ataxia, incontinence, visual loss
1737/FDepressionVER: delayed; CSF: OCB+; MRI: cerebral atrophy + WM high signal changes8 months; impaired visuospatial skills, frontal lobe dysfunction, impaired abstract thinking, concept formation, judgment, apraxis, poor attention, long tract signs, posterior column signs
1841/MChange in personality, forgetfulness, labile indifference and poor verbal memoryVER: delayed; CSF: OCB+, MRI: WM high signal changes; EEG: diffuse slow activity8 years; impaired verbal fluency, impaired verbal and performance IQ, visuospatial deficit, left/right disorientation, dyscalculia, dysgraphia, urinary incontinence, wheelchair bound and incontinent
35/MMemory decline, poor concentrationVER: delayed; CSF: OCB+; MRI: WM high signal changes; EEG: diffuse slow activity4.5 years; personality change, poor verbal memory, dyscalculia, reduced verbal fluency, bilateral optic atrophy, gait ataxia, severe cognitive impairment
1943/MAcute onset of psychotic depression leading to attempted suicideVER: delayed; CSF: OCB+; MRI: WM high signal changes3 months; responded to intravenous methylprednisolone, nystagmus, extensor plantar respnses
2043/FDepression leading to suicidal attempt, impaired remote memoryVER: delayed; CSF: OCB+; MRI: cerebral atrophy + WM changes2.5 years; long tract signs, gait ataxia, sphincter involvement