Table 2.
Individual cases with perivenous demyelination
| Case | Age/Sex | Prior Attack | PI/V | Presenting symptoms | Time to maximal deficit (days) | Presenting MRI | IPMSSG criteria at presentation | CSF | Clinical course prior to biopsy/autopsy | Time to biopsy/ autopsy | Path | Treatment | Treatment response | Last EDSS | Clinical course | Onset to death or last follow up |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 25F | No | No | Headache Mild meningismus Encephalopathy DLC Quadriparesis Bilateral optic neuritis | 27 | Diffuse T2-weighted signal involving bilateral medulla, pons, cerebellum, midbrain, left temporal lobe; enhancement uncleara | Yes | WBC 10 PRO 50 GLU NA OCB 2 | NA | Autopsy day 27 | PVD CD +CMA | IVMP PLEX | None | 10 | Monophasic- fatal | 27 days |
| 2 | 67F | No | No | Encephalopathy DLC Quadriparesis Seizure | 20 | Multifocal bilateral large T2-weighted subcortical and cortical lesions without enhancement, mild mass effect | Yes | WBC 46 PRO 63 GLU 82 OCB 0 | NA | Autopsy day 20 | PVD +CMA | IVMP PLEX | Partial; died of cardiac arrest while neurologically improving | 10 | Monophasic- fatal | 20 days |
| 3 | 30F | No | No | Headache Encephalopathy (by 2 weeks) Ataxia Vomiting DLC (by 6 weeks) | 50 | MRI 6 weeks after onset: multiple bilateral small foci of increased periventricular, corpus callosum, brainstem T2-weighted signal minimal enhancement; clear deep grey matter involvement by 9 weeks with progression of lesions still with minimal enhancement | Yes | WBC 8 PRO 248 GLU 70 OCB 0 | Biopsy at 5 weeks followed by progression during treatment with IVMP and PLEX | Biopsy day 39 Autopsy day 50 | PVD +CMA | IVMP PLEX | None | 10 | Monophasic- fatal | 50 days |
| 4 | 37F | No | No | Headache Encephalopathy DLC Gait impairment Urinary incontinence | 2 | NA | Yes | NA | NA | Biopsy day 3 Autopsy day 6 | PVD +CMA | NA | None | 10 | Monophasic- fatal | 6 days |
| 5 | 17F | No | No | Headaches, severe Hemiparesis, left Arm weakness, right Bilateral visual impairment Abulia Encephalopathy DLC Required mechanical ventilation | 3.5 | Extensive areas of increased T2-weighted signal involving temporoparietal regions bilaterally, but also right occipital and left subfrontal areas; mild enhancement moderate mass effectb | Yes | WBC 540 PRO 130 GLU 61 OCB NT | NA | 8 days | PVD -CMA | IVMP Oral glucocortico steroid | Partial | 3.5 | Monophasic-survived | 13.6 years |
| 6 | 62F | No | URI | Headache Encephalopathy DLC Hemiparesis | 3 days | Day 3: numerous large (>2 cm) ill-defined, bilateral fluffy-appearing lesions with mass effect and oedema; faint rim of enhancement in subcortical white matter. Day 24 (after glucocorticosteroids): the enhancement resolved; residual T2 abnormality | Yes | NT | NA | 7 days | PVD Cortex NA | IV DEX | Partial | 1 | Monophasic- survived | 3 years |
| 7 | 43M | No | No | Partial seizures Epilepsy partialis continua Leg weakness, numbness | 2 weeks | Normal Day 9: normal Month 21: Focal parasagittal and paracentral white matter lesion with faint enhancement | No | WBC 4 PRO 31 GLU 61 OCB 0 | Partial seizures resolved with anti-epileptic drugs; no other treatment; no further attacks or progression; follow up MRI suggested glioma at 21 months | 22 months | PVD Cortex NA | None | NA; residual sensory loss after biopsy | 3 | Monophasic- survived | 16.7 years |
| 8 | 40M | No | No | Isolated seizure | NA | Incomplete ring enhancing left frontal mass with mass effect and oedema | No | NT | NA | 7 days | PVD +CMA | Oral glucocortico steroid | Complete | 0 | Monophasic- survived | 4.9 years |
| 9 | 53F | No | URI | Headache Encephalopathy Gerstmann syndrome Hemiparesis Gait ataxia | 2 | Day 2 MRI: Large ill-defined left hemispheric T2-weighted lesion with mild mass effect and shift; no enhancement; involved insula and thalamus; right hemisphere normal | Yes | NT | NA | 6 days | PVD –CMA | IV DEX | Complete | 0 | Monophasic- survived | 15.6 years |
| 10 | 61F | No | PV | Headache Encephalopathy DLC Nausea Meningismus, mild Hemiparesis Required mechanical ventilation | 3 | Large frontotemporal mass with oedema, midline shift and crossing the anterior corpus callosum with faint enhancement | Yes | NT | NA | 3 days | PVD –CMA | IVMP | Partial | 5 | Monophasic- survived | 2.5 years |
| 11 | 45F | No | URI | Encephalopathy Ataxia Neurogenic bladder Pseudobulbar affect | 10 weeks | Multifocal bilateral increased T2 in white matter, confluent; partial enhancementb | Yes | WBC 4 PRO 30 GLU 89 OCB 0 | Subacute stereotypical exacerbation | 10 weeks | PVD –CMA | IVMP | Complete initial response | 10 | Relapsing: recurrence at 6 and 8 months improved with IVMP and oral glucocorticosteroid, then severe recurrence at 9 months progressing to death despite IVMP and PLEX | 10 months |
| 12 | 5M | No | URI | Headache Vomiting Irritability Meningismus Ataxia Neck and leg pain Urinary retention Paraparesis | 4 weeks | CT scan: normal at presentation MRI 5 months later: multifocal bilateral T2 midbrain, pons, cervical cord, largest right PO and corpus callosum with atrophy (no gadolinium given) | Yes | WBC 40 PRO 18 GLU 51 OCB 0 | Initial syndrome spontaneously improved, then worsened with bilateral optic neuritis one month later improved with oral glucocorticosteroids, then relapse again prior to biopsy. | 7 months | PVD CD +CMA | IVMP | Near complete initial | 10 | Relapsing glucocorticosteroid responsive syndrome (at least 7); EDSS 2.5 16 months after onset; eventually unable to wean glucocorticosteroids; patient died secondary to status epilepticus 7 years after onset | 7 years |
| 13 | 68F | Yesb | No | Diplopia Dysarthria Gait ataxia Face numbness | 6 weeks | Patchy and punctuate solitary brain stem mass | No | WBC 2 PRO 49 GLU 59 OCB 0 | Possibly related 1–2 week spell of diplopia 30 years priorb | 7 weeks | PVD CD Cortex NA | IVMP | Partial | 7 | Relapsing stable at last follow up MRI lesion smaller, but leading edge of enhancement without new lesions | 1.5 years |
NT = not tested; NA = not available; PVD = perivenous demyelination; CD = Confluent demyelination; PC = perivenous demyelination with coalescence; CMA = cortical microglial activation; LOC = level of consciousness; DLC = depressed level of consciousness;WBC = white blood cell count; PRO = protein; EDSS = Expanded Disability Status Scale; GLU = glucose; OCB = oligoclonal bands; PLEX = plasma exchange; IV = intravenous; IVMP = intravenous methylprednisolone; CSF = cerebral spinal fluid; IPMSSG = International Paediatric Multiple Sclerosis Study Group ADEM criteria; DEX = dexamethasone.
a Description according to report; images unable to be obtained for review.
b Two episodes of binocular horizontal diplopia lasting 1–2 weeks, 30 years prior to index attack. The patient was not evaluated at the time.