Table 3.
Case | Clinical phenotype | Neurological symptoms | Presence of rash | Time of onset after Rash in days | Level rash | VZV DNA PCR | Presence/absence of VZV-IgG/IgM elevation | Imaging findings | Antibody status | Follow-up | Reference# |
---|---|---|---|---|---|---|---|---|---|---|---|
Cases with MOG IgG | |||||||||||
Male, 69 a | LETM | Paraparesis | + | 10 | Left L2-3 dermatome | + | nk | Lesion from the bottom of the medulla oblongata to the upper (T2) thoracic region | MOG-IgG +, cell-based-assay | (15) | |
Female, 34 a | Myelitis | Paraparesis, loss of pain and temperature sensation below her groin, absent vibration sense in both lower limbs | primary VZV infection | 21 | na | nd | nd | normal | MOG-IgG + | Clinical remission | (25) |
MOG IgG remained positive | |||||||||||
Female, 42 a | LETM | nk | + | 1 | C dermatome | nk | Increased VZV IgM | Myelitis: C2-4,T 1, Medulla oblongata | MOG-IgG + | Clinical remission (EDSS 6 to 1), relapse, NMOSD criteria fulfilled | (20) |
Male, 30 a | LETM | Sensomotor paralytic syndrome (sensory level below T6), subsequent gait ataxia, neurogenic bladder disturbance | + | 6 | Right T6 dermatome | – | CSF VZV ASI increased (9.4) | Lesion: T1 to the conus medullaris with only a very faint leptomeningeal contrast enhancement | MOG-IgG + (1:1280), cell-based assay | Clinical remission | Present case |
MOG IgG turned negative | |||||||||||
Cases with AQP4 IgG | |||||||||||
Female, 63 a | LETM | Paresis (3–4/5) and mild hypoesthesia of the left leg, sensory impairment for temperature and pain of the right leg and the trunk below level T10, urine incontinence | + | 14 | Along the lumbar spine | – | CSF VZV ASI normal | Lesion from C7 to Th9 with marked oedema and moderate gadolinium enhancement | AQP4-IgG +, tissue-based indirect immunofluorescence assays | Partial clinical remission (after plasmapheresis) | (7) |
AQP-4 IgG turned negative | |||||||||||
Female, 51 a | LETM, | Decreased power (3/5), hyperreflexia along with sensory loss in the right upper and lower extremity, hyperesthesia in the entire left lower extremity | + | 49 | Right C5 dermatome | – | nk | Enhancing intramedullary lesion C2 -4, centrally into the right of the midline with signal changes at the T1 level without enhancement or expansive appearance | AQP4-IgG first attack nd, relapse + (>1:160) | Two relapses, diagnosis NMOSD, persistent AQP-4 IgG, clinical remission | (8) |
Female, 59 a | LETM | nk | + | 15 | C dermatome | nk | Increased VZV IgM | Myelitis: C1-6 | AQP4-IgG + | Clinical remission (EDSS 2 to 1), no relapse, NMOSD criteria fulfilled | (20) |
Female, 29 a | LETM | Acute quadriplegia | + | 7 | Left T4–6 dermatomes | – | Increased VZV IgM | nk | AQP4-IgG first attack nd, relapse + (1:80), tissue-based indirect immunofluorescence assays | Partial clinical remission, relapse - LETM, NMOSD criteria fulfilled | (9) |
Female, 77 a | LETM | Paraparesis, sensory level by L4, urine retention | + | 2 | Left L4–S1 dermatomes | + | nk | Lesion extending from C2–C3 to T12 with no gadolinium enhancement | AQP4-IgG first attack nd, relapse + indirect immunofluorescence serum assay (1:10) | Severe sequelae, relapse, NMOSD criteria fulfilled | (10) |
Female, 48 a | LETM | Right arm abduction paresis, brisk reflexes in the lower limbs, diminished reflexes in the upper limbs, extensor plantar response bilaterally | + | 14 | Right C6 dermatome | – | nk | Cervical LETM | AQP4-IgG positive, cell-based assay | Fully recovered, except for mild sensory symptoms, NMOSD criteria fulfilled | (11) |
Female, 53 a | LETM | Hyperhidrosis of left side of her face, neck, arm and upper chest, muscle weakness of her left leg, sensory impairment for light touch and temperature in her chest and legs | + | 7 | T5-6 dermatome | nk | CSF VZV IgG index increased (7.9) | Lesion extending from T1-7 | AQP4-IgG + | Relapse | (12) |
Female, 55 a | LETM | Dysesthesia of the right side of the face, neck, bilateral upper extremities, and T4-T10 levels, urine incontinence | + | 14 | Left C3-T4 dermatomes | – | CSF VZV ASI increased (4.53) | Lesion extending from the lower part of the medulla oblongata to C5, with marked edema and moderate gadolinium enhancement and abnormal gadolinium enhancement of the left spinal posterior root | AQP4-IgG +, cell-based assay | Mild response to treatment, relapse, NMOSD criteria fulfilled | (6) |
Female, 17 a | Area postrema syndrome and LETM | Right eye mydriasis, piloerection, poikilothermia, mild hypoesthesia, and pain in the right arm and trunk in the T2-T3 dermatomes/intractable vomiting | + | 21 | Right T2 dermatome | – | IgM VZV ASI increased (7.0) | Lesions involved the area postrema, right ventrothalamic area, periaqueductal gray, optic tracts, and cervical and thoracic regions, longitudinally extended from C1-5 and from C6-T6 and axially involving two-thirds of the spinal cord | AQP4-IgG + | NMOSD criteria fulfilled, resolution | (13) |
A, age; AQP4, aquaporin-4; ASI, antibody specific index; C, cervica; CSF, cerebrospinal fluid; LETM, longitudinally extensive transverse myelitis; MOG, myelin oligodendrocyte glycoprotein; L, lumbar; MRI, magnetic resonance imaging; not applicable, na; nd, not done; NMOSD, neuromyelitis optica spectrum disease; nk, not known; T, thoracic; VZV, varicella zoster virus; +, positive; -, negative.