Table 2.
Case | Age | Sex | Comorbidities | Prodrome & duration (days) | Signs and Symptoms | CSF | Treatment | MRI & EEG | Outcome | |
---|---|---|---|---|---|---|---|---|---|---|
Sayao et al. (11) | 39 | F | - | Headache, Nausea Vomiting Fever Imbalance |
7 days | Opsoclonus, mild left facial palsy, bilateral mild intention tremor of the upper extremities, left arm myoclonus | - | Supportive care | Brain MRI: normal | Resolution of OMS Persistence of mild ataxia |
Afzal et al. (32) | 43 | F | - | Dizziness Nausea Fever Myalgia |
10 days | Opsoclonus, myoclonus, gait ataxia | 92 WBC Proteins 110 mg/dl Normal glucose |
IVIG i.v and oral steroids Supportive care |
Brain MRI: normal | Resolution of OMS after ten days Near complete recovery |
Birlutiu et al. (33) | 57 | F | Larynx neoplasm (4 years before) Diabetes mellitus |
Dizziness Fever Vomiting Diarrhea Sleepiness |
2 days | Opsoclonus (persisting during sleep), myoclonus, neck stiffness, ataxia | 49 WBC Proteins 42mg/dl Normal glucose |
IV Dexamethasone Clonazepam Supportive care | Brain MRI: SVD | Died 4 weeks after admission |
Cooper et al. (4) | 48 | F | Endometrial cancer (with previous chemotherapy) |
Confusion Nuchal rigidity |
7 days | Opsoclonus, myoclonus mainly in the lower extremities, later coma | 67 WBC Proteins 131mg/dl Normal glucose |
Plasmapheresis Supportive care | Brain MRI: normal EEG: severe diffuse slowing | Slow resolution of OMS Complete neurologic rehabilitation after 90 days |
Hébert et al. (34) | 63 | F | - | Headache Malaise Nausea Fever Gait ataxia |
5 days | Opsoclonus, Jaw tremor, Mild bilateral upper limb tremor, Multifocal myoclonus |
36 WBC Proteins 48mg/dl Normal glucose |
IVIG Supportive care |
Brain MRI: normal | Rapid resolution of myoclonus Near complete resolution of opsoclonus after 3 days Complete remission at two months |
Khosla et al. (30) | 62 | M | Stage IV non-small cell lung cancer | Fever Lethargy |
10 days | Neck stiffness Encephalopathy Truncal-limb ataxia Myoclonus Opsoclonus | 17 WBC Albumin 30.6 mg/dl Normal glucose |
Supportive care | Brain MRI: normal | Resolution of OMS after 60 days Persistence of ataxia Improved mental status |
Alshekhlee et al. (35) | 53 | M | Hypertension Coronary artery disease Peripheral artery disease |
Malaise Irritability Fever Headache Cervical pain |
few days | Neck stiffness, encephalopathy, myoclonus, opsoclonus, intermittent action tremor | 81 WBC Proteins 110 g/dl Normal glucose |
- | EEG: mild slowing of the posterior dominant rhythm | Severe disability after three months Severe myoclonus and encephalopathy Improved after 8 months, showing only mild action tremor |
Rasmussen et al. (36) (poster) | 52 | M | - | Fever Rash Headache Back-pain |
- | Myoclonus, Opsoclonus Poor vision | ↑ WBC ↑ protein ↑IgGIndex |
Steroids and IgG Rituximab Supportive care | - | Improvement of opsoclonus Persisting myoclonus at discharge |
Shellenback et al. (37) (poster) | 69 | M | Liver transplant (6 years before) Tacrolimus therapy | Fatigue Fever Nausea Diarrhea |
14 days | Myoclonus, Opsoclonus, Coma |
↑ WBC ↑ protein WNV-Ab |
Supportive care | Brain MRI: thalamic T2W hyperintensity EEG: diffuse slowing | Discharged from intensive care unit No additional data |
Tan et al. (38). (poster) | 47 | M | - | Malaise Rash Fever Headache Arthralgia |
10 days | Opsoclonus, Myoclonus, Generalized ataxia |
46 WBC Proteins 105mg/dl |
IV Methylprednisolone 1g/5 days | Brain MRI: normal | Resolution of OMS Persistence of mild ataxia |
47 | F | - | Rash Fever Arthralgia Headache |
7 days | Opsoclonus, Myoclonus, Ataxic gait |
76 WBC Proteins 78mg/dl |
IV Methylprednisolone 1g/ 5 days | Brain MRI: normal | Resolution of OMS after 30 days Complete recovery |
|
Zaltzman et al. (39) | 61 | F | Diabetes Mellitus Obesity Hyperlipidemia Hypertension | Fever Nausea Vomiting Dizziness |
14 days | Opsoclonus, Myoclonus, Tremor of the head, Truncal ataxia |
13 WBC | IV Methylprednisolone 1g/ 5 days | Brain MRI: SVD | Resolution of OMS after 90 days Complete recovery |
55 | M | - | Fever General Weakness Vomiting Dizziness |
14 days | Myoclonus, Opsoclonus and ocular, flutter, Truncal ataxia, Action tremor |
- | - | Brain MRI: SVD | Complete recovery after several months | |
Our patient | 75 | F | Hypertension Diabetes mellitus Autoimmune thyroiditis |
Headache Nausea Vomiting Fever |
5 days | Neck stiffness, Opsoclonus, Myoclonus, Encephalopathy |
17 WBC Albumin 30.6mg/dl Normal glucose |
IV Dexamethasone Supportive care | Brain MRI: SVD EEG: diffuse slowing |
Slow improvement Resolution of myoclonus after 21 days Resolution of opsoclonus after 60 days Complete recovery after 6 months |
EEG, Electroencephalography; MRI, Magnetic Resonance Imaging; SVD, Small Vessel Disease; WBC, White Blood Cells; M, Male; F, Female; WNV-Ab, antibodies against West-Nile Virus; IVIG, intravenous immunoglobulin; OMS, opsoclonus – myoclonus syndrome.