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. 2021 Aug 19;2021:9800488. doi: 10.1155/2021/9800488

Table 3.

Electrophysiology, CSF, imaging, treatment, and clinical outcomes.

Case NCS finding CSF Imaging Treatment Outcome
Protein
(15-45 mg/dl)
Cell (<5/μl)
1 4 weeks after ICIs: not suggestive of neuropathy 78 OL (+) 17 IVIG over 5 days for the first time Significant improvement in muscle weakness. 18 days after discharge, the disease developed an ascending paralysis of the extremities.
44 days later: acute and chronic demyelinating polyneuropathy 175 OL (-) 3 PLEX for 7 days and solumedrol for 5 days instead of IVIG for reoccurrence Melanoma progression occurred about 1 year after the last dose of pembrolizumab. Rechallenging with ipilimumab. Only ongoing bilateral leg tingling and diarrhea
2 Acute motor and sensory axonal neuropathy 58.33 8 MRI of the brain and spinal cord was normal. Oral treatment with dexamethasone and then switched to IVIG along with prednisone Symptoms improved during 1 week of treatment. After 1 month, walking and standing was normal.
No recurrence of tumor
3 AIDP 405 4 Spinal MRI: abnormal thickening and enhanced posterior nerve roots (L4-L5, L5-S1), no features of metastatic disease Dexamethasone for 6 days and IVIG for 5 days Hydrocephalus, ventricular enlargement and bed-bound
Died after withdraw from life support
4 Dysautonomia secondary to AIDP 91 0 IVIG+prednisone+PLEX
Life-sustaining therapies
Respiratory distress, tachycardia, and hypotension
Died after withdrew life-sustain
5 AIDP 124 41 Brain as well as the lumbar and thoracic spine MRI: no clear cord or cauda equina involvement despite spine metastases IVIG for 5 days Rehabilitation
No other potential agents were given and alive 6 months after the use of last immunotherapies.
6 AIDP 105 8 Spine MRI: severe stenosis of lumbar spinal canal IVIG for 5 days and methylprednisolone during 7 days A rapid clinical improvement was observed within the first 3 days. Methylprednisone was tapered progressively over 10 weeks. Completely recovered. 6 months later, cancer remains stable.
7 MFS 175 5 Brain and cervical spine MRI: normal IVIG and intravenous methylprednisone followed by oral prednisolone Severe global limb weakness and respiratory decompensation developed after 3 days of IVIG.
Respiratory function and motor weakness improved after 9 days of corticosteroid treatment.
Rehabilitation after 3 months of treatment
8 AIDP 140 2 Brain and cord MRI: nonspecific cerebral white-matter lesions IVIG for 5 days along with prednisone for 1 month Improvement.
Disease progressed with increased number and size of lung metastases. Died from sepsis
9 AIDP 88 2 Brain and spinal MRI: no evidence of metastases or ischaemic and/or hemorrhagic lesions Methylprednisolone
IVIG for 5 days
Prednisone
Without clinical benefit
Leg weakness, numbness in patient's fingers, and paresthesia dramatically improved.
A further improvement was obtained after 2 weeks of prednisolone.
10 Acute sensorimotor polyradiculoneuropathy with mixed axonal/demyelinating <45 <5 Spinal CT: normal IVIG for 5 days and prednisone 30 mg daily at the same time 3 weeks later, improved rapidly and transition to pembrolizumab with no evidence of GBS
11 AIDP 230 0 Spinal MRI: normal IVIG Good recovery
12 AMAN 37 mg/dl but elevated IgG levels 2 Prednisone
IVIG
Symptoms continued to worsen.
Minimally improved but prevented further progression
13 AIDP No lumbar puncture was performed. Spinal MRI: degenerative changes with no evidence of cord compression Methylprednisone and
IVIG
Prednisone
19 days after admission, weakness and numbness mostly resolved.
1 month later, neurological recovery except for mild residual paresthesias of the feet
14 Multiple cranial neuropathy and AIDP 350 7 Lumbar spine MRI: a reduction of gadolinium enhancement of nerve roots and cauda equina IVIG
Steroid pulse therapy
Multiple cranial neuropathies were moderately improved after 4 weeks of treatment.
Muscle weakness remarkably improved after the 3 courses of therapy.
15 AIDP 680 <5 Methylprednisolone along with IVIG
PLEX
Strength diminished to 2/5 in the bilateral UEs and LEs.
Respiratory status worsened.
Respiratory status improved, and motor function gradually recovered.
16 AIDP 560 <5 Brain CT: a hemorrhage within one of his metastatic lesions and associated vasogenic edema Methylprednisolone along with IVIG for 5 days
PLEX
Acute hypoxic respiratory failure, requiring mechanical ventilation. After five days of treatment without any clinical improvement.
A hemorrhage within one of metastatic lesions and associated vasogenic edema. Died after the withdrawn of care
17 AIDP 175 Lymphocytic pleocytosis Brain and spinal MRI: abnormal enhancement involving the bilateral 5th, 7th and 8th cranial nerves, cauda equina nerve roots as well as the conus surface and peripheral nerves at the thoracolumbar junction Methylprednisolone Motor symptoms in hands and lower extremities improved rapidly after 2 days and gradually recovered over a 12-week period.
Weakness resolved completely, residual minimal paresthesias
18 AIDP 339 4 Prednisone (60 mg/day)
IVIG (0.4 g/kg) for 5 days
Symptoms worsened
Gradually improved
3 months later, he was able to walk with a cane.
19 Asymmetric, subacute to early chronic and ongoing lumbar polyradiculoneuropathy with axonal involvement and demyelinating >300 1 Spinal MRI: diffuse enhancement surrounding the entire conus and all of the nerve roots Dexamethasone Partial response systemically and neurologic improvement
6.5 months later, melanoma progressed.
20 MFS and demyelinating sensorimotor polyneuropathy 125 0 IVIG 2 g/kg for 5 days and methylprednisolone followed by a weaning dose of oral prednisolone
PLEX
Modest clinical improvement
Significant functional improvement and complete recovered at last. But melanoma had progressed.
21 AIDP 86 0 IVIG over 5 days
Prednisone 90 mg/day
Dysphagia requiring nasogastric tube for feeding
After 6 months, neurological condition improved significantly and the dysphagia completely was resolved. Numbness was still present but improved.
22 AIDP 73 <5 IVIG for 5 days
Methylprednisolone
Did not lead to any clinical improvement
Clinical recovery started 48 h later and was nearly complete after 6 weeks.
23 Acute sensorimotor polyradiculopathy with mixed axonal/demyelinating features 39 <5 Thoracic spine and lumbar spine CT: degenerative changes IVIG Marked improvement on treatment day 5
24 GBS presenting as dysautonomia, a length-dependent, sensorimotor polyneuropathy with axonal and demyelinating properties No lumbar puncture was performed. IVIG Dysautonomia and weakness persisted, and cardiovascular and respiratory status improved by 2 months.
Persistent urinary retention, oropharyngeal dysphagia, and generalized weakness
25 AMSAN with autonomic symptoms 115 15 Spinal MRI: normal IVIG (0.4 g/kg/d) for 5 days
Methylprednisolone (1 g/d for 5 days, then 500 mg/d for 3 days) followed by tapering oral prednisone (1 mg/kg/d)
PLEX (5 changes over 2 weeks, followed by weekly exchanges)
Symptoms stabilized with mild improvement, yet one month later, it developed worsening weakness and ongoing painful paresthesia. Persistent nausea coupled with postural hypotensin and constipation
6 weeks later (12 weeks after initial treatment), the patient had only mild weakness.
9 months later, melanoma progressed.
26 GBS 85 0 Spine MRI: normal IVIG+PLEX Within 2 hours, respiratory muscle paralyzed, and ventilator support was applied.
She was extubated after 11 days and expired within a few hours.
27 Acute, generalized, symmetrical, and sensorimotor neuropathy, impossible to distinguish axonal or demyelinating disorder because of severe limb edema 160 0 Methylprednisolone
PLEX
Clinical status did not improve.
Died of multivisceral failure within a few days
28 AMSAN 89 0 Cervical spine MRI: normal IVIG The muscle strength of all limbs slightly increased. But 3 days later, died from respiratory insufficiency
29 AIDP 167 <2 Brain and spinal MRI: normal Methylprednisolone Recovery
30 AIDP 56 Normal Prednisolone
IVIG
The neurologic symptoms reached the peak within 3 weeks and decreased over the next 2 months.
31 MFS 81 OL (+) Normal Central nervous system imaging showed no cerebral or vertebral pathology. Prednisolone
IVIG
PLEX
A slight improvement with these treatments
2 months later, died from pneumonia
32 GBS 107.5 61 Prednisolone
IVIG
The pain was greatly diminished.
8 months later, the motor and sensor function of extremities were still slowly recovering.
33 GBS 204.6 OL (+) Normal Prednisolone
IVIG
Methylprednisolone
The mild persistent weakness of his feet extensors and mild sensory loss and ataxia

AIDP: acute inflammatory demyelinating polyradiculoneuropathy; MFS: Miller Fisher syndrome; IVIG: intravenous immunoglobulin; PLEX: plasma exchange; AMAN: acute motor axonal neuropathy; AMSAN: acute motor and sensory axonal neuropathy; OL: oligoclonal bands.