Table 3.
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.