Background
Methods
Subjects
Features required for diagnosis | |
Progressive weakness in both arms and legs (might start with weakness only in the legs) | |
Areflexia (or decreased tendon reflexes) | |
Features that strongly support diagnosis | |
Progression of symptoms over days to 4 weeks | |
Relative symmetry of symptoms | |
Mild sensory symptoms or signs | |
Cranial nerve involvement, especially bilateral weakness of facial muscles | |
Autonomic dysfunction Pain (often present) | |
High concentration of protein in CSF | |
Typical electrodiagnostic features | |
AMAN | |
None of the features of AIDP except one demyelinating feature allowed in one nerve if dCMAP <10% LLN | |
Sensory action potential amplitudes normal | |
AMSAN | |
None of the features of AIDP except one demyelinating feature allowed in one nerve if dCMAP < 10% LLN | |
Sensory action potential amplitudes < LLN |
Evaluation of functional impairment
Electrophysiological study
Anti-ganglioside antibody assay
Results
Characteristics of the patients
Characteristic | Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | Case 6 |
---|---|---|---|---|---|---|
Age (y)/Sex | 29/F | 48/F | 29/F | 57/M | 53/F | 39/M |
Antecedent events | Abortion | mild Traumatic brain injury | Cesarean section | Chest trauma | Endoscopic endonasal resection of Rathke cyst | Femoral fracture |
Time between trauma and symptom onset (days) | 14 | 10 | 8 | 10 | 12 | 12 |
Time between treatment initiation and symptom onset (days) | 6 | 5 | 6 | 11 | 9 | 5 |
Time to nadir (days) | 9 | 7 | 10 | 5 | 12 | 7 |
Time to discharge (days) | 33 | 43 | 21 | 56 | 38 | 22 |
Symptoms at nadir | ||||||
Motor function | Weakness on both limbs (G2/5) | Weakness on both limbs (G2/5) | Weakness on both limbs (G3/5) | Weakness on both limbs (G1/5) | Weakness on both limbs (G2/5) | Weakness on both limbs (G3/5) |
Deep tendon reflexes | Absent (G —) | Absent (G —) | Decreased (G1+) | Absent (G —) | Absent (G —) | Decreased (G1+) |
Muscular atrophy at nadir | + | + | + | + | + | + |
Cranial nerve function | III, IV, VI, VII | II, III, IV, VI | III, IV, VI | V, IX, X | − | III, IV |
Respiratory muscle involvement | − | + | − | + | − | − |
Objective sensory function | Normal | Normal | Abnormal | Normal | Normal | Abormal |
Serum anti-ganglioside antibody | GQ1b | GM1 | GM1,GD1b | − | Missing | GM1,GD1b |
Protein (g/L)/AD in CSF | 0.98/yes | 0.64/yes | 1.10/yes | 0.92/yes | 0.54/yes | 0.72/yes |
Treatment | IVIG | IVIG; MV | IVIG | IVIG; HC; MV | IVIG | IVIG |
Electrophysiological features
Case 1 | Case 2 | Case 3 | Case 4 | Case 5 | Case 6 | ||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
L | R | L | R | L | R | L | R | L | R | L | R | ||
CMAP(mV) | Median nerve | 2.0 | 3.5 | 0.4 | 2.6 | 2.2 | 3.2 | 2.4 | 1.2 | -- | -- | 2.5 | 3.4 |
Ulnar nerve | 1.1 | 1.8 | 1.9 | 3.2 | 2.0 | 2.6 | 1.9 | 1.2 | -- | -- | 1.6 | 2.8 | |
Tibial nerve | 1.5 | 2.1 | 0.8 | 0.6 | 1.5 | 3.2 | 1.0 | 1.0 | -- | -- | -- | -- | |
Peroneal nerve | 1.2 | 1.1 | 1.2 | 1.0 | 2.1 | 2.0 | 0.6 | 0.4 | -- | -- | -- | -- | |
MCV(m/s) | Median nerve | 49 | 45 | 65 | 60 | 62 | 58 | 55 | 57 | -- | -- | 58 | 50 |
Ulnar nerve | 50 | 46 | 60 | 56 | 61 | 64 | 52 | 56 | -- | -- | 47 | 49 | |
Tibial nerve | 44 | 43 | 44 | 43 | 42 | 45 | 50 | 47 | -- | -- | -- | -- | |
Peroneal nerve | 44 | 44 | 40 | 42 | 41 | 41 | 46 | 45 | -- | -- | -- | -- | |
DL(ms) | Median nerve | 3.7 | 3.8 | 3.8 | 3.5 | 3.9 | 3.3 | 3.6 | 3.8 | -- | -- | 2.7 | 3.2 |
Ulnar nerve | 3.2 | 3.1 | 3.8 | 3.6 | 2.8 | 2.9 | 3.0 | 2.9 | -- | -- | 3.2 | 2.9 | |
Tibial nerve | 6.2 | 5.3 | 5.1 | 5.1 | 4.3 | 4.0 | 4.3 | 4.5 | -- | -- | -- | -- | |
Peroneal nerve | 4.6 | 4.4 | 4.9 | 5.0 | 4.1 | 4.3 | 4.6 | 4.3 | -- | -- | -- | -- | |
SNAP(uV) | Median nerve | 6.3 | 8.3 | 15.3 | 13.5 | 15.5 | 13.6 | 12.6 | 11.7 | 11.0 | 12.5 | 8.7 | 10.3 |
Ulnar nerve | 4.2 | 6.3 | 22.6 | 20.5 | 14.3 | 11.5 | 10.2 | 11.0 | 9.2 | 8.5 | 7.3 | 8.2 | |
Tibial nerve | 8.5 | 8.2 | 22.1 | 19.3 | 12.4 | 10.6 | 15.3 | 11.5 | 6.3 | 7.4 | 8.5 | 8.7 | |
Peroneal nerve | 12.4 | 15.3 | 23.6 | 20.2 | 15.8 | 17.3 | 21.6 | 24.3 | 19.6 | 15.8 | 14.7 | 17.2 | |
SCV(m/s) | Median nerve | 59 | 58 | 63 | 60 | 57 | 53 | 51 | 50 | 59 | 56 | 52 | 56 |
Ulnar nerve | 57 | 55 | 64 | 59 | 62 | 58 | 55 | 51 | 52 | 54 | 55 | 59 | |
Tibial nerve | 59 | 59 | 50 | 53 | 56 | 53 | 50 | 49 | 55 | 53 | 50 | 56 | |
Peroneal nerve | 52 | 57 | 53 | 55 | 57 | 52 | 50 | 51 | 54 | 51 | 52 | 57 | |
F-wave(%) | Median nerve | 25.0 | 35.0 | 0.0 | 0.0 | 35.0 | 30.0 | 35.0 | 15.0 | -- | -- | 20.0 | 15.0 |
Ulnar nerve | 0.0 | 20.0 | 0.0 | 0.0 | 60.0 | 45.0 | 15.0 | 0.0 | -- | -- | 0.0 | 0.0 | |
Tibial nerve | 0.0 | 0.0 | 0.0 | 0.0 | 20.0 | 30.0 | 30.0 | 0.0 | -- | -- | -- | -- |
Anti-ganglioside antibodies
Treatment and outcomes
Discussion
Author/Year | Number of case | Sex/number | Median age, years(range) | Antecedent events | Time from trauma to symptom onset (days) | EMG | Nerve biopsy |
---|---|---|---|---|---|---|---|
Rattananan et al. (2014) [1] | 5 | F/3 | 61 (35–68) | Surgery | within 30 days | Neuropathy with active denervation; | Perivascular inflammatory collections;increased axonal degeneration. |
Staff et al. (2010) [2] | 21 | F/11 | 65 (24–83) | Surgery | within 30 days | Neuropathy with active denervation; | Increased epineurial perivascular inflammation;17 patients had increased axonal degeneration. |
Huang et al. (2015) [3] | 4 | M/4 | 57 (50–69) | Spine Surgery: | within 1 week | Neuropathy and 2 cases with active denervation | not done |
Scozzafava et al. (2008) [4] | 1 | M/1 | 28 (28) | Spinal cord injury | within 1 day | Severe axonal polyneuropathy | not done |
Tan et al. (2010) [5] | 1 | M/1 | 44 (44) | Head injury | 1 week | Neuropathy with active denervation; | Presence of lymphocytes and severe axonal degeneration. |
Al-Hashel et al. (2013) [6] | 2 | F/1 | 39 (31–47) | Traumatic bone injury | within 1 week | 1 with features of mixed axonal and demyelinating neuropathy | not done |
Rivas et al. (2008) [7] | 1 | M/1 | 55 (55) | Head injury | 1 week | An inexcitability of all nerves with active denervation; | A severe loss of myelinated axons without significant demyelination. |