There are few reports of neuropathic tremor after Guillain Barré syndrome (GBS). 1 We describe a patient with tremor after an axonal motor form of GBS who responded to pregabalin treatment.
A 13‐year‐old boy presented with acute ascending tetraparesis. Muscle strength was grade 4 on the MRC Scale in the upper and lower limbs. Sensory examination was normal and deep tendon reflexes were hypoactive. Nerve conduction studies (NCS) and electromyography (EMG) were consistent with axonal motor neuropathy. He was treated with intravenous immunoglobulin 400 mg/kg/day for 5 days. Recovery was marked by complete motor improvement within 30 days, but also by the emergence of an appendicular, postural and kinetic 8–10 Hz low‐amplitude tremor in the upper limbs and tongue (Video 1). Deep tendon reflexes improved and were normoactive at the time of the tremor onset. No other cause for the tremor was identified. Brain MRI was unremarkable. He was diagnosed with neuropathic tremor associated with Guillain‐Barré syndrome and treated with propranolol up to 120 mg/day with no significant response. Pregabalin (PGB) was started and titrated up to 300 mg/day with improvement in tremor magnitude, and such reduction was maintained at the last follow‐up. The response to pregabalin was assessed using the Fahn–Tolosa–Marin tremor rating scale before, after the introduction of PGB 300 mg, and after 2 years of follow‐up (Table 1).
Video 1.
Postural and kinetic 8–10 Hz tremor involving the hands and tongue of a patient recovering from Guillain‐Barré syndrome.
TABLE 1.
Improvement of tremor after treatment with pregabalin
| TRS | Upper limbs | Tongue | |
|---|---|---|---|
| Part A | Part B | Part A | |
| TRS at baseline | A2 | B2 | A3 |
| TRS at PGB 300 mg a | A1 | B0 | A0 |
| TRS at 2‐year follow‐up | A1 | B0 | A0 |
Note: TRS, Fahn–Tolosa–Marin tremor rating scale; A, assessment of tremor severity at rest, with posture holding and with action and maneuvers; B, tests action tremor while writing, drawing, and pouring liquids; A0, no tremor; B0, normal; A1, slight, barely perceivable, may be intermittent; B1, mildly abnormal, slightly untidy, tremulous; A2, moderate, amplitude <2 cm, may be intermittent; B2, moderately abnormal, legible but with considerable tremor; A3, marked, amplitude 2–4 cm.
Abbreviation: PGB, pregabalin.
After 2 weeks of pregabalin introduction.
Neuropathic tremor (NT) is defined as tremor appearing in association with a peripheral neuropathy when no other neurological diseases that can cause tremor are present. 2 NT is seen more frequently with demyelinating neuropathies, such as chronic inflammatory demyelinating neuropathy (CIDP), especially those with anti‐nodal/paranodal IgG4 antibodies. 1 The characteristics of NT are similar to essential tremor (predominantly postural kinetic tremor affecting both hands) and frequency has varied in reports between 3.3 and 10 Hz. 3 No relationship has been established between tremor severity and motor or sensory loss. 3 There are few reports of NT after GBS, only one involving the tongue. 4
Essential tremor (ET) and NT involve neuronal systems that have oscillatory activity. ET is believed to result from dysfunction in the feedforward control of movement involving olivocerebellar and cerebellocortical circuits. 2 Conversely, NT probably results from peripheral slowing in afferent signals leading to distortion in feedback mechanisms. 2 Drugs used to treat ET, like propranolol and primidone, were mostly ineffective for neuropathic tremor. 3 These drugs probably act in cerebellar feedforward circuits not involved in NT. Propranolol was unsuccessfully tried in most of cases of tremor after GBS. 4 Pregabalin is a GABA analog that decreases the release of other neurotransmitters, including noradrenaline, glutamate and substance P. 5 Through this mechanism, pregabalin probably modulates peripheral sensory afferents and the feedback control of movements. 5 Response to pregabalin has been reported in CIDP. 3 We found only one report of NT after axonal GBS on pregabalin (titrated to 450 mg daily) with improvement. 5
Based on the scarce reports, propranolol seems to have poor efficacy in NT after GBS. Pregabalin may be a safe and effective treatment option for these cases.
Author Roles
(1) Research Project: A. Conception, B. Organization, C. Execution; (2) Statistical Analysis: A. Design, B. Execution, C. Review and Critique; (3) Manuscript Preparation: A. Writing of the first draft, B. Review and Critique.
P.R.N.: 1A, 1 2, 3
T.M.F.V.: 1, 2, 3
A.R.B.P.: 2C, 3
P.L.G.S.B.L.: 2C, 3
P.B.N.: 2C, 3
M.B.K.: 1, 2, 3
Disclosures
Ethical Compliance Statement: We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. The authors confirm that the patient provided verbal and written consent for this work but because this article is a case report no IRB approval was necessary.
Funding Sources and Conflicts of Interest: No specific funding was received for this work. The authors declare that there are no conflicts of interest relevant to this work.
Financial Disclosures for Previous 12 Months: The authors declare that there are no additional disclosures to report.
Acknowledgments
We would like to thank the patient and his family for participating in this research.
Paulo Ribeiro Nóbrega and Thais de Maria F. Vasconcelos have contributed equally to this research.
References
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