Among cranial nerves, hypoglossal nerve involvement is extremely rare in Guillian-Barré Syndrome (GBS), with few case reports available in the literature. Here, the authors present a case of GBS with 12th cranial nerve involvement.
A 38-year-old nondiabetic, nonalcoholic male presented with acute onset progressive weakness in all four limbs starting from the lower limbs for the past 8 days. No history suggestive of any sensory or bladder/bowel abnormality was noted. His history including having suffered an episode of diarrhea 3 weeks earlier lasting for 2 days, which had remitted spontaneously. On examination, total areflexic flaccid quadriparesis was noticed (power of proximal muscles 1/5 according to the Modified Medical Research Council grading, in all 4 limbs) and mild left-sided facial nerve weakness. One day after admission, i.e., on the ninth day after the onset of weakness of the limbs, he developed difficulties in talking and in moving the tongue. Examination on the 12th day of illness revealed tongue atrophy and fasciculations and deviation of the tongue to the right; the findings were corroborative of right-sided hypoglossal palsy [Figure 1]. Nerve conduction study (NCS) in all four limbs was performed, which showed features compatible with demyelinating sensory-motor polyradiculoneuropathy. In addition, the left facial NCS revealed suggestions of neuropathy. The cerebrospinal fluid (CSF) study showed albuminocytological dissociation (cell count 3/cmm; all lymphocytes; protein 87 mg/dL). Magnetic resonance imaging (MRI) of the brainstem and cervical spine was normal [Figure 2]. Antiganglioside antibody evaluation showed positive results for immunoglobulin M (IgM) GM2 and GT1b, and immunoglobulin G (IgG) GM2 antibodies. Vasculitis profile, including antinuclear antibody, rheumatoid factor, complement component 3 (C3), and complement component 4 (C4), was within normal limits. A final diagnosis of GBS complicated by right-sided hypoglossal nerve palsy and asymptomatic left-sided facial nerve weakness was made. The patient was treated with intravenous immunoglobulin for 5 days at a dose of 0.5 g/kg daily. Limb weakness improved gradually over 3 weeks, whereas tongue weakness improved marginally. The follow-up at 4 months revealed almost complete recovery of muscle power, while tongue atrophy showed only partial improvement.
GBS is associated with cranial nerve involvement in approximately 45-75% of cases. The most common nerve to be involved is the seventh cranial nerve (facial nerve) followed by nerves to extraocular muscles (third, fourth, and sixth), and the least involvement is shown by the lower cranial nerves.  Among all cranial nerves, hypoglossal nerve involvement is extremely uncommon in GBS with only three cases having been reported till date. ,, Tan et al. reported a case of severe GBS presenting with quadriplegia and complicated by complete paresis of multiple motor cranial nerves including the hypoglossal nerve.  Polo et al. also mentioned a case of an atypical variant of GBS in a 23-year-old male with multiple cranial nerve palsies including of the hypoglossal nerve, where the patient developed diplopia, facial diplegia and a nasal voice, weakness of the neck and tongue muscles, dysphagia, abolition of reflexes of the left arm, and right triceps reflex, but without involvement of the respiratory muscles or other limbs.  Recently, Nanda et al. reported a case of a 13-year-old male who developed bilateral facial and bulbar weakness and became anarthric due to hypoglossal nerve palsy.  The patient required ventilation for bulbar involvement. His antiganglioside antibody panel showed positivity for IgM GM2 and GT1b, and IgG GM1, GM2, GM3, and GT1b antibodies. Those cases had in common a relatively young patient profile and features of other lower cranial nerve involvement (nasal intonation, dysphagia, respiratory difficulty). However, our patient was middle-aged, did not develop complete bulbar palsy, and recovered without need of ventilator support. Moreover, only IgM GM2 and GT1b, and IgG GM2 antibodies were found to be positive.
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Read associated Erratum: Erratum: Hypoglossal nerve palsy in a case of Guillain-Barré syndrome with this article
[Figure 1], [Figure 2]