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|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 2 | Page : 331-334
Isolated acute bulbar palsy with anti-GM2 antibodies: A rare occurrence
Anish Mehta, Rohan Mahale, KS Abhinandan, Mirza Masoom Abbas, Mahendra Javali, Srinivasa Rangasetty
Department of Neurology, MS Ramaiah Medical College and Hospital, Bengaluru - 560 054, Karnataka, India
|Date of Web Publication||3-Mar-2016|
Department of Neurology, MS Ramaiah Medical College and Hospital, Bengaluru - 560 054, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Mehta A, Mahale R, Abhinandan K S, Abbas MM, Javali M, Rangasetty S. Isolated acute bulbar palsy with anti-GM2 antibodies: A rare occurrence. Neurol India 2016;64:331-4
|How to cite this URL:|
Mehta A, Mahale R, Abhinandan K S, Abbas MM, Javali M, Rangasetty S. Isolated acute bulbar palsy with anti-GM2 antibodies: A rare occurrence. Neurol India [serial online] 2016 [cited 2021 Mar 3];64:331-4. Available from: https://www.neurologyindia.com/text.asp?2016/64/2/331/177620
Isolated bulbar palsy is a rare regional variant of Guillain-Barre Syndrome (GBS). It may occur in isolation or with mild proximal upper limb weakness, when it has been referred to as the pharyngeal-cervical-brachial (PCB) variant. The latter occurs in 2% of patients with GBS. It usually affects the oropharyngeal muscles. Anti-ganglioside antibody immunoglobulin G (IgG) [anti-GQ1b, GT1a, GM3, and GT1b] has been associated with isolated bulbar palsy. The prevalence of anti-GM2 antibodies in patients with GBS comprises less than 10% of all antibodies detected and has been shown to be strongly associated with preceding cytomegalovirus infection., However, the clinico-serological association of immunoglobulin M (IgM) anti-GM2 antibodies with isolated bulbar palsy has not been reported previously.
A 48-year-old male presented with acute onset dysphagia to both solids and liquids with nasal quality of voice for the past 4 days. There was no history of facial or limb weakness, sensory disturbances, unsteadiness, or any antecedent infection. There were no comorbidities. On examination, his vital parameters were stable. He was conscious and oriented. Speech had a nasal quality. Extraocular movements were full. Palatal movements were sluggish on both sides without the presence of a gag reflex. The tongue movements were normal. His muscle power was grade 5/5 in all 4 limbs, with no neck flexor weakness. The deep tendon reflexes were absent and the plantar reflex on both sides were flexor. His sensory system examination was normal. The complete hemogram, liver, renal and thyroid function tests, and serum electrolytes were normal. The plain images of magnetic resonance imaging (MRI) of the brain was normal. Cerebrospinal fluid examination (CSF) showed an albumin-cytological dissociation with a protein of 153mg/dl and the cell count of 2/mm 3. CSF oligoclonal band study was negative. Nerve conduction studies (NCSs) showed prolonged distal motor latencies and the presence of F waves of motor nerves (median and ulnar in the upper limbs, and common peroneal and posterior tibial in the lower limbs) with reduced compound motor action potentials and nerve conduction velocities. The amplitude of sensory nerve action potential (SNAP) was reduced in bilateral upper limbs along with an absent sural SNAP suggestive of demyelinating sensorimotor polyneuropathy [Figure 1],[Figure 2],[Figure 3],[Figure 4]. Acetyl cholinesterase receptor antibodies and anti-muscle specific kinase antibodies were negative, ruling out the possibility of myasthenia gravis. Serological testing for anti-ganglioside immunoglobulin (Ig) IgG and IgM antibodies against GQ1b, GT1b, GD1b, GD1a, GM3, GM2, and GM1 was performed (Euroimmun ®) and was found to be positive for IgM anti-GM2 antibodies. The serological testing for cytomegalovirus using enzyme-linked immunosorbent assay for the IgM and IgG classes was negative. A diagnosis of isolated acute bulbar palsy (the regional variant of GBS) was made based on clinical, serological, CSF, and electrophysiological findings. A nasogastric tube was inserted. Intravenous immunoglobulin was planned to be administered; however, he did not show any worsening of symptoms, hence the procedure was deferred. He started to show an improvement in his clinical status, and the nasogastric tube was removed after 5 days following which he was able to swallow both solids and liquids. He was discharged after a week of hospital stay. At follow up visit after 2 months, he had shown a complete recovery.
|Figure 1: Motor nerve conduction study of bilateral median and ulnar nerves|
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|Figure 2: Motor nerve conduction study of bilateral common peroneal and posterior tibial nerves|
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|Figure 3: Sensory nerve conduction study of bilateral median and ulnar nerves|
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|Figure 4: Sensory nerve conduction study of sural and superficial peroneal nerves|
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The PCB variant of GBS refers to the occurrence of weakness restricted in the oropharyngeal, neck, and proximal upper limb muscles without weakness or areflexia of the lower extremities. Acute oropharyngeal palsy is another regional variant of GBS, which may occur in isolation or with mild proximal upper limb weakness. There are few anecdotal reports on isolated acute oropharyngeal palsy without cervical or brachial involvement. O'Leary et al, described three cases of acute oropharyngeal palsy as a regional variant of GBS. Onodera et al., reported the case of a patient with acute bulbar palsy as an isolated neurological symptom, whose serum had an elevated level of anti-GT1a IgG antibody. Hamidon (2005) reported a case of PCB variant of GBS presenting with isolated bulbar palsy; however, serology testing for anti-ganglioside antibody was not done. Rojas-Garcia et al., reported acute bulbar palsy in patients with anti-ganglioside antibodies against NeuNAc (alpha 2-3) Gal. Kasemsap et al., reported isolated bulbar palsy with anti-GM3 and -GT1b antibodies. Our patient also presented with isolated acute bulbar palsy with areflexia. However, there was no ophthalmoplegia or limb weakness. The NCS was suggestive of demyelinating sensorimotor polyneuropathy. His CSF showed albumin-cytological dissociation and the serological testing was positive for IgM anti-GM2 antibodies. IgG anti-ganglioside antibodies are commonly associated with the GBS subtypes and its variants and include the GM1, GD1a, GT1a, GQ1b, GQ1b, and GT1a subtypes. However, the role of GM2 antibodies in GBS variants remains unknown. A possibility of acute polyneuritis cranialis was considered. However, there are no reports of isolated paralysis of bulbar muscles in polyneuritis cranialis. Usually, acute polyneuritis cranialis is characterized by a rapid onset of symmetrical cranial nerve dysfunction without the involvement of limb muscles.
To the best of our knowledge, there are only a few reports on isolated bulbar palsy with positive anti-ganglioside antibodies in the literature. The present report adds further insights to the understanding of pathogenesis of this rare condition and the utility of comprehensive anti-ganglioside antibody testing in the GBS variants.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]
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