We report a 40-year-old man who has Marfan's syndrome and was recently diagnosed to have pulmonary tuberculosis when he presented with chronic cough. He was admitted with bilateral lower limb weakness which was ascending in nature. He eventually required ventilation. It was initially thought to be isoniazid-neuropathy. However, stopping the drug did not improve the condition and the patient developed bilateral lower motor neuron 7th cranial nerve palsy. Nerve conduction, MRI and CSF studies were done to confirm a first case report of AMSAN variant progressing to CIDP in a patient with Marfan's syndrome and pulmonary tuberculosis.
We report a case of cardiac arrhythmia occurring in a Guillain-Barré syndrome (GBS) patient after succinylcholine administration during third endotracheal intubation, on day 13 of illness. The probable cause of arrhythmia is succinylcholine-induced hyperkalemia. Of interest, this case demonstrated in the same patient that arrhythmia only occurred during third intubation, when duration of illness is prolonged, and not during previous two intubation episodes, despite succinylcholine was also being used. In GBS, muscle denervation resulted in up-regulation of acetylcholine receptors at neuromuscular junctions, causing the muscle cell membrane to become supersensitive to succinylcholine, leading to severe hyperkalemia and arrhythmia when succinylcholine was administered.
Heterotopic ossification (HO) is the abnormal development of bone within soft tissue and a rare complication in Guillain-Barré syndrome (GBS). Only a few cases had been reported so far. We present the case of a 39-year-old man who had been diagnosed to have GBS about 10 years ago, presenting with severe limitation of both active and passive range of motion in bilateral shoulder, elbow and hip joints and was found to have massive heterotopic ossification. In our patient, it could be a myriad of factors such as prolonged ICU stay with mechanical ventilation and hypoxia, long-standing immobilization and hypomobility with incomplete flaccid paralysis.
Acute Guillain-Barre syndrome (GBS) is characterized by an acute onset of limb weakness and areflexia. There are a few rare variants that have been described and one of them is the pharyngeal-cervical-brachial (PCB) variant (oropharynx, neck, and proximal upper limb muscles). However, in this patient, the only presentation was bulbar involvement with fast recovery within days. This is likely to be the milder form of PCB that has rarely been described before. A 19-year-old Malay lady presented with progressive dysphagia associated with nasal voice for one week duration. There was no limb weakness. Examination showed generalized areflexia. Pharyngeal and palatal muscles were markedly weak. Cerebrospinal fluid (CSF) examination showed raised protein level. Nerve conduction studies revealed generalized demyelinating motor polyneuropathy consistent with GBS. The patient fully recovered within three days and was discharged well.
Bickerstaff's brainstem encephalitis (BBE) is a rare immune-mediated disorder characterized by ophthalmoplegia, ataxia and disturbance of consciousness, which may overlap with Guillain-Barré syndrome (GBS) if there is additional limb weakness. We report a 7-month-old boy presented with ophthalmoplegia followed by a rapidly ascending paralysis of all four limbs and disturbance of consciousness. The initial impression was BBE with overlapping GBS. This was supported by sequential nerve conduction study (NCS) findings compatible with an acute inflammatory demyelinating polyneuropathy (AIDP). He received intravenous pulse methylprednisolone, intravenous immunoglobulin and plasmapharesis with complete clinical recovery after 6 weeks of illness and improved NCS findings from week 16. This is the first case of paediatric BBE with overlapping GBS with an AIDP subtype of GBS. It expands the clinical spectrum of this condition in children. Our case highlights the importance of sequential NCS in paediatric BBE with overlapping GBS for accurate electrophysiological diagnosis and prognosis particularly if the first NCS findings are not informative.