A 68-year-old male presented with acute onset paraesthesias of both upper limbs and lower limbs followed by difficulty in getting up from a squatting position for 1 day. He also had complaints of difficulty in swallowing more for liquids since then. He presented to our hospital walking independently. There was no history of preceding fever, loose stools, or upper respiratory illness. He was a known diabetic for 10 years and hypertensive for 2 years with good drug compliance and history of recurrent stroke (twice once in 2007 and other in March 2017) in the past recovered with no residual deficits. At the time of presentation, he was conscious, well oriented, and communicating well with normal speech. Spino-motor examination showed a flaccid quadriparesis of power 3/5 of all four limbs along with neck and truncal muscle weakness. Upper limbs were more affected than lower limbs with proximal weakness more than distal. Deep tendon reflexes were absent with bilateral plantar flexor. Gag reflex was weak. His SBC (single breath count) was 15. Other cranial nerves and sensory system examinations were normal. No evidence of meningeal signs. With a working diagnosis of Guillain-Barré syndrome, he had been evaluated for the same.
His complete blood picture (haemoglobin—14 g/dL; total leucocyte counts—7100 cu mm; platelets—154,000/μL) was normal. Erythrocyte sedimentation rate was 27 mm after 2 h. Chest X-ray and urine analysis were normal and showed no evidence of infection. His sugars were 97 mg/dL. His renal functions and liver functions were normal. Serum electrolytes (sodium—135 mEq/L; potassium—3.79 mEq/L; ionised calcium—1.201 mmol/L; magnesium—1.8 mEq/L; phosphorus—2.6 mg/dL) were normal. His thyroid functions (serum TSH—3.5 mIU/L; free T4—1.8 ng/dL) were normal. Magnetic resonance imaging (MRI) of the brain with whole spine screening was normal. Serum CPK levels were not significantly elevated (414 U/L). We used Nihon Kohden with model number Neuropack X1 MEB – 2300K, Nihon Kohden Corporation, Japan, for nerve conduction studies (NCS). Nerve conduction studies (NCS) of all four limbs were suggestive of severe demyelinating neuropathic changes with temporal dispersion—considering all the features favouring the diagnosis of Guillain-Barré syndrome (GBS). He was treated with intravenous immunoglobulin (IVIg) given for 5 days according to weight base dose after obtaining informed consent. He had worsening bulbar symptoms on the second day of hospital stay for which he was intubated to secure airway and put on mechanical ventilatory support. Cerebrospinal fluid (CSF) analysis was acellular with mildly elevated proteins. After the complete course of IVIg, he was gradually weaned off from the ventilator by the seventh day and also had improvement of symptoms. He had autonomic symptoms of hypothermia, profuse sweating, and paralytic ileus during the ICU stay, which resolved gradually after treatment with IVIg. He had persistence of hoarseness of voice for which ENT surgeon opinion for vocal assessment done showed mild bilateral restriction of vocal cord mobility with an ulcer over left posterior commissure secondary to intubation. He was able to eat and swallow orally with no signs of any aspiration, and his motor symptoms improved. He was able to walk without support and hence was discharged after 2 weeks of hospital stay.
He has again come to our outpatient clinic after 2 months with complaints of sudden onset of asymmetrical ptosis (left more than right) with bulbar symptoms (hoarseness of voice and nasal regurgitation to liquids) and dysarthria for 3 days since July 7, 2018. He had a history of upper respiratory tract infection for 3 days, 1 week preceding the present symptoms. On examination, he had bilateral asymmetrical ptosis (left more than right) with poor gag reflex and single breath count was 19 with the rest of the neurological examination normal. MRI brain plain was done, and acute stroke was ruled out. Blood investigations were normal. Nerve conduction studies of all four limbs showed evidence of axonal neuropathy in both lower limbs and demyelinating neuropathy in upper limbs with temporal dispersion. Serum CPK levels were normal. With a diagnosis of myasthenia gravis—MGFA type IIB clinically—RNS showed postsynaptic neuromuscular disorder pattern classical for myasthenia gravis. Serum AChR antibodies were strongly positive (15.9 nmol/L). Contrast-enhanced computed tomography (CECT) of the thorax showed no evidence of thymoma. He was started on anticholinesterases, steroids (low dose), and immunosuppressants (azathioprine) following which he had an improvement in symptoms.
Here, our patient had initially Guillain-Barré syndrome who is treated and recovered completely; 2 months later, he has worsening bulbar symptoms with asymmetrical ptosis (left more than right). Additional investigations like RNS (done by RMS Portable Aleron, PEMGM2\122026\ADBX, Recorders and Medicare Systems Private Limited, India) showing postsynaptic disorder and strongly positive AChR antibodies were more favouring the diagnosis of myasthenia gravis.