Isolated unilateral palatal and vocal cord palsy as an initial presentation of chronic inflammatory demyelinating polyradiculoneuropathy

Abstract

Abstract Introduction Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired autoimmune disease of the peripheral nervous system. Its clinical presentation and excellent response to steroids is a hallmark to the disease entity. It is usually a straight forward diagnosis in typical cases. Atypical CIDP often is of diagnostic concern and has been a challenge for practising neurologists. Case description Here, we present a 56-year old female presented with isolated left palatal palsy preceded by a short febrile illness. Clinically consistent with post-infectious bulbar palsy, she was treated with intravenous pulse steroids and antibiotics. She had progressive disease and later developed quadriparesis with neck flexor weakness with progressive bulbar weakness and hypercapnic respiratory failure. Discussion and evaluation MRI whole spine with contrast showed nerve root enhancement. Nerve conduction studies of all four limbs and LP and CSF analysis were consistent with CIDP. She was treated with intravenous immunoglobulin (0.4 g/kg for five consecutive days). She showed mild improvement of symptoms initially and later had deterioration of symptoms within a week interval. She received two doses of Rituximab of 1 gram each within 3 weeks apart, after which she has gradual improvement. She was able to tolerate orally and able to walk without support following 1 month after treatment, and there were no further relapses except for the persistence of minor sensory symptoms after 6 months of follow-up. Conclusion Isolated cranial nerve involvement presenting as CIDP is a rare entity. Intravenous immunoglobulin and newer immunosuppressants like CD-20 monoclonal antibodies like rituximab have a significant role in the treatment.