Rare Cause of Type 2 Respiratory Failure: Arnold Chiari Malformation.

S y r i n g o m y e l i a i . e . I m p a i r e d neuromuscular transmission is a rare cause (0.01%) of type 2 respiratory failure. Here i am discussing a case who presented with hypercapnic respiratory failure and on evolution found to be afflicted with extensive syrinx involving cervical spinal cord. A prediabetic 45 yrs old lady presented in our casualty with complaints of cough with expectoration, generalized body weakness, unable to walk since 10 days prior to admission, and swelling over feet since 2 days. On investigations she was found to have type 2 respiratory failure with cor pulmonale. There was no evidence of pulmonary embolism. CBC, liver and kidney function tests were normal. RA factor, and ANA were negat ive . She was managed with nebulization, BIPAP, antibiotics, iv s teroids and other support ive treatment. She responded well and discharged on BIPAP 20/10 and other supportive management. On follow up visit She was doing well with respect to her respiratory effort, but still she was not able to stand and walk on her own and complained of increase weakness in both upper arms. MRI brain and spine was ordered for her neurological weakness. This revealed a syringomyelia with myelomalacia at cervical cord. Syringomyelia and other neuromuscular disorders leads to increased load versus strength in respiration. This type of hypoventilation causes symptoms like excessive day time somnolence, poor quality sleep, and orthopnea.