Baclofen Withdrawal Presenting as Irritability in a Developmentally Delayed\nChild
A 14-year-old boy with cerebral palsy, developmental delay and seizures was brought to the\npediatric emergency department by his parents for “crying constantly,” which\nbegan 3 days prior to presentation without apparent reason. The patient had significant\ncognitive and motor impairment; he was non-verbal and communicated with minimal non-verbal\ngestures, such as smiles and grimaces. He required assistance for all activities of daily\nliving. Despite administration of ibuprofen at home, his distress increased steadily.\nSubsequently, he was unable to sleep and had decreased oral intake. The parents denied\ntrauma, fevers, ear discharge, seizure activity, hematuria, dysuria, choking episodes,\nvomiting, or diarrhea. His last bowel movement on the day prior was soft without any blood.\nThe patient has an intrathecal baclofen pump, which had not alarmed and was examined and\nrefilled two weeks prior by a neurosurgeon at another institution. His parents were unaware\nof the infusion rate of the baclofen; however, maintenance doses range from 22 micrograms to\n1.4 mg daily, depending on patient response. At the time he was taking oxcarbazapine for his\nseizure disorder; his last seizure was 18 months prior. He had scoliosis and a chronic left\nhip dislocation. The patient ate a soft pureed diet and some solids by mouth. He had no\nknown drug, food or environmental allergies.\nOn physical examination, he was crying and appeared uncomfortable, but was intermittently\nconsolable by his parents. His was afebrile, with a heart rate of 145 beats per minute,\nblood pressure 130/76 mmHg, respiratory rate 18 breaths per minute, and oxygen saturation\n99% on room air. His eyes had no excessive tearing; flourescein examination revealed\nno uptake. No foreign body was visualized in the nares or auditory canals; tympanic\nmembranes bilaterally were normal. The chest was clear and the cardiac examination was only\nsignificant for tachycardia. The abdomen was soft, non-tender and nondistended; a baclofen\npump was palpable in the right lower quadrant; a rectal examination was normal and stool was\nguaic negative. Examination of the back revealed scoliosis without pain to palpation along\nthe spine. His penile and testicular examination was normal. His extremity examination\nrevealed a deformity of the left hip and no tenderness to palpation, swelling or erythema\nalong the extremities. No skin lesions were noted. At baseline, he was awake, alert, and\nresponded non-verbally with gestures. He moved his eyes in all four quadrants, had no facial\nasymmetry, localized sounds and tracked objects by turning his head. He had 5/5 strength in\nall extremities with minor contractures that could be passively ranged bilaterally. He had\n2+ to 3+ reflexes bilaterally without clonus. His neurological examination\non the day of presentation was significant for increased tone and spasticity in all four\nextremities and clonus bilaterally at the ankles.\nComplete blood count, urinalysis and serum chemistries were within normal limits. Three\nmilligrams of midazolam was administered intravenously resulting in cessation of\npatient’s crying, relaxation of the upper and lower extremities, and decreased heart\nrate and blood pressure. An abdominal and pelvic radiograph revealed a chronic left hip\ndislocation, and discontinuity of the baclofen pump catheter (). The neurosurgical service was consulted, and\ncomputerized interrogation of the baclofen pump via wireless device revealed no recent\nalarms and a sufficient medication volume in the reservoir. Mechanical interrogation via\ninsertion of a needle into the access port was abnormal, with normal forward flow but an\ninability to aspirate cerebrospinal fluid. Due to the clinical presentation, radiographic\nfindings, and the results of the mechanical interrogation, the patient was diagnosed with a\nbaclofen pump malfunction secondary to disconnection of the tubing. In the absence of severe\nwithdrawal symptoms, 10 mg of oral baclofen 3 times daily was provided with relief of\nsymptoms, and the patient and family were transferred back to the primary neurosurgeon for\ndefinitive repair.