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Table 1 Summary of reported cases of ITB withdrawal

From: Status dystonicus resembling the intrathecal baclofen withdrawal syndrome: a case report and review of the literature

Author/Reference

Patient and Primary Diagnosis

Reason for ITB failure

Features Reported (those in italics were not a feature of the case we report)

Management and Outcome

Hansen et al. [12]

11 F Cerebral Palsy

Removal of infected ITB Pump several hours previously

Dystonia, Pyrexia, Rhabdomyolysis, Tachycardia, Hypertension, Reduced O2 Saturation, Diaphoresis

Admitted to ICU and continuously sedated. Oral baclofen, dantrium, cyproheptadine, propofol and clonidine. Pump replaced with good result.

Samson-Fang et al. [7]

9 M Cerebral Palsy

Disconnection of distal tubing from proximal segment. Time to onset unknown.

Dystonia, Pyrexia (39.5°C), Rhabdomyolysis (15,200 IU/L), Agitation, Hypertension, Seizure, Scrotal and Ankle Oedema, Clotting Derangement, Metabolic Acidosis, Transaminase Elevation, Leukocytosis, Renal Failure

Oral baclofen and clorazepate. Pump system revised. Patient subsequently deferevesced and tone returned to baseline.

Kao et al. [4]

4 M Cerebral Palsy

Pump noted to be empty and beeping. Time to onset unknown.

Dystonia, Pyrexia (42.1°C), Tachycardia, Tachypnoea, Hypertension, Reduced O2 Saturation, Wheeze, Crepitations, Leukocytosis

Admitted to PICU. Paracetamol and bronchodilators. Following pump refill, fever and tachycardia resolved over four to eight hours. Recovery.

Salazar et al. [13]

14 M Cerebral Palsy

Less than 1 mL left in pump reservoir

Dystonia, Rhabdomyolysis (2500 IU/L), Tachycardia, Agitation, Hypertension, Diaphoresis, Haematemesis, Melaena, Clotting Derangement, Transaminase Elevation

Pump refilled. Diazepam and cyproheptadine used to control tone. Discharged on hospital day four.

Saveika et al. [14]

14 F Cerebral Palsy

Pump Stall 8 hrs previously

Dystonia, Pyrexia (37.8°C), Tachycardia, Agitation, Pruritis

Oral baclofen and cyproheptadine. Recovery within 24 hours.

Alden et al. [5]

14 M Adrenoleukodystrophy

Catheter failure 1 week following spinal surgery

Dystonia, Pyrexia (40°C), Tachypnoea

IV lorazepam and oral baclofen. Pump and catheter revision. Discharged on post-operative day two.

Zuckerbraun et al. [15]

7 F Traumatic Brain Injury

Due for refill the following day

Dystonia, Pyrexia (40.8°C), Tachycardia, Impaired Consciousness, Tachypnoea, Diaphoresis, Seizure, Leukocytosis

Baclofen bolus and pump refill. Defervesced over four hours.

Zuckerbraun et al. [15]

14 M Traumatic Brain Injury

ITB catheter disconnection. Time to onset unknown.

Dystonia, Pyrexia (41°C), Tachycardia, Agitation, Hypotension

Four day admission. Misdiagnosed with viral illness and discharged. Withdrawal episode diagnosed on the basis of a subsequently identified catheter disconnection. Catheter subsequently revised with an improvement in tone.