Spasticity / Movement Disorders
Pearl Galido, DO
Resident Physician
Northwell
Manhasset, New York, United States
Lisa Hu, MD
Attending Physician, MD
Northwell
Glen Cove, New York, United States
Janice Lester, MLS
Senior Librarian
Northwell
New Hyde Park, New York, United States
Pearl Galido, DO
Northwell
Manhasset, New York, United States
A 61-year-old male survivor of assault sustained right temporal-parietal depressed skull fracture, subarachnoid hemorrhage, parenchymal contusions, facial fractures, and neck hematoma requiring urgent right frontal craniectomy, jaw fixation, and wiring. Acute hospital course was complicated by two episodes of cardiac arrest, the first for an estimated 5-10 minutes due to need for wire cutting, and the second for 5 minutes. Following successful cardiopulmonary resuscitation, the patient had worsening motor control and incoordination.
At the acute inpatient rehabilitation facility, physical exam was remarkable for severe dysmetria, motor and truncal ataxia, and myoclonic motions with activation of all extremities. A neuromuscular movement disorders consult was requested and electroencephalogram (EEG) performed after which the patient was diagnosed with Lance-Adam's syndrome. Treatment plan for the patient includes trial of valproic acid, zonisamide and clonazepam. With the support of occupation, physical, and speech therapy, the patient is slowly progressing towards more controlled and purposeful movements.
Discussions: Lance-Adams syndrome is a rare condition described as active myoclonus that affects patients who survive cardiopulmonary arrest. Anoxic brain injury is hypothesized as the etiology of the condition. A standard treatment is not yet recognized, but multiple cases report the use of benzodiazepines and anti-epileptic drugs as a promising treatment.
Conclusions: For patients that survive cardiopulmonary arrest and later present with high amplitude, low frequency jerking movements during muscle activation, Lance-Adams syndrome should be considered in the differential.