Journal Club
December 2012
Brought to you by the AOCPMR Student Council
Article title:
Movement Disorders after Resuscitation from Cardiac Arrest
Authors:
Arun Venkatesan, MD, PhD., Steven Frucht, MD
Journal/Source:
Neurologic Clinics; Vol 24, 2006; 123-132.
Discussion:
There are many movement disorders that arise after resuscitation from cardiac arrest. Recently, there has
been an increase in the report of Lance-Adams syndrome. The increase in awareness of the syndrome has
prompted case literature reviews. This review article focuses on the investigation of posthypoxic myoclonus
(PHM), the predominate symptom observed in Lance Adams syndrome (chronic PHM). The previously rare
syndrome was discovered in 1963 by Lance and Adams and is characterized by the development of myoclonus
as a result of cerebral hypoxia after cardiac arrest.
Posthypoxic myoclonus can be divided into two types: acute and chronic. Myoclonic status epilepticus termed,
abnormal movements greater than 30 min a day, is observed in a third of patients with acute PMH, displaying
poor prognosis. Myoclonus develops with in twenty-four hours of hypoxic episodes. Literature review of
Wijdicks and colleagues research by Venkatesan and Frucht, demonstrate that multiple patients diagnosed with
acute PHM with myoclonic status epilepticus, elicite poor prognosis. Patients were either in a vegetative state or
barely survived due to the underlying brain injury, the few that did survive had a good prognosis overall.
Chronic PHM presents with the onset of myoclonus days to weeks after a hypoxic injury. This condition is also
known as Lance-Adams syndrome. This syndrome most commonly exhibits limb myoclonus. Myoclonus is
considered to be a positive movement as seen with electromyography activity (EMG studies). In addition to
positive myoclonic jerks, negative myoclonic jerks are another common symptom and is associated with
morbidity. Patients with Lance-Adams syndrome suggest a cortical, subcortical, or mixed cortical-subcortical
origination responsible for the myoclonic limb jerking.
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Currently, there is no distinction between the area of origin and the subtype of myoclonus that develops.
However, research suggests specific chemical abnormalities in the pathophysiology within the development of
chronic PHM. Venkatesan and Frucht states, low levels of a neurotransmitter, a serotonin metabolite (5-HIAA)
are indicated in CSF. Administering a serotonin precursor has improved symptoms associated with chronic
PHM. The hypothesis that there is a neurochemical pathogenesis premises has led to the development of the
research focusing on the relationship between regulation of estrogen and serotonin activity. Kompoliti’s
discovery suggests that the majority of patients with Lance-Adams syndrome are female. This lays down the
foundation for further investigation of specific hormone modulation and effects.
Treatment for chronic PHM is limited and poses a challenge for many reasons. On main challenge is the rare
occurrence and small number of patients that have Lance Adams syndrome. The current efficacy of drugs
reported in literature has been based qualitatively not quantitatively. This information is used as a guide to help
plan treatment options. The first-line drugs of choice are clonazepam, valproate, piracetum, and newly added
levetiracetam have a notable efficacy of 50% when instituted in patients. 5-HTP also demonstrates to be
efficacious with a 40% improvement in symptoms. Symptoms of myoclonus, ataxia, and speech improve over
a long period of time even without first-line medical therapy. Currently, Lance Adams syndrome is not well
understood. Clarification on hypothesizes, research ideas, and variations of treatment are all being pursued.
Venkatesan and Frucht compiled research in order to demonstrate the clinical spectrum and pathophysiology
behind Lance-Adams syndrome. Knowledge and insight gained from learning about Lance-Adams syndrome
can help physiatrists with medical management and resourcefulness of utilizing therapy modalities to their full
extent during treatment.
Reviewer:
Kathleen Minerly, OMS II, President of AOCPMR Chapter at NYCOM.
Questions:
1) What actions can increase or decrease myoclonic jerks? How can this affect the patients ADL’s?
2) How are EMG studies used to determine positive or negative myoclonus movements?
3) How do negative myoclonic jerks affect patients with Lance-Adams syndrome? What common assisted
living device is utilized to help rehabilitate?
4) In terms of medical management of chronic PHM, while on an Inpatient Traumatic Brain Injury Unit,
what drug could be used with a first-line agent drug when treating chronic PHM to help prevent severe
nausea?
5) How do disability scores in terms of ambulation and communication reflect in time? What type of
modalities can help assist with progress for the patient?
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