Pediatric Neurology 51 (2014) 573e575

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Pediatric Neurology
journal homepage: www.elsevier.com/locate/pnu

Clinical Observations

The Correlation of Dystonia Severity and Serum Transaminases

in a Child With a Brain Injury
Serge Mrkobrada MD a, *, Vithya Gnanakumar MD b
a
Alberta Children’s Hospital, University of Calgary, Alberta, Canada
b
Department of Clinical Neurosciences, Foothills Medical Centre, Alberta Children’s Hospital, University of Calgary, Alberta, Canada

abstract
BACKGROUND: Severe anoxic brain injury can lead to prolonged episodes of status dystonicus. Sustained dystonia
can result in skeletal muscle breakdown and elevation of serum transaminases, which can initially be confused
with polypharmacy-related hepatotoxicity or an underlying metabolic condition. PATIENT: We present a 19-month-
old boy who sustained a severe anoxic brain injury in the setting of a viral upper respiratory tract infection. Within
2 weeks after injury, he developed prolonged periods of severe dystonia. RESULTS: Serum creatine kinase peaked at
4504 U/L, alanine transaminase at 183 U/L, and aspartate transaminase at 198 U/L. CONCLUSIONS: This child
demonstrated a clear correlation between severity of dystonia after brain injury and changes in serum alanine
transaminase, aspartate transaminase, and creatine kinase. In the literature, aspartate transaminase and alanine
transaminase elevations have been reported in seizures, myopathies, and extreme exercise. This is the first report
of serum transaminase elevation secondary to dystonia. Early identification of skeletal muscle causes of increased
alanine transaminase and aspartate transaminase may prevent unnecessary investigations and can reduce concern
about medication-related hepatotoxicity.
Keywords: brain injuries, dystonia, rhabdomyolysis, pediatrics
Pediatr Neurol 2014; 51: 573-575
Crown Copyright Ó 2014 Published by Elsevier Inc. All rights reserved.

Introduction dystonicus, derangements of liver enzymes have been re-

ported, leading to further investigations with no underlying
Status dystonicus was first described as “increasingly cause determined.5 We present a 19-month-old boy who
frequent and severe episodes of generalized dystonia, developed status dystonicus from severe anoxic brain
which necessitates urgent hospital admission” by Jankovic injury, resulting in skeletal muscle breakdown and subse-
in 1982.1 It is a rare condition that may occur secondary to quent elevation of serum transaminases.
severe anoxic brain injury.2,3
Numerous medical problems can result, including res-
piratory failure, hyperpyrexia, dehydration, and rhabdo- Patient Description
myolysis with renal failure.4 In association with status
A previously healthy 19-month-old boy developed respiratory
distress secondary to croup. He had a witnessed respiratory arrest in the
hospital, and cardiopulmonary resuscitation was carried out for about
This study was performed at the Division of Physical Medicine & Rehabilitation,
13 minutes. His initial brain magnetic resonance imaging was consistent
Department of Clinical Neurosciences, Alberta Children’s Hospital, University of
with a severe anoxic brain injury with areas of restricted diffusion in
Calgary.
The authors have no conflicts of interest to declare. both frontoparietal lobes, the thalami, caudate heads, putamina, and the
This material was presented on March 14, 2014 at the Department of Clinical globus pallidus. Within 2 weeks of injury, he developed significant
Neuroscience Grand Rounds, University of Calgary. dystonia with ongoing posturing. During dystonic episodes, he exhibited
Article History: postures for seconds to minutes at a time with brief breaks in between,
Received May 1, 2014; Accepted in final form June 18, 2014 displaying decerebrate posturing on the right and decorticate posturing
* Communications should be addressed to: Dr. Mrkobrada; Depart- on the left. The episodes increased in severity and peaked 34 days after
ment of Clinical Neurosciences, Alberta Children’s Hospital; University respiratory arrest with more than 12 cumulative hours of dystonia per
of Calgary; 2888 Shaganappi Trail NW Calgary; Alberta, Canada T3B 6A8. day (Figure). Various oral medications were tried: gabapentin, clonaze-
E-mail address: [email protected] pam, oral baclofen, chloral, and trihexyphenidyl. The rest of his

0887-8994/$ - see front matter Crown Copyright Ó 2014 Published by Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.pediatrneurol.2014.06.012
574 S. Mrkobrada, V. Gnanakumar / Pediatric Neurology 51 (2014) 573e575

FIGURE.
Severity of dystonia and serum marker levels. The number of hours of dystonia was determined by a retrospective chart review of nursing notes. ALT, AST,
and CK levels are normalized by calculating percentage increase from upper limit of normal. CK, creatine kinase; ALT, alanine transaminase; AST, aspartate
transaminase. (Color version of this figure is available in the online edition.)

medications included clonidine and propranolol for the treatment of transaminases was due to hepatotoxicity from poly-
dysautonomia. The dystonic episodes were severe, and as a result, he pharmacy. Hepatotoxicity is a known side effect of
developed contractures of multiple joints and suspected compartment
clonidine, clonazepam, and propanolol. In addition to
syndrome of the left forearm. An intrathecal baclofen pump was inserted
50 days after respiratory arrest with significant improvement in dystonia
polypharmacy, an underlying metabolic disorder was
intensity and duration. investigated as etiology of elevated serum transaminases.
An interesting aspect of this child’s presentation was an observed A metabolic screen was ordered, but no underlying dis-
increase in serum transaminases during the hospital stay. Aspartate order was evident.
transaminase (AST) peaked at 198 U/L 36 days after respiratory arrest, The rise in AST and ALT correlated with severity of his
and alanine transaminase (ALT) peaked at 183 U/L 44 days after respi- dystonia. AST and ALT are enzymes present in various tissue
ratory arrest. The levels of serum transaminases seemed to correlate
types. AST can be evident in liver, skeletal muscle, brain,
with dystonia severity (Figure). As his dystonia improved, AST and ALT
levels slowly normalized. In spite of the elevation of serum trans-
heart, and erythrocytes.6 ALT is more specific, and elevations
aminases, other markers of liver damage, gamma-glutamyl transferase of ALT usually indicate liver damage. However, skeletal
(GGT), alkaline phosphatase (ALP), international normalized ratio (INR) muscle can also be a source of ALT, and serum transaminases
and/or partial thromboplastin time (PTT), bilirubin, and albumin, may be released into the blood stream from skeletal muscle
remained unchanged. However, serum markers of skeletal muscle injury, damage.7 Clues that suggest a skeletal muscle source of AST
creatine kinase (CK) and lactate dehydrogenase (LDH), were elevated, and/or ALT elevation include an increase in CK and LDH with
and their levels also correlated with dystonia severity (Figure). CK
no increase in GGT, bilirubin, and/or PTT.7 Additionally, an
peaked at 4504 U/L 41 days after respiratory arrest, and LDH peaked at
758 U/L 51 days after respiratory arrest.
initial AST-to-ALT ratio of 3:5 that equalizes within 4 days of
He was discharged from hospital 5 months after injury into a transaminase peaking has been reported.7 Our patient’s
community-based rehabilitation program. At the time of discharge, he presentation was consistent with a skeletal muscle source of
was able to fix and follow and progressed to being able to bring his hands transaminase elevation. In the literature, increases in AST
to the midline. Overall, his dystonia improved significantly, and although and ALT from skeletal muscle injury have been reported in
he had little dystonia at rest, he continued to have dystonia when crying seizures, myopathies, and extreme exercises.7,8 This is the
and initiating volitional movements.
first case report of transaminase elevation secondary to
dystonia. Early identification of skeletal muscle causes of
Discussion increased serum transaminases is important; it may prevent
unnecessary investigation into other causes of hepatic
We present a child who developed status dystonicus dysfunction and can reduce concern about medication-
after severe anoxic brain injury. An interesting aspect of related hepatotoxicity.
his clinical presentation was the elevation of serum
transaminases which correlated with dystonia severity. There was no financial support received for this work.
Initially it was suspected that the elevation of
 S. Mrkobrada, V. Gnanakumar / Pediatric Neurology 51 (2014) 573e575 575

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