Abstract
Paroxysmal sympathetic hyperactivity (PSH) is a clinical syndrome of agitation and
involuntary motor activity that particularly occurs in patients with severe acquired
brain injury. The aim of the present study is to substantiate the assertion that paroxysmal
non-epileptic attacks resembling PSH also occur in patients with Juvenile Neuronal
Ceroid Lipofuscinosis (JNCL, Batten disease), which is the most common neurodegenerative
disease in children.
The paper describes a case series of five patients with JNCL which during a period
of fifteen years have been followed clinically and by consecutive investigations of
the autonomic nervous system using heart rate variability (HRV) investigations. Following
adolescence a significant autonomic imbalance with very low parasympathetic activity
and an unchanged high sympathetic excitatory activity was documented. In addition,
episodes of anxiety and agitation combined with involuntary movements were reported.
Beyond the frightened facial expression and involuntary increased motor activity,
excessive sweating, increased body temperature, high heart and respiratory rates were
reported, and typically, the episodes occurred to stimuli that were either non-nociceptive
or only minimally nociceptive. Thus, from a clinical point of view the non-epileptic
paroxysmal condition with anxious behavior, agitation and motor hyperactivity seen
in patients with JNCL fits to the clinical description of PSH which normally occurs
following acutely acquired brain injury, and as the neuropathological basis in JNCL
for development of PSH is similar to what is seen in patients with traumatic brain
injuries, it seems reasonable to propose that PSH also occurs following adolescence
in patients with JNCL.
Keywords
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Article info
Publication history
Published online: July 28, 2018
Accepted:
July 28,
2018
Received:
March 15,
2018
Identification
Copyright
© 2018 Elsevier B.V. All rights reserved.
