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April 17-18, 2019 | Frankfurt, Germany
Parkinson’s, Huntington’s & Movement Disorders
International Conference on
Journal of Brain and Neurology | Volume 3
An interesting case of Neuroacanthocytosis with head banging: A close differential of
Huntington’s Disease Like (HDL)
Raghavendra Bakki Sannegowda
Father Muller Medical College, India
N euroacanthocytosis (NA) is a rare progressive
degenerative disorder of the basal ganglia
associated with red blood cell acanthocytes.
It includes choreoacanthocytosis (ChAc) and
Macleod syndrome (MLS) as core NA syndromes.
SIBs are usually manifestated by lip and tongue-
biting and finger chewing but head scratching and
head banging are also mentioned in literature. We
report a case of ChAc who presented to us with
head banging. A 17 year old male presented to us
with 4 years history of self-injurious behaviour
(SIB) in the form of repeated head banging to
wall and other hard surfaces along with other
self-mutilating behaviour like lip biting, tongue
biting and chewing fingers. From past 2 years
patient also developed other symptoms like
abnormal involuntary movements, slurred speech
with oro-mandibular dyskinetic movements with
lingual dystonia during speech and food intake.
He also had choreo-athetoid movements which
were generalized involving both the upper and
lower limbs. Peripheral blood Smear showed 18
% acanthocytes. MRI brain revealed significant
caudate atrophy without cortical atrophy. He
was treated symptomatically with tetrabenazine,
anticholinergics and baclofen and advised regular
follow up. Though Huntington’s disease like
(HDL-2), and pantothenase kinase- associated
degeneration (PKAN), can present with similar
neurological manifestations with acanthocytes,
early presentations with autosomal recessive
inheritance, significant self-injurious behaviour
(SIB) and predominant caudate atrophy along
with specific genetic testing differentiates ChAc
from others.
Speaker Biography
Raghavendra Bakki Sannegowda is currently affiliated to Associate
Professor, Department of Neurology, Father Muller Medical College,
INDIA, continuing research in the specialized scientific area of
Neurology. He is serving as an honorary author for Journal of
Neurological Disorders & other reputed journals and has authored
several articles along with chapters in different books related to
Neurology, Neuroscience.
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