Ramam Sripada et al., Int. J. Res. Pharm. Sci., 2020, 11(3), 3761-3767
REVIEW ARTICLE
INTERNATIONAL JOURNAL OF RESEARCH IN
PHARMACEUTICAL SCIENCES
Published by JK Welfare & Pharmascope Foundation Journal Home Page: www.pharmascope.org/ijrps
Huntington’s Disease- An Updated Review
Hima Bindu Mylapalli
1
, Ramam Sripada
*2
, Sireesha Maram
1
, Sudeepthi Padala
1
,
Aksha Susmitha Jangam
1
, Dasaratha Dhanaraju Magharla
1
1
Department of Pharmacy Practice, GIET School of Pharmacy, Rajahmundry, Andhra Pradesh, India
2
Department of Pharmacy Practice, GIET School of Pharmacy, Rajahmundry, Andhra Pradesh, India
Article History:
Received on: 05 May 2020
Revised on: 05 Jun 2020
Accepted on: 10 Jun 2020
Keywords:
Chorea,
Dystonia,
Huntington’s disease
ABSTRACT
Huntington’s disease (HD) is a rare autosomal dominant, fatal neurodegenera-
tive disorder of the central nervous system characterized by unwanted chore-
atic movements, behavioural disruption, psychiatric disturbances and demen-
tia. This condition is characterized by progressive degeneration of neurons
within the basal ganglia, primarily the caudate and the putamen. As the dis-
ease progresses, neuronal losses occur in the white matter, cerebral cortex
and thalamus. In this article, the authors reviewed the genetic aspects, etio-
logical factors, stages of the disease condition along with the signs and symp-
toms, various diagnostic procedures besides with the pharmacological and
non-pharmacological management of the Huntington’s disease. This disease
is inherited within the families, and the pathophysiology of Huntington dis-
ease is restricted to the brain, where degeneration begins initially in the stria-
tum, spreads to the cortex and eventually appears throughout the brain.The
pathogenesis of this disease is still unrevealed, and there is no treatment avail-
able for the cure of the disease. There were many drugs of choices avail-
able for symptomatic treatment aiming to improve the quality of life in the
patient. The non-pharmacological therapy for managing Huntington’s disease
includes physiotherapy, speech therapy and psychotherapy.At a therapeutic
setting, all the needs of the patients are to be addressed as the advancement
in the development of new therapeutic agents are paving the way for the better
outcomes in the management of Huntington’s disease and thereby promising
better healthcare for these patients.
*
Corresponding Author
Name: Ramam Sripada
Phone: 8374214213
Email: ramampharmd7@gmail.com
ISSN: 0975-7538
DOI: https://doi.org/10.26452/ijrps.v11i3.2545
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INTRODUCTION
Huntington’s disease (HD) is a rare autosomal domi-
nant, fatal neurodegenerative disorder of the central
nervous system characterized by unwanted chore-
atic movements, behavioural and psychiatric distur-
bances and dementia (Raymund, 2010). This con-
dition is characterized by progressive degeneration
of neurons within the basal ganglia, primarily the
caudate and the putamen (Bilney et al., 2003). As
the disease progresses, neuronal losses occur in the
white matter, cerebral cortex and thalamus (Von-
sattel et al., 1985). Huntington’s disease is char-
acterized by both voluntary as well as involuntary
movement disorders. Voluntary movements can be
affected by bradykinesia and akinesia, resulting in
difϑiculty in initiating movements. Postural stabil-
ity may also be impaired due to loss of balance dur-
ing movements resulting in sudden and frequent
falls (Kirkwood et al., 2001). Involuntary move-
ments include chorea, athetosis,dystonia and tics
© International Journal of Research in Pharmaceutical Sciences 3761