HUNTINGTONS DISEASE

A DISSERTATION REPORT SUBMITTED TO

MAR DIOSCORUS COLLEGE OF PHARMACY IN PARTIAL FULFILMENT
FOR THE AWARD OF THE DEGREE

BACHELOR OF PHARMACY
Under the guidance of

Mrs.SIMI.SM, M.Pharm
Assistant professor
Department of Pharmacology
Mar Dioscorus College of Pharmacy
Mount Hermon , Sreekaryam,
Thiruvananthapuram
Submitted by

SILPA.M.R
(FINAL YEAR B.PHARM)

MAR DIOSCORUS COLLEGE OF PHARMACY

MOUNT HERMON , SREEKARYAM,
THIRUVANANTHAPURAM-695017
2016-2017
1
 CERTIFICATE

This is to certify that this dissertation entitled HUNTINGTONS

DISEASE done by SILPA.M.R for the award of BACHELOR OF
PHARMACY degree comprises of bonafied work done by her in Mar
Dioscorus College Of Pharmacy, Thiruvananthapuram, under my
supervision and guidance and to my full Satisfaction.

Place:Thiruvananthapuram Mrs.SIMI.M,Mpharm

Date: Assistant professor

Department of Pharmacy Practice

Mar Dioscorus College of Pharmacy

Mount Hermon , Sreekaryam,

Thiruvananthapuram-695017

Kerala

2
 ACKNOWLEDGEMENT
First and the foremost I thank Almighty god for always being with me and
giving me energy and confidence for the work.

Primarily I thank my parents Mr.G.Muraleedharan and Mrs.Raji.P.L

and my sister Sruthy.M.R who were with me all through supporting and
encouraging me,when and where ever required,their valuable prayers took me
safely through all the hurdles in my life.

I wish to express my sincere and heartfelt gratitude to Mrs.Simi.S M

M.Pharm,Assistant professor in Pharmacology, Mar Dioscorus College Of
Pharmacy,Trivandrum,for her encouragement,inspiration,guidance and valuable
suggestions and enthusiastic support through out the course of the work.

I express my sincere gratitude to our honorable Principal Dr M.A

kuriachan,M.Pharm,Mar Dioscorus College Of Pharmacy,Trivandrum,for
giving an opportunity to do this project and also for his kind help through out
my project work.

I express my gratitude to our staff in charge Mrs.Sivazeena

T.S,M.Pharm,Assistant professor in pharmaceutical Chemistry,Mar
Dioscorus College Of Pharmacy,Trivandrum,for her sincere co-operation for the
completion of this work.

I express my gratitude to all faculty members of Mar Dioscorus College Of

Pharmacy for their unrelenting support and encouragement.

Last,but not the least,I thank each and everyone who had supported me for the
creation and completion of project.

SILPA.M.R

3
CONTENTS

CHAPTERS PAGE NO

1.INTRODUCTION 7

2.HISTORY 8

2.EPIDEMIOLOGY 9

3.ETIOLOGY 10

4.COMPLICATIONS 11

5.PATHOPHYSIOLOGY 12

6.SIGNS&SYMPTOMS 15

7.DIAGNOSIS 17

8.SUBSTANCES WITH RISK OF HUNTINGTONS DISEASE 19

9.TREATMENT AND DRUGS 20

10.MANAGEMENT 23

11.PROGNOSIS 25

12.CONCLUSION 26

13.REFERENCES 27

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 ABBREVATION

HD :Huntingtons disease
IT 15 :Interesting Transcript 15
CT :Computed Tomography
MRI :Magnetic Resonance Imaging
PET :Positron Emission Tomography
IL 16 :Interleukin 16
GABA :Gamma Amino Butyric Acid
NMS :Neuroleptic Malignant Syndrome
NMDA :N-Methyl D-Aspartate

5
 HUNTINGTONS DISEASE

An edited microscopic image of medium spiny neurons (yellow) with nuclear

inclusions (orange), which occur as part of the disease process

6
 INTRODUCTION
Huntington's disease is an inherited disease characterized by the progressive
loss of brain and muscle function.It is a dominantly inherited progressive
neurological disease characterised by chorea,an involuntary brief movement
that tends to flow between body regions.

Huntington disease (HD) is an autosomal dominant inherited neurogenerative

disease characterised by progressive motor,behavioural and cognitive
decline,resulting in death within 15 to 20 years after diagnosis[1]

The earliest symptoms are often subtle problems with mood or mental abilities.
As the disease advances, uncoordinated, jerky body movements become more
apparent. Huntington's disease was originally called Huntington's chorea
("chorea" is the Greek word for dancing). This is because the involuntary
movements associated with the condition can look like jerky dancing.

Huntington's disease is caused by an inherited faulty gene Huntington disease

(HD) is an incurable, adult-onset, autosomal dominant inherited disorder
associated with cell loss within a specific subset of neurons in the basal ganglia
and cortex[1]

Mental abilities generally decline into dementia.[ The mutant protein in

Huntington's diseasehuntingtinresults from an expanded CAG repeat
leading to a polyglutamine strand of variable length at the N-terminus[4r]

The disease is caused by an autosomal dominant mutation in either of an

individual's two copies of a gene called Huntingtin Diagnosis is by genetic
testing, which can occur at any point in time regardless of whether or not
symptoms are present. The best evidence for treatment of the movement
problems is with tetrabenazine.

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 HISTORY
Huntington's Disease was first recognised as an inherited disorder in 1872
when a 22-year-old American doctor, George Huntington, wrote a paper
called On Chorea. His paper was later published in the Medical and Surgical
Reporter of Philadelphia and the disorder he described became known as
Huntington's Chorea.

In those days, people with chorea, like the involuntary muscle jerks and
twitches characteristic of HD, were often thought to be possessed by devils. It is
believed that at least one of the alleged "witches" executed in Salem,
Massachusetts in the 1690's had HD.

Today the term Huntington's (or Huntington) Disease is more commonly

used than Huntington's Chorea. The discovery of the gene in 1993 was a
major milestone in the history of HD. The scientific community worked very
hard to find the gene and the process was long and tedious, requiring great
patience and perseverance. Nevertheless the job is not over until an effective
treatment is found. The scientists working on HD today are brilliant and
passionately committed to finding a cure. It's hoped that one day researchers
will find the answer to the puzzle of HD and support groups like this
Association will no longer have to exist.[1]
Until now, only three patients with Huntingtons disease (HD) and a neuroleptic
malignant syndrome (NMS) have been reported in the literature.

8
 EPIDEMIOLOGY

The available information on the world distribution of Huntington's disease

(HD) from population surveys and death rate analysis is summarised and
discussed in the light of genetic studies. It is concluded that most European
populations, both Northern and Southern, show a relatively high prevalence (4
8 per 100,000), and that the disorder may also be frequent in India and parts of
central Asia

. HD is notably rare in Finland and in Japan, but data for Eastern Asia and
Africa are inadequate. The disorder may have been underestimated in the
American black population[5]

Sex

HD affects males and females in relatively equal numbers.

Age

The onset of disease usually occurs in the fourth or fifth decade of life, with a
wide range in age from childhood to later years in life. Juvenile onset has a
large repeat expansion and occurs most often when the father is the affected
parent (a form of genetic anticipation).

Race
No significant differences exist among national and ethnic groups in the number
of CAG repeats; however, the higher frequency of HD among white persons
and its lower prevalence in other populations, including black persons and
Japanese persons

9
 ETIOLOGY
The faulty gene that causes Huntington's disease is found on chromosome
number 4. A normal copy of the gene produces Huntingtin a protein. The faulty
gene is larger than it should be and produces a larger form of huntingtin.

. A person with the Huntington's gene has one good copy of the gene and one
faulty copy of the gene. His/her child will inherit either the good copy or the
faulty one. The child who inherits the good copy will not develop Huntington's
disease, while the child who inherits the faulty copy will.

The child has a 50% chance of inheriting the faulty gene. If the child inherits the
faulty gene, each of his/her children will have a 50% chance of inheriting the
faulty gene.[4]

Doctors and scientists refer to the disease as an autosomal dominant disorder -

only one copy of the faulty gene, inherited from either the mother or the father,
is necessary to produce the disease.

A child who does not inherit the faulty gene will not develop HD and cannot
pass it on to his/her children. A child who inherits the faulty gene will develop
HD if he/she reaches the age when symptoms are due to emerge.

Researchers had always been presumed that brain deposits of the mutant protein
that causes the disease, called huntingtin, lured an overactive immune response.
But since the immune cells that make IL-6 also make huntingtin, it's possible
that mutant huntingtin might wrongly set these cells on attack mode throughout
the body[9]

10
 COMPLICATIONS

After the onset of Huntington's disease, a person's functional abilities gradually

worsen over time. The rate of disease progression and duration varies. The time
from disease onset to death is often about 10 to 30 years. Juvenile onset usually
results in death within 10 years.

The clinical depression associated with Huntington's disease may increase the
risk of suicide. Some research suggests that the greater risk of suicide occurs
before a diagnosis is made and in middle stages of the disease when a person
has begun to lose independence.

Eventually, a person with Huntington's disease requires help with all activities
of daily living and care. Late in the disease, he or she will likely be confined to
a bed and unable to speak. However, he or she is generally able to understand
language and has an awareness of family and friends.

Common causes of death include:

Pneumonia or other infections

Injuries related to falls

Complications related to the inability to swallow[4][5]

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 PATHOPHYSIOLOGY
Huntingtin is a 348-kDa protein that is ubiquitously expressed with highest
levels found in neurons. It is primarily cytoplasmic, but it also localizes to the
nucleus and organelles. In addition to the glutamine stretch -- the result of CAG
expansion -- the N-terminus contains a polyproline region; both are involved in
the pathophysiology. The normal functions of huntingtin have not been
completely elucidated; thus far, it has been found to play a role in myriad
normal processes including: axonal and vesicular transport, endocytosis, post-
synaptic signaling, and cell survival pathways.

Mutant huntingtin undergoes N-terminal cleavage resulting in polyglutamine

fragments, which can oligomerize and form aggregates, both cytoplasmic, and
nuclear. The N-terminal fragments, oligomers of these fragments, and the fully
formed inclusions have been implicated in the toxicity of HD. Mutant
huntingtin adversely affects a multitude of intracellular processes and causes
widespread disruption, including.

Mitochondrial dysfunction

Transcriptional dysregulation of various genes

Altered axonal transport of critical factors

Disrupted calcium signaling

Abnormal protein interactions

Alterations in proteosomal function

Autophagy[3]

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In grades 3 and 4, there is notable cell loss of extrastriatal structures (eg, the
cortex, globus pallidus, thalamus, subthalamic nucleus, substantia nigra,
cerebellum). Several elegant studies using MRI have demonstrated progressive
striatal atrophy in HD. Cortical atrophy is also apparent before the appearance
of motor symptoms, with posterior frontal regions affected earliest with
progression to involve occipital, parietal, and other cortical regions, eventually
resulting in significant and widespread thinning.

The medium-sized, spiny GABA-containing neurons of the striatum appear

to be most vulnerable to huntingtin toxicity. It has been proposed that the
"indirect pathway," comprising GABAergic striatal projections to the external
globus pallidus, is affected earlier in HD, thus resulting in loss of inhibition of
this structure, with subsequent enhanced inhibition of GABAergic projections to
the subthalamic nucleus; this produces decreased excitation of the glutamatergic
projections to the globus pallidus internus and overall diminished inhibitory
GABAergic outflow to the thalamus. The disinhibition of the thalamus allows
for enhanced excitatory outflow to the cortex, resulting in hyperkinetic or
choreic movements. As HD progresses into late stages the "direct pathway" also
becomes profoundly affected, resulting in loss of GABAergic inhibition of the
internal globus pallidus and enhanced GABAergic inhibitory outflow to the
thalamus. Thalamic inhibition of excitatory cortical connections produces a
hypokinetic or akinetic state as seen in the last stages of HD (Figure). [13]

13
Figure. Neurophysiologic changes resulting in disinhibition of thalamus in early HD and
inhibition of thalamus in late HD. D1 = dopamine 1 receptor; D2 = dopamine 2 receptor; GPe
= globus pallidus externa; GPi = globus pallidus inerna; STN = subthalamic nucleus; Thal =
thalamus

14
 SIGNS AND SYMPTOMS
The hallmark symptom of Huntington's disease is uncontrolled movement of the
arms, legs, head, face and upper body.

Early symptoms

Slight uncontrollable movements

Clumsiness

Stumbling

Some slight signs of lack of emotion

Lack of focus, slight concentration problems

Lapses in short-term memory

Depression

Mood changes - this may include antisocial behaviour and aggression.[1]

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Symptoms Affecting Movement

Involuntary writhing, shaking, jerking and uncontrolled movements

(chorea)

Lack of coordination, small unintentional motions, slow saccadic eye

movements

Rigidity, abnormal posturing

Abnormal facial expressions, difficulty chewing, swallowing and

speaking

Malnutrition and weight loss due to swallowing problems

Cognitive Disorders

Problems with planning, abstract thinking, initiation of appropriate

action, etc.

Impairment with short-term memory and deficiency in long-term memory

Dementia

Neuropsychiatric Problems

Anxiety

Depression

Loss of display of emotions

Aggression

Egocentrism[4][9]

16
 DIAGNOSIS
Diagnosis is based on a thorough personal and family medical history, physical
examination (including a neurological exam), and a series of laboratory tests.
The physician will ask about recent changes in intellectual or emotional
function, which may be early signs of Huntington's disease.

Genetic Testing and Huntington's Disease

Genetic testing involves taking a blood sample for DNA analysis to determine
whether the distinct mutation for Huntington's disease has occurred in gene IT-
15. A sample of DNA also may be required from a closely related affected
relative, ideally a parent. This helps confirm the diagnosis of HD and is
important if the family's history is in any way unclear, uncertain, or unusual.
Persons who test positive and are considering pregnancy are advised to seek
genetic counselling before they conceive.

Presymptomatic testing can be performed on adults, children, and even foetuses

in the womb. Genetic testing of a foetus holds special challenges and risks, and
some testing facilities choose not to do it.

At-risk couples wanting to have children may choose to undergo in vitro

fertilization with pre-implantation screening.

Testing Guidelines

A positive test result can have profound, unanticipated impacts on patients and
their families. Anyone contemplating genetic testing should obtain testing
guidelines from the testing center or from an organization devoted to the

17
interests of Huntington's disease patients and their families. Guidelines
recommend that testing centers follow these practices:
Counseling should be provided before and after the test, and before the
results are known.[1]
Test results should be strictly confidential and should be disclosed only in
person, and only to the individual being tested, regardless of the outcome.
To protect the interests of minors, including confidentiality, testing should
not be conducted for persons under 18 without a compelling medical reason,
such as the appearance of HD symptoms in a child.

Computed Tomography (CT scan) & Huntington's

Disease

This painless diagnostic procedure produces computer-generated images of the

brain's internal structures. Patients with HD often show shrinkage in two areas
of the brain - the caudate nuclei and put amen - and enlargement of cavities
within the brain called ventricles. The presence of these structural changes is
not conclusive for Huntington's disease nor does their absence rule it out.[2]

CT scans combined with other procedures such as magnetic resonance imaging

(MRI scan) and/or positron emission tomography (PET scan) can be a helpful
diagnostic tool, especially when evaluated in the context of family history and
clinical symptoms.

The doctor will examine the patient; ask about family history, personal medical
history, and recent emotional and intellectual changes. The doctor may also
recommend a psychiatric evaluation.[4]

18
 SUBSTANCES WITH RISK OF HUNTINGTONS
DISEASE

ALCOHOL

The research on alcohol abuse in Huntington's disease (HD) has, focused more
on prevalence than the neurochemical link between the two. The etiological role
of alcohol in inducing movement disorder in alcoholic patients, with or without
a family history of HD, was first implicated in 1970s

The glutamate excitotoxicity has been implicated in the pathophysiology of

movement disorder and other manifestations of HD. The gamma-amino butyric
acid (GABA) neuron loss in HD may also be secondary to glutamate
excitotoxicity. Alcohol has been shown to inhibit N-methyl-D-aspartate
(NMDA) receptors in rats and mice] as well as to retard the progression of HD
disorder in humans. But the chronic NMDA inhibition has also been reported to
lead to NMDA upregulation, increasing the risk of glutamate excitotoxicity.

Thus alcohol intake may initially inhibit NMDA receptors and mask the
manifestation of HD; but chronic alcohol use may lead to receptor upregulation,
resulting in NMDA excitotoxicity and manifestation of HD. In our patient, the
movement disorder became manifest under alcohol intoxication and withdrawal,
and disappeared during abstinence. Also, in his maternal cousin who had a
manifest HD, the movement disorder used to worsen during alcohol
intoxication. The findings in both these subjects cannot be explained entirely by
the NMDA mechanism underlying the movement disorder.[1][15]

19
 TREATMENT AND DRUGS
No treatments can alter the course of Huntington's disease. But medications can
lessen some symptoms of movement and psychiatric disorders. And multiple
interventions can help a person adapt to changes in his or her abilities for a
certain amount of time.

Medication management is likely to evolve over the course of the disease,

depending on the overall treatment goals. Also, drugs to treat some symptoms
may result in side effects that worsen other symptoms. [2]

Medications for movement disorders

Drugs to treat movement disorders include the following:

Tetrabenazine (Xenazine) is specifically approved by the Food and Drug

Administration to suppress the involuntary jerking and writhing movements
(chorea) associated with Huntington's disease. A serious side effect is the risk of
worsening or triggering depression or other psychiatric conditions.

Other possible side effects include drowsiness, nausea and restlessness.

Antipsychotic drugs, such as haloperidol (Haldol) and chlorpromazine, have

a side effect of suppressing movements. Therefore, they may be beneficial in
treating chorea. These drugs may, however, worsen involuntary contractions
(dystonia) and muscle rigidity.

Newer drugs, such as risperidone (Risperdal) and quetiapine (Seroquel), may

have fewer side effects but still should be used with caution, as they may also
worsen symptoms.

20
Other medications that may help suppress chorea include amantadine,
levetiracetam (Keppra) and clonazepam (Klonopin). At high doses, amantadine
can worsen the cognitive effects of Huntington's disease. It may also cause leg
swelling and skin discoloration.

Side effects of levetiracetam include nausea, stomach upset and mood swings.
Clonazepam may worsen the cognitive side effects of Huntington's disease and
cause drowsiness. It also has a high risk of dependence and abuse.[3]

Medications for psychiatric disorders

Medications to treat psychiatric disorders will vary depending on the disorders

and symptoms. Possible treatments include the following:
Antidepressants include such drugs as citalopram (Celexa, Lexapro),
fluoxetine (Prozac, Sarafem) and sertraline (Zoloft). These drugs may also
have some effect on treating obsessive-compulsive disorder. Side effects may
include nausea, diarrhea, drowsiness and low blood pressure.

Antipsychotic drugs such as quetiapine (Seroquel), risperidone

(Risperdal) and olanzapine (Zyprexa) may suppress violent outbursts,
agitation, and other symptoms of mood disorders or psychosis. However,
these drugs may cause different movement disorders themselves.

Mood-stabilizing drugs that can help prevent the highs and lows
associated with bipolar disorder include anticonvulsants, such as valproate
(Depacon), carbamazepine (Carbatrol, Epitol, Equetro) and lamotrigine
(Lamictal). Common side effects include weight gain, tremor and
gastrointestinal problems

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Psychotherapy

A psychotherapist a psychiatrist, psychologist or clinical social worker

can provide talk therapy to help a person manage behavioral problems, develop
coping strategies, manage expectations during progression of the disease and
facilitate effective communication among family members.

Speech therapy

Huntington's disease can significantly impair control of muscles of the mouth

and throat that are essential for speech, eating and swallowing. A speech
therapist can help improve your ability to speak clearly or teach you to use
communication devices such as a board covered with pictures of everyday
items and activities. Speech therapists can also address difficulties with muscles
used in eating and swallowing.

Physical therapy

A physical therapist can teach you appropriate and safe exercises that enhance
strength, flexibility, balance and coordination. These exercises can help
maintain mobility as long as possible and may reduce the risk of falls.

Instruction on appropriate posture and the use of supports to improve posture

may help lessen the severity of some movement problems.

When the use of a walker or wheelchair is required, the physical therapist can
provide instruction on appropriate use of the device and posture. Also, exercise
regimens can be adapted to suit the new level of mobility.

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 MANAGEMENT

There is no cure for HD, but there are treatments available to reduce the severity
of some of its symptoms. For many of these treatments, evidence to confirm
their effectiveness in treating symptoms of HD specifically are incomplete. As
the disease progresses the ability to care for oneself declines and carefully
managed multidisciplinary caregiving becomes increasingly
necessary. Although there have been relatively few studies of exercises and
therapies that help rehabilitate cognitive symptoms of HD, there is some
evidence for the usefulness of physical therapy, occupational therapy,
and speech therapy. An association between caffeine intake and earlier age of
onset in Huntington's disease has been found but, since this finding was based
on retrospective questionnaire data rather than a blinded, randomized trial or
case-control study, this work is a poor basis for guiding lifestyle decisions.[2]

Physical therapy

Weight loss and eating difficulties due to dysphagia and other muscle
discoordination are common, making nutrition management increasingly
important as the disease advances. Thickening agents can be added to liquids as
thicker fluids are easier and safer to swallow.] Reminding the affected person to
eat slowly and to take smaller pieces of food into the mouth may also be of use
to prevent choking. If eating becomes too hazardous or uncomfortable, the
option of using a percutaneous endoscopic gastrostomy is available. This is a
feeding tube, permanently attached through the abdomen into the stomach,
which reduces the risk of aspirating food and provides better nutritional
management. Assessment and management by speech and language
therapists with experience in Huntington's disease is recommended.

23
Medications

Tetrabenazine was approved in 2008 for treatment of chorea in Huntington's

disease in the US. Other drugs that help to reduce chorea
include neuroleptics and benzodiazepines.] Compounds such
as amantadine or remacemide are still under investigation but have shown
preliminary positive results. Hypokinesia and rigidity, especially in juvenile
cases, can be treated with antiparkinsoniandrugs, and myoclonic hyperkinesia
can be treated with valproic acid. [2]

Psychiatric symptoms can be treated with medications similar to those used in

the general population. Selective serotonin reuptake
inhibitors and mirtazapine have been recommended for depression,
while atypical antipsychotic drugs are recommended for psychosis and
behavioral problems. Specialist neuropsychiatric input is recommended as
people may require long-term treatment with multiple medications in
combination.

Education

The families of individuals who have inherited or are at risk of inheriting HD,
have generations of experience of HD which may be outdated and lack
knowledge of recent breakthroughs and improvements in genetic testing, family
planning choices, care management, and other considerations. Genetic
counseling benefits these individuals by updating their knowledge, seeking to
dispel any unfounded beliefs that they may have and helping them consider
their future options and plans. [7][14]

24
 PROGNOSIS
The length of the trinucleotide repeat accounts for 60% of the variation in the
age symptoms appear and the rate they progress. A longer repeat results in an
earlier age of onset and a faster progression of symptoms. Individuals with more
than sixty repeats often develop the disease before age 20, while those with
fewer than 40 repeats may not ever develop noticeable symptoms. The
remaining variation is due to environmental factors and other genes that
influence the mechanism of the disease. [3]

Life expectancy in HD is generally around 20 years following the onset of

visible symptoms. Most life-threatening complications result from muscle
coordination and, to a lesser extent, behavioral changes induced by declining
cognitive function. The largest risk ispneumonia, which causes death in one
third of those with HD. As the ability to synchronize movements deteriorates,
difficulty clearing the lungs and an increased risk of aspirating food or drink
both increase the risk of contracting pneumonia. The second greatest risk
is heart disease, which causes almost a quarter of fatalities of those with HD..
Suicide is the third greatest cause of fatalities, with 7.3% of those with HD
taking their own lives and up to 27% attempting to do so. It is unclear to what
extent suicidal thoughts are influenced by behavioral symptoms, as they signify
sufferers' desires to avoid the later stages of the disease. Other associated risks
include choking, physical injury from falls, and malnutrition.[8]

25
 CONCLUSION
It may be concluded that Huntingtons disease is neurodegenerative disorder
characterized by midlife. onset, involuntary movements, cognitive decline and
behavioral disturbances. It is a progressive, autosomal and inherited disorder of
the central nervous system chararacterized by widespread degenerative changes
of the cerebral cortex and basal ganglia and other brain regions and the
development of prominent chorea and dementia. Genetically, there is no way to
predict this disease prior to its occurrence. Although the diseases progression
cannot be stopped or reversed, therapies and support can partially alleviate
symptoms and improve quality of life. Treatments include medication, mental
health care and speed, swallowing and physical therapies Symptomatic
treatments of Huntingtons disease include use of Dopamine antagonists,
Presynaptic dopamine depleters, Antidepressants, Tranquilizers, Anxiolytic
Benzodiazepines, Anticonvulsants and AntibioticsSeveral medications
including baclofen, idebenone and vitamin E have been studied in clinical trials
with limited samples. More research work and clinical trials should be done to
search effective drug molecules that would provide a more rational approach in
the treatment of. Disease Huntingtons disease. The more research should be
aimed to develop therapeutic agents that alter the course of neurodegenerative
disease like Huntingtons disease by preventing neuronal death or stimulating
neuronal recovery. The goal of current research is to develop treatments that can
prevent, retard or reverse neuronal cell death. More specific treatments for
Huntingtons disease should become feasible with advances in knowledge of
their etiology[1][8]

26
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2. Frank, S (January 2014). "Treatment of Huntington's

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0244-z. PMC 3899480.PMID 24366610.

3. Dayalu, P; Albin, RL (February 2015). "Huntington disease: pathogenesis

and treatment.". Neurologic Clinics. 33 (1): 101
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4. Walker FO (2007). "Huntington's disease".Lancet. 369 (9557): 218

28. doi:10.1016/S0140-6736(07)60111-1. PMID 17240289.

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Harper P, Jones L. Huntington's Disease Third Edition. Oxford: Oxford
University Press. pp. 159189. ISBN 0-19-851060-8.

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Dementia". emedicine, WebMD. Medscape. Retrieved 16 May 2010.

7. Chorea and Huntingtons disease by International Parkinson and Movement

Disorder Society
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9.Causes,symptoms and treatment of Huntington,s disease :medical news

today.com articles/159552.php

10.Hammond K, Tatum B (26 June 2010). "The Behavioral Symptoms of

Huntington's Disease". Huntington's Outreach Project for Education, at
Stanford. Retrieved 4 August 2014.
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12.https://ghr.nlm.nih[National Library of Medicine].gov/condition/huntington-

disease

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Health/PMC/Neurotherapeutics 3899480

14.Myers, RH. Huntington's disease genetics. NeuroRx. 2004; 1: 255

262,crossref/pubmed/scopus

15.King M. Alcohol abuse in Huntington's disease. Psychol Med. 1985

Nov;15(4):815-9. PubMed abstract

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