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Kennedy Disease
Get the facts on Kennedy Disease treatment, diagnosis, staging, causes, types, symptoms. Information and current news about clinical trials and trial-related data, Kennedy Disease prevention, screening, research, statistics and other Kennedy Disease related topics. We answer all your qestions about Kennedy Disease.
Question: Why is Kennedy Disease (muscular atrophy) inherited through the X chromosome but not the Y chromosome? Please answer this question if you do know, as it is not found anywhere else on the web. Help of any kind would be appreciated.
Thank you.
Or rather, Why does Kennedy disease involve the X chromosome but not the Y chromosome?
Answer: the disease is passed on the X chromosome and women are usually not affected, only carriers because if they get the disease on one X chromosome , they have another X to cover for the bad X, so they use this one instead. with boys, they have XY and if their mother passes on the bad X they don't have another X to cover for it, therefore they have the disease.
hope this helped
Question: What is the difference between Lou Gehrig's Disease and Kennedy Disease?
Answer: Kennedy is inherited and it affects males. Read the facts about them both here:
http://www.ninds.nih.gov/disorders/kenne…
http://www.ninds.nih.gov/disorders/amyot…
Question: Why was John Kennedy's long-term suffering with venereal disease left out of his autopsy?
Has this somehow affected his daughter Caroline's ability to speak/think clearly? VD certainly "rotted" the mind of Al Capone.
source: Dr. William Herbst, Kennedy's physician
Bias,
you are like Hitler and get upset over anything that bothers your image of reality.
An autopsy lists all info. It may also cite a cause of death. But, it does not ignore/fail to list other things that are not a direct cause of death.
Answer: It didn't contribute to his death, silly. And it doesn't have anything to do with his daughter.
Question: Can Kennedy disease be cure?
Answer: Unfortunately, Kennedy's Disease is considered incurable.
"Kennedy's Disease (also known as Spinal Bulbar Muscular Atrophy, SBMA, or Kennedy's Syndrome) is a rare and currently incurable and non-treatable X-linked recessive genetic progressive neuro-muscular disease."
More information:
http://www.kennedysdisease.org/
Question: what is moter neurones ,kennedy type disease about?
Answer: Kennedy’s Disease (also known as Spinal Bulbar Muscular Atrophy, SBMA, or Kennedy’s Syndrome) is a rare and currently incurable and non-treatable X-linked recessive genetic progressive neuro-muscular disease. Both the spinal and bulbar neurons are affected causing muscle weakness and wasting (atrophy) throughout the body which is most noticeable in the extremities (legs/arms), it is especially noticeable in the face and throat, and causes speech and swallowing difficulties, major muscle cramps as well as other symptoms (please see "KD Symptoms" section of this Web site for more detail).
KD is an adult-onset disease with symptoms usually appearing between the ages of 30 and 50. However, earlier onsets have been recorded.
Generally males with this inherited gene develop symptoms, while females with this gene are usually just carriers. Although, in rare cases, females have been known to exhibit symptoms as well. Life expectancy is noted to be at or almost normal.
It is estimated that 1 in 40,000 individuals worldwide have Kennedy’s Disease. However, many go misdiagnosed or not diagnosed for years. The number one misdiagnosis is Lou Gehrig’s Disease (ALS) a fatal disease.
You can find more info here:
http://www.kennedysdisease.org/about.htm…
Question: Can anyone give me more info about kennedy's disease?
Answer: Kennedy Disease is a rare, slowly progressive muscular disorder that affects males only and is inherited as an X-linked genetic trait. Uncontrollable twitching (fasciculations) followed by weakness and wasting of the muscles becomes apparent some time after the age of fifteen. The muscles of the face, lips, tongue, mouth, throat, vocal chords, trunk and limbs may be affected. Very large calves may also be found in some patients with this disorder. Kennedy disease is caused by a mutation in the androgen receptor (AR) gene. Androgen insensitivity leads to abnormal swelling of the breasts (gynecomastia), small testes and infertility.
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Resources
National Ataxia Foundation
2600 Fernbrook Lane
Suite 119
Minneapolis, MN 55447-4752
USA
Tel: 7635530020
Fax: 7635530167
Email: naf@ataxia.org
Internet: http://www.ataxia.org
Families of Spinal Muscular Atrophy
P.O. Box 196
Libertyville, IL 60048
Tel: (847)367-7620
Fax: (847)367-7623
Tel: (800)886-1762
Email: sma@fsma.org
Internet: http://www.curesma.com
Muscular Dystrophy Association
3300 E. Sunrise Dr
Tucson, AZ 85718
USA
Tel: 5205292000
Fax: 5205295300
Tel: 8003444863
Email: mda@mdausa.org
Internet: http://www.mdausa.org
National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
8A07
Bethesda, MD 20892-2540
Tel: (301)496-5751
Fax: (301)402-2186
Tel: (800)352-9424
Email: braininfo@ninds.nih.gov
Internet: http://www.ninds.nih.gov/
Kennedy's Disease Association
PO Box 1105
Coarsegold, CA 93614-1105
Tel: (559)658-5950
Email: info@kennedysdisease.org
Internet: http://www.kennedysdisease.org
For a Complete Report:
This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdbli…
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org
Question: does anyone have kennedy's disease besides me? THIS IS A NEUROLOGICAL MUSCLE DISEASE
Answer: Kennedy disease (KD) or X-linked spinal-bulbar muscle atrophy is a neuromuscular disease associated with mutations of the androgen receptor (AR). Because of its endocrine manifestations related to the impairment of the AR, it can be viewed as a variation of the disorders of the androgen insensitivity syndrome (AIS). It is named after WR Kennedy, a neurologist who was among the first to describe this disease.
Contents
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* 1 Genetics
* 2 Pathology
* 3 Signs and symptoms
o 3.1 Neuromuscular
o 3.2 Endocrine
o 3.3 Homozygous females
* 4 History
* 5 References
* 6 External links
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Genetics
As a sex-linked disease, KD affects males, while females are carriers. The gene for the AR is located on the X chromosome (Xq11-q12).
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Pathology
The distinctive AR mutation of Kennedy disease, reported in 1991, involves multiplied CAG repeats in the first exon (trinucleotide repeats). Such a CAG repeat encodes a polyglutamine tract in a part of the androgen receptor outside of the binding sites. The more CAG repeats are present, the more severe the disease. The mechanism by which this type of mutation causes neuromuscular disease is not completely understood, specifically as complete AIS does not affect neuromuscular activity. KD may share mechanistic features with other neurodegenerative disorders that are caused by polyglutamine expansion, such as Huntington's disease.
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Signs and symptoms
Ages of onset and severity of manifestations in affected males vary from adolescence to old age, but most commonly develop in middle adult life. The latest onset was described in a male of 84 years of age. KD does not usually compromise longevity. The syndrome has neuromuscular and endocrine manifestations:
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Neuromuscular
Early signs often include weakness of tongue and mouth muscles, fasciculations, and gradually increasing weakness of proximal limb muscles with muscle wasting. In some cases, premature muscle fatigue begins in adolescence. Neuromuscular management is supportive, and the disease progresses very slowly and often does not lead to extreme disability.
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Endocrine
Endocrine manifestations of this disorder are variable and rarely include underdevelopment of internal or external genitalia. In other words, most people affected with Kennedy disease are relatively normal XY men with normal fertility. However, exaggerated and persistent gynecomastia is common and often the only symptom, while in more severe forms testicular atrophy and infertility have been described. Many affected men have the mildly high LH, testosterone, and estradiol levels characteristic of other forms of the androgen insensitivity syndrome.
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Homozygous females
Homozygous females, whose both X chromosomes have a mutation leading to CAG expansion of the AR gene, show only mild symptoms of muscle cramps and twitching. No endocrinopathy has been described.
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History
This disorder was described by Kennedy in 1968. In 1991 it was recognized that the AR is involved in the disease process. The disease is probably more common than originally thought. A study in Scandinavia suggested a prevalence of 1.3/8,500 making KD the most common form of motor neuron disease in the specific area studied; nobody had been diagnosed before 1995. It has been suggested that some men with KS are may be misdiagnosed to have amyotrophic lateral sclerosis (ALS, also Lou Gehrig's disease).
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References
1. Kennedy WR, Alter M, Sung JH. Progressive proximal spinal and bulbar muscular atrophy of late onset: a sex-linked recessive trait. Neurology 1968;18:671-680. PMID 4233749.
2. La Spada A, Wilson EM, Lubahn DB, Harding AE, Fischbeck KH. Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy. Nature 1991;352:77-79.
3. Fischbeck KH, Lieberman A, Bailey CK, Abel A, Merry DE. Androgen receptor mutation in Kennedy's disease. Phil Trans R Soc Lond B 1999;354:1075-1078.
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External links
* OMIM 313200
Retrieved from "http://en.wikipedia.org/wiki/Kennedy_di…
Categories: Neurological disorders | Disability | Genetic disorders | Endocrinology | Eponymous diseases
Question: Has any one herd of Kennedy's Disease and what it is?
Answer: Kennedy's disease is an inherited motor neuron disease that affects males. It is one of a group of disorders called spinal muscular atrophy (SMA). Onset of the disease is usually between the ages of 20 and 40, although it has been diagnosed in men from their teens to their 70s. Early symptoms include tremor of the outstretched hands, muscle cramps with exertion, and fasciculations (fleeting muscle twitches visible under the skin). Eventually, individuals develop limb weakness which usually begins in the pelvic or shoulder regions. Weakness of the facial and tongue muscles may occur later in the course of the disease and often leads to dysphagia (difficulty in swallowing), dysarthria (slurring of speech), and recurrent aspiration pneumonia. Some individuals develop gynecomastia (excessive enlargement of male breasts) and low sperm count or infertility. Still others develop non-insulin-dependent diabetes mellitus.
Kennedy's disease is an x-linked recessive disease, which means the patient's mother carries the defective gene on one of her X chromosomes. Daughters of patients with Kennedy's disease are also carriers and have a 1 in 2 chance of having a son affected with the disease. Parents with concerns about their children may wish to talk to a genetic counselor.
Is there any treatment?
Currently there is no known cure for Kennedy's disease. Treatment is symptomatic and supportive. Physical therapy and rehabilitation to slow muscle weakness and atrophy may prove helpful.
What is the prognosis?
Kennedy's disease is slowly progressive. Individuals tend to remain ambulatory until late in the disease, although some may be wheelchair-bound during later stages. The life span of individuals with Kennedy's disease is usually normal.
What research is being done?
The NINDS supports a broad spectrum of research on motor neuron diseases, such as Kennedy's disease. Much of this research is aimed at increasing scientific understanding of these diseases and, ultimately, finding ways to prevent, treat, and cure them.
Organizations
Kennedy's Disease Association
P.O. Box 1105
Coarsegold, CA 93614-1105
info@kennedysdisease.org
http://www.kennedysdisease.org
Tel: 559-658-5950
Muscular Dystrophy Association
3300 East Sunrise Drive
Tucson, AZ 85718-3208
mda@mdausa.org
http://www.mda.org
Tel: 520-529-2000 800-344-4863
Fax: 520-529-5300
National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291
Question: Why was Kennedy's Long Term Suffering of Venereal Disease Left Out of His Official Autopsy?
I know Dems... if I don't give a source, it must be a lie.
http://www.time.com/time/magazine/articl…
Isn't it amazing for Dems to worship so much and know so little about their "heroes"?
Answer: That would tarnish his name. They wouldn't want to do that. They also wouldn't want to tell everyone that his secret servicemen used to bring him call girls.
Question: President John F. Kennedy had this disease whose symptoms include:? -inability to maintain glucose levels between meals
-plasma Na+ levels drop
-blood pH decreases
-K+ levels increase
-weight loss
-muscular weakness
These are symptoms of. . .
a) diabetes mellitus, type I
b) hypoadrenalism, or Addison's disease
c) hyperadrenalism, or Cushing's syndrome
d) hyperthyroidism, or Grave's disease
e) hypothyroidism, myxedema
Answer: B :)
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