Activation Pattern In Control Subjects
The pattern of brain activation induced by unilateral high-frequency passive vibratory stimulation in our control cohort is in keeping with previous PET data on elementary somatosensory function, as reviewed recently by Paulesu and colleagues . Our study confirms that this rather crude sensory stimulus produces strong activation in cortical areas S1 and S2. The pattern of normalized group rCBF increases in our study was markedly lateralized to the contralateral hemisphere, similar to previous reports . Transcallosal connections have, nevertheless, been demonstrated in posterior S1 , adjoining parietal cortex and secondary sensory cortical areas and, likewise, previous PET and functional MRI experiments have provided evidence for bilateral sensory cortical processing in humans. However, it remains an issue of further inquiry with higher temporal resolution imaging modalities, how ipsilateral sensory area recruitment is affected by habituation and/or other task-related issues. For instance, evoked potential recordings in cats indicate that habituation occurs more rapidly in S2 compared with S1 areas .
Finding Suggests That Treatment For One Disease Could Work For The Other Two
- Date:
- Loyola University Health System
- Summary:
- A study has found that abnormal proteins found in Alzheimer’s, Parkinson’s and Huntington’s diseases share a similar ability to cause damage when they invade brain cells. The finding suggests that an effective treatment for one neurodegenerative disease might work for other neurodegenerative diseases as well.
A Loyola University Chicago study has found that abnormal proteins found in Alzheimer’s disease, Parkinson’s disease, and Huntington’s disease all share a similar ability to cause damage when they invade brain cells.
The finding potentially could explain the mechanism by which Alzheimer’s, Parkinson’s, Huntington’s, and other neurodegenerative diseases spread within the brain and disrupt normal brain functions.
The finding also suggests that an effective treatment for one neurodegenerative disease might work for other neurodegenerative diseases as well.
The study by senior author Edward Campbell, PhD, first author William Flavin, PhD, and colleagues is published in the journal Acta Neuropathologica.
“A possible therapy would involve boosting a brain cell’s ability to degrade a clump of proteins and damaged vesicles,” Campbell said. “If we could do this in one disease, it’s a good bet the therapy would be effective in the other two diseases.”
Campbell stressed the study’s findings need to be followed up and confirmed in future studies.
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Mechanism Of Injury / Pathological Process
The most obvious neuropathology in HD occurs within the neostriatum , comprising gross atrophy of the caudate nucleus and putamen, accompanied by selective neuronal loss and astrogliosis . Marked neuronal loss also is seen in deep layers of the cerebral cortex. Other regions, including the globus pallidus, thalamus, subthalamic nucleus, substantia nigra, and cerebellum, show varying degrees of atrophy depending on the stage of the disease.
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What Are The Symptoms Of Huntingtons Disease
This disease typically does not start to affect the body until well into adulthood. Symptoms usually begin to emerge at some point between ages 30 and 50 but have also appeared as early as 2 and as late as 80. Huntingtons Disease affects people in three areas, their physical abilities, cognitive abilities, and mood regulation.
Physical The Hallmark symptom of this disease is the uncontrolled and involuntary movement of the arms, face, legs, head, and upper body.
Cognitive Huntingtons Disease also dramatically affects thinking skills. More specifically, reasoning, memory, judgment, and concentration skills are affected. There may also be significant organizational and planning skill deficits.
Mood Many people who are affected by Huntingtons Disease exhibit uncharacteristic irritability or anger. They may also develop anxiety and depression despite having very stable mental health previously. It has also been widely recorded that those suffering from Huntingtons disease are also likely to develop OCD
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Cause Signs And Symptoms Treatment And Management Age Of Onset Of Parkinsons And Huntingtons Disease:
Cause:
Parkinsons Disease: PD is caused by the degeneration of the neurons in Substantia nigra of the midbrain.
Huntingtons Disease: HD is caused by the mutations in the HTT gene.
Age of Onset:
Parkinsons Disease: PD usually occurs after the age of 50.
Huntingtons Disease: HD usually occurs in the thirties or forties.
Symptoms:
Parkinsons Disease: PD causes tremors, rigidity, slowing of movements and gait disturbances.
Huntingtons Disease: HD causes higher function abnormalities such as problems in thinking and reasoning together with characteristic chorea.
Treatment:
Parkinsons Disease: PD is treated with dopamine-enhancing drugs such as levodopa, dopamine agonists, etc.
Huntingtons Disease: HD has no curative treatment and main the treatment is supportive.
Life expediency:
Parkinsons Disease: PD doesnt have an effect on life expectancy. However, it reduces the quality of life.
Huntingtons Disease: HD patients live 15-20 years after the appearance of the first symptom.
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How Is Huntingtons Disease Treated
At this point in time, there is no cure for Huntingtons Disease. There is also no known way to slow or prevent the disease from progressing. For now, treatment focuses on the management of symptoms and optimizing the quality of life for those affected. Each person affected by Huntingtons Disease will experience their symptoms uniquely. Meaning no two patients are the same, and no two patients exhibit identical symptoms.
There are a wide variety of medications and therapies that are available to help people with Huntingtons Disease. It is important to practice patience and perseverance when beginning a new medication as it takes time to figure out how these medications interact with your body and with each other. It may take weeks or months to figure out the correct combination and dosage of medications.
Speech therapy is also a very important part of a treatment plan for Huntingtons Disease. One of our highly specialized virtual speech therapists can offer support and guidance on the road to managing symptoms. Connect with one of our speech therapists by scheduling your free introductory calltoday!
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On The Right Track To Treat Movement Disorders: Promising Therapeutic Approaches For Parkinsons And Huntingtons Disease
- 1Center for Integrative Biology, Faculty of Sciences, Universidad Mayor, Santiago, Chile
- 2Faculty of Medicine, Biomedical Neuroscience Institute, University of Chile, Santiago, Chile
- 3Program of Cellular and Molecular Biology, Institute of Biomedical Sciences, University of Chile, Santiago, Chile
- 4Center for Geroscience, Brain Health, and Metabolism, University of Chile, Santiago, Chile
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Psychotic Symptoms And Others
In addition to the symptoms we already mentioned, other symptoms may appear in both diseases. For example, in Alzheimers disease, delirium appears occasionally, while it rarely ever does in Parkinsons. Its vital to remember that delirium is an organic disorder that mainly affects consciousness and attention.
Regarding psychotic symptoms, visual hallucinations can appear in both diseases, more or less in the same proportion. Delusions may also arise. They occur often in Alzheimers and occasionally in Parkinsons.
How Can Speech Therapy Help
Huntingtons Disease affects each person differently, so the course of treatment with a speech and language pathologist will vary from patient to patient as well. To begin, the speech therapist will work to establish the areas of communication and speech that pose the greatest challenge. From there they will develop a plan to help support their client and improve not only their communication but their quality of life as a whole.
Whether it is swallowing problems or more frequent coughing and choking due to poor muscle tone or communication problems related to cognitive and thinking skills, a highly qualified speech therapist can provide an ample amount of support and guidance.
Some people whose communication is affected by Huntingtons Disease may attempt to withdraw or avoid social interaction simply because it is too difficult or painful. It is widely agreed upon that the earlier that intervention and support are introduced, the better the chance is of therapy being successful. This disease is degenerative and can change over time, so frequent and consistent time with a speech therapist can be very important.
If you or someone you love is struggling with communication due to Huntingtons Disease, there is help available. Let us help to connect you with one of our highly specialized and qualified speech therapists and schedule your free introductory call today!
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How Does Huntingtons Disease Affect Speech
In many cases, Huntingtons Disease affects ones ability to speak clearly and effectively, as this disease can cause a loss of coordination within the throat and mouth muscles that facilitate speaking and breathing. Some of the most common changes in speech due to Huntingtons Disease are slurred words, loss of control of the volume of speech, hoarseness of the voice, or improper pauses when speaking. In these cases, speech therapy can be an incredibly valuable resource for supporting someone affected by Huntingtons disease.
Will The Research Lead To A Breakthrough
Experts in these fields told Healthline this particular research does provide some encouragement.
James Hendrix, director of global science initiatives at the Alzheimers Association, said although the three diseases involve different proteins and have different effects on the brain, there is still some commonality.
He likened it to studying the motors of cars, airplanes, and boats. Although theyre different modes of transport, they still have similar engines.
Its valuable to have this cross talk. You dont want to work in a silo, Hendrix told Healthline. A discovery in one area can revolutionize another field.
George Yohrling, PhD, the senior director of mission and scientific affairs at the Huntingtons Disease Society of America, agrees.
Theyre looking at whats going on at the cellular level. What cellular machinery is being disrupted, he told Healthline.
It gets down to the cellular level, added Hendrix. If you can understand whats going wrong, you might be able to prevent that mechanism from happening.
A breakthrough is sorely needed for all these diseases.
Late last month, the Centers for Disease Control and Prevention announced that the death rate from Alzheimers in the United States increased 55 percent between 1999 and 2014.
In addition, about 50,000 people in the United States are diagnosed with Parkinsons every year. An estimated 500,000 Americans are living with the disease.
That would be wonderful, said Yohrling.
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Difference Between Huntingtons And Parkinsons
A human beings body faces several changes in its entire existence, and it undergoes various transformations. These transformations may either be physical or mental.
Considering the brain alone, a human brain at a certain human age group undergoes changes that lead to various changes. Two of these diseases are 1. Huntingtons disease, and 2. Parkinsons disease.
What Is The Life Expectancy For People With Huntingtons Disease
The life expectancy of a person that has Huntington disease will depend on when do the symptoms start. Doctors estimate that it takes approximately 15 to 20 years for the persons death when they develop symptoms. It is important to note that the estimation does not take into account that the patient commits suicide before they suffer from a fatal condition.
Studies have also shown that patients that inherit the disease from their father have an earlier onset of symptoms. This would mean that those who got the gene from their fathers will probably have a lower life expectancy.
Professionals define another form of juvenile Huntingtons disease, which happens to people younger than 20 years old. This particular form has a shorter disease duration since the symptoms appear as early as eight years old or less. It also has a different progression of symptoms comparing it to the regular disease. Rigidity is the main symptom, instead of chorea, and seizures are another common thing for these patients. Besides, there is cognitive decline and dementia, sadly, at this very early age, showing the worse of the disease progression.
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What Is Huntingtons Disease
Huntington disease usually appears in a persons thirties or forties. Early signs and symptoms can include depression, irritability, poor coordination, small involuntary movements, and trouble learning new information or making decisions. Many people with Huntington disease develop involuntary, repetitive jerking movements known as chorea. As the disease, progresses symptoms become more pronounced. People with this disorder also experience changes in personality and decrease in thinking abilities. Affected individuals usually live about 15 to 20 years after signs and symptoms begin.
There is no care for this disorder, and it is largely determined genetically due to mutations in the HTT gene. The juvenile form of this disorder also exists. Chorea can be controlled with medicines. However, other higher function abnormalities are difficult to control.
Coronal section from an MR brain scan of a patient with HD.
Gene Therapies For Pd And Hd
For the development of new therapies for PD and HD, it is important to include, especially for HD and genetic forms of PD, genetic correction/editing of the mutated gene. Nowadays, there are several gene silencing/editing technologies, including RNA interference , antisense oligonucleotides , and clustered interspaced short palindromic repeats , which can be used as therapies for the treatment of PD and HD. For a more in-depth knowledge of gene therapy delivery systems and other cellular targets, reviews are published elsewhere .
As previously stated, PD is characterized by the selective degeneration of dopaminergic neurons in the SN, thus approaches aiming to revert this loss based on the delivery of genes encoding for enzymes required for DA synthesis could be useful. The first enzyme for DA synthesis is TH, which requires the enzyme GTP-cyclohydrolase-1 to synthesize a cofactor for DA biosynthesis . TH converts tyrosine into L-dopa, which finally is converted into DA by the aromatic L-amino acid decarboxylase . Therapies to deliver enzymes involved in DA synthesis have been proved in preclinical and clinical studies showing its benefits.
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Activation Pattern In Parkinsons Disease And Huntingtons Disease Patients
The major finding emerging from our investigations in Parkinsons disease and Huntingtons disease patients is that task-related differences in cortical and subcortical sensory-evoked activation exist by comparison with healthy control subjects and, like previous H215O-PET studies on motor processing, there was a considerable degree of congruency between activation patterns in Parkinsons disease and Huntingtons disease . Among distributed areas with relative rCBF reductions, basal ganglia activation was reduced in both patient groups. This is in keeping with findings in cats rendered parkinsonian with MPTP, where marked decreases of neuronal responses to tactile stimuli have been demonstrated in globus pallidus . Similar reductions of striatal responsiveness to sensory stimulation have been reported in MPTP treated monkeys, and were reversible after apomorphine .
Ethics Approval And Consent To Participate
The study was approved by the Ethical Committee of Mahidol University Institutional Review Board, Mahidol University, Thailand and complied with the standard guideline of the Declaration of Helsinki. All participants signed an informed consent form before data collection. In addition, informed consent was obtained from all participants involved in in the study and all participants gave the permission for the publication.
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Cellular Reprogramming For Pd And Hd
In the adult brain, NSCs are present in the subventricular zone of the lateral ventricle and the subgranular zone of the dentate gyrus. These NSCs are capable of generating neuroblasts, which differentiate into mature neurons . Despite the presence of a niche for the generation of new neurons, these cells have limited migration to remote regions, like the SN and striatum. Hence, the idea to generate new local neurons from preexisting cells has been studied for the last 10 years. Initial studies have demonstrated that fibroblasts can be reprogrammed to dopaminergic neurons through the ectopic expression of transcription factors . Considering the reprogramming of cells for the treatment of neurodegenerative diseases, astrocytes were initially considered as an attractive alternative and their reprogramming to neurons forming functional synapses was demonstrated .
Recently, using AAV-based reprogramming of striatal astrocytes, Wu et al. demonstrated that astrocytes could be converted to MSNs in the striatum of R6/2 and YAC128 mice. Converted neurons expressed specific MSNs markers, showed electrophysiological properties, and projected their axonal terminals to the GP and SNpr. All these findings were accompanied by a reduction in striatal atrophy, attenuation of the phenotypic deficit, and an extended life span of R6/2 mice with converted MSNs .
What Is The Difference Between Huntingtons Disease And Parkinsons
Unfortunately, many people know someone who is affected by Huntingtons Disease or Parkinsons disease. The key difference between these two degenerative brain disorders is that while Huntingtons Disease causes emotional difficulties, cognitive abilities, and abnormal repetitive movements, Parkinsons causes a slowing of movements and affects mobility and muscle control due to rapid brain degeneration.
Some of the other significant differences between the two are:
Stage of Life When Symptoms Appear: While Huntingtons Disease symptoms can begin to appear in early adulthood, Parkinsons Disease more commonly affects those who are 70 or older.
Cause: Huntingtons Disease is caused by a defective gene, whereas Parkinsons is caused by the degeneration of neurons in the center of the brain.
Life Expectancy Sadly, those affected by Huntingtons Disease are expected to live only 15-20 years after the first onset of symptoms. Conversely, Parkinsons has no effect on life expectancy but can dramatically reduce the quality of life for those affected.
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Parkinsons & Huntingtons: Whats The Difference
- Demystifying Medicine
Many individuals are either indirectly or directly affected by Parkinsons Disease and Huntingtons Disease. However, most people do not know what causes their loved ones to develop these diseases. This video will explain the motor circuit in the brain that is responsible for Parkinsons and Huntingtons Disease and how they differ from each other. Furthermore, patients Sarah and Jade describe their daily lives as well as how they manage the symptoms of their disease.
References
Depression With Huntingtons Disease
Due to the nature and lower life expectancy of Huntingtons disease, it is common for a diagnosis to lead to depression. Patients with Huntingtons are at a higher risk of suicide.
If you are struggling with your Huntingtons diagnosis or prognosis, contact the Substance Abuse and Mental Health Services Administration National Helpline online or call 1-800-662-4357 to seek help.
If you are having suicidal thoughts, dial 988 to contact the 988 Suicide & Crisis Lifeline and connect with a trained counselor. If you or a loved one are in immediate danger, call 911.
For more mental health resources, including a helpful list of links and hotline numbers, see our National Helpline Database.
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