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 .
Activation Pattern In Parkinson’s Disease And Huntington’s Disease Patients
The major finding emerging from our investigations in Parkinson’s disease and Huntington’s 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 Parkinson’s disease and Huntington’s 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 .
Huntingtons Disease Vs Parkinsons Disease Differences
Huntingtons disease results from degeneration of neurons and structures deep within the brain, that are responsible for coordination and movement. It is an autosomal dominant genetic disorder. This actually means that you need to inherit one copy of the defective gene in order to develop the disorder.
Although symptoms of HD can develop at almost any age, they typically start when an individual is aged between 35 and 55 years old. Only 6 to 10 percent of the cases are Juvenile HD .
Parkinsons disease is an age-related progressive deterioration of certain nerve systems in the brain, that affects balance, movement, and muscle control. Symptoms develop slowly and may start off with slight tremors in one hand.
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More Information About Huntington Disease
The following is an English-language resource that may be useful. Please note that THE MANUAL is not responsible for the content of this resource.
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Genetics Home Reference: What is Huntington disease? This web site describes Huntington disease and discusses what causes it and how it is inherited, as well as provides links to its diagnosis and treatment.
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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Can Huntingtons Disease Be Treated
Currently, there is no known cure for Huntingtons disease or any treatment that can slow down the disease. The available treatments are only for symptoms in order to give the patient a better lifestyle.
For decreasing chorea, the patient is given medication that reduces the brains molecules that cause the erratic movements. These can be risperidone, olanzapine, tetrabenazine, pimozide, and many others, depending on their needs and the drugs availability.
In recent studies, tetrabenazine has proven to be the most effective against these symptoms, but the FDA hasnt approved it.
For psychiatric symptoms, there is a whole other spectrum of drugs that diminish these types of symptoms. Doctors treat irritability and depression with the same type of drugs, such as serotonin reuptake inhibitors, tricyclic antidepressants, and carbamazepine.
For psychotic symptoms like hallucinations, other drugs function for treatment like olanzapine, risperidone, clozapine, and quetiapine.
A new surgical study was recently born that consists of the transplant of neural cells with special fetal cells. It has shown improvement in some of the patients that applied to the research, but it is still too early to have precise results.
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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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.
What Is The Survival Rate Of Huntington’s Disease
The rate of disease progression and duration varies. The time from disease emergence to death is often about 10 to 30 years. Juvenile Huntington’s disease usually results in death within 10 years after symptoms develop. The clinical depression associated with Huntington’s disease may increase the risk of suicide.
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Parkinson’s & Huntington’s: What’s The Difference
- Demystifying Medicine
Many individuals are either indirectly or directly affected by Parkinson’s Disease and Huntington’s 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 Parkinson’s and Huntington’s 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
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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How Does One Get Parkinsons Disease
A patient gets Parkinsons disease due to degeneration or destruction of the nerve cells which produce dopamine. Absence of the neurotransmitter called dopamine makes it difficult for the brain to control and coordinate muscle movements which in turn produces symptoms of tremors. It must be remembered that Parkinsons disease is a progressive neurological disorder.
What Causes Huntingtons Disease
Huntingtons disease is caused by a mutation in the HTT gene. The HTT gene is responsible for making the huntingtin protein, which is thought to play an important role in nerve cells of the brain.
In Huntingtons disease, a DNA segment within this gene, called the CAG trinucleotide repeat, is repeated more often than is normal.
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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!
What Is Parkinsons Disease
Parkinsons disease is a degenerative disorder of the central nervous system mainly affecting the motor system. The motor symptoms of Parkinsons disease result from the degeneration of dopamine generating cells in the substantia nigra in the midbrain. The causes of this cell death are poorly understood. Early in the course of the disease, the most obvious symptoms are shaking, rigidity, slowness of movement and difficulty in walking and gait. Later, thinking and behavioral problems arise, with dementia commonly occurring in the advanced stages of the disease. Depression is the most common psychiatric symptom. Other symptoms include sensory, sleep problems and emotionally related problems.
Parkinsons disease is more common in older people, and most cases occur after the age of 50 when it is seen in young, it is called young onset Parkinsons disease.Diagnosis is by medical history and physical examination. There is no cure for PD, but medications, surgery, and multidisciplinary management can provide relief from the disabling symptoms. The main classes of drugs useful for treating motor symptoms are levodopa, dopamine agonists, and MAO-B inhibitors. These drugs too can cause disabling side effects. Deep brain stimulation has been tried as a treatment modality with some success.
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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.
Patient Groups Versus Controls
There were significant relative differences in vibration-evoked rCBF between patients and controls that can be summarized as follows: in Huntington’s disease, relatively decreased activation of contralateral S2 , S1 , parietal areas 39 and 40 , and lingual gyrus , bilateral prefrontal cortical areas 8, 9, 10 and 44 , along with decreased activation of contralateral basal ganglia in Parkinson’s disease, relatively decreased activation of contralateral sensorimotor cortex , lateral premotor cortex , S2 , contralateral posterior cingulate , bilateral prefrontal cortex and contralateral basal ganglia in both entities, relatively enhanced activation of ipsilateral sensory cortical areas, notably caudal S1 , S2 and insular cortex . The data are summarized in Table 6 and Table 7 .
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How Do Treatments Differ
MS treatments can ease your symptoms during an attack or slow down the diseaseâs effects on your body.
Steroids like prednisone calm the inflammation that damages your nerves.
Plasma exchange is another therapy if steroids donât work. Your doctor will use a machine to remove the plasma portion of your blood. The plasma gets mixed with a protein solution and put back into your body.
Some people with both diseases who take anti-inflammatory medicines like steroids see their Parkinsonâs symptoms get better.
Disease-modifying treatments slow down MS nerve damage and disability. They include:
National Institute for Neurological Disorders and Stroke: âTremor Fact Sheet.â
Neurology: âParkinsonâs Disease in Multiple Sclerosis – A Population-Based, Nationwide Study in Denmark .â
Mayo Clinic: âMultiple Sclerosis: Overview,â âMultiple Sclerosis: Symptoms and Causes,â âMultiple Sclerosis: Treatment,â âParkinsonâs Disease: Causes,â âParkinsonâs Disease: Definition,â âParkinsonâs Disease: Risk Factors,â âParkinsonâs Disease: Symptoms.â
Christopher Reeve Foundation: âHow the spinal cord works.â
National Association for Continence: âParkinsonâs Disease.â
National Multiple Sclerosis Society: âMS Symptoms,â âWho Gets MS? .â
National Parkinson Foundation: âNon-Motor Symptoms.â
Multiple Sclerosis Trust: âLhermitteâs sign.â
Johns Hopkins Medicine: âPlasmapheresis.â
FDA.
Cerebral Dopamine Neurotrophic Factor And Mesencephalic Astrocyte
In 2003, a protein called mesencephalic astrocyte-derived neurotrophic factor was characterized and demonstrated to promote survival of embryonic dopaminergic neurons in vitro . Then, a homologous protein called CDNF was discovered with a protective role for dopaminergic neurons. Several studies evidence the protective role of CDNF and MANF in dopaminergic neurons against the injury caused by -syn oligomers . The intrastriatal injection of CDNF prevents the loss of TH-positive neurons in a 6-OHDA-lesioned rat model of PD , and protected dopaminergic neurons in 6-OHDA and MPTP mouse models of PD . MANF has been tested in the 6-OHDA-lesioned rat model showing beneficial effects . CDNF and MANF diffuse to the brain significantly better than GDNF, and CDNF was more efficient in reducing amphetamine-induced ipsilateral rotations in the 6-OHDA rat PD model in comparison with GDNF treatment . In 6-OHDA-lesioned monkeys, PET imaging showed a significant increase of DA transporter ligand-binding activity in lesioned animals treated with CDNF .
The first phase III clinical trial using CDNF in PD patients is being conducted since 2017. In this study, an implanted drug delivery system for Ipu of recombinant human CDNF is used in patients with idiopathic mild-advanced PD . Additionally, another phase III clinical trial to evaluate the beneficial effects of CDNF in PD patients is still on course . Currently, the delivery of CDNF for HD treatment has not been described.
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Possible Link To Alzheimers
Though Alzheimers, Huntingtons, and Parkinsons are distinctly different diseases, some evidence has emerged that shows a common link between the three.
All three diseases have proteins within the cells that do not assemble properly. Though the molecular and cellular changes that occur in each disease vary greatly, this protein degradation has been shown to precede early clinical signs in each disease. This is promising news, as more studies are being done to determine whether this can either predict or prevent these neurodegenerative diseases.
Pluripotent Stem Cells As A Source Of Differentiated Cell
Pluripotent stem cells are an unlimited source of cells with the potential to give rise to any type of cell of the body. Cells differentiated from embryonic stem cells and induced pluripotent stem cells are widely used as in vitro models for many diseases, including neurodegenerative diseases, and also as a source of cell-replacement therapies. Initial studies demonstrated that, when midbrain-derived dopaminergic neurons where grafted in the striatum of rodent models of PD , long-term survival of these cells was observed, which were tyrosine hydroxylase -positive neurons, completely reversed amphetamine-induced rotational behavior and lacked neuronal overgrowth . Importantly, midbrain human dopaminergic neurons grafted in MPTP-lesioned non-human primates survived in the grafted area, expressed TH, extended fibers to the surrounding striatum, and did not present neuronal overgrowth .
As highlighted previously, since HD is caused by a genetic mutation, and differentiated MSNs progenitors come from HD patients, it is imperative to correct the mutation present in these cells, along with the replacement of the target neurons and other cell types, like interneurons and glial cells, as they may provide a healthy and functional environment for the new neurons to integrate to the local circuitry and survive. Currently, no clinical trials are assessing the use of PSCs in HD patients.
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Treatment Of Huntington Disease
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Antipsychotic and other drugs to relieve symptoms
As soon as possible after the diagnosis is made, people with Huntington disease should establish advance directives, indicating what kind of medical care they want at the end of life.
No cure exists for Huntington disease. However, certain drugs, including antipsychotic drugs may help control the agitation. Drugs that reduce the amount of dopamine , can help stop the abnormal movements..
Antidepressants can be used to treat depression, if present.