scholarly journals Do Changes in Synaptic Autophagy Underlie the Cognitive Impairments in Huntington’s Disease?

2021 ◽  
pp. 1-12
Author(s):  
Hilary Grosso Jasutkar ◽  
Ai Yamamoto
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Impairments ◽  
Critical Role ◽  
Pathological Changes ◽  
Cognitive Changes ◽  
Underlying Pathophysiology ◽  
Insight Into ◽  
Early Manifestation ◽  
Made In

Although Huntington’s disease (HD) is classically considered from the perspective of the motor syndrome, the cognitive changes in HD are prominent and often an early manifestation of disease. As such, investigating the underlying pathophysiology of cognitive changes may give insight into important and early neurodegenerative events. In this review, we first discuss evidence from both HD patients and animal models that cognitive changes correlate with early pathological changes at the synapse, an observation that is similarly made in other neurodegenerative conditions that primarily affect cognition. We then describe how autophagy plays a critical role supporting synaptic maintenance in the healthy brain, and how autophagy dysfunction in HD may thereby lead to impaired synaptic maintenance and thus early manifestations of disease.

Does CAG repeat number predict the rate of pathological changes in Huntington's disease?

1998 ◽  
Vol 44 (4) ◽  
pp. 708-709 ◽  
Author(s):  
A. Rosenblatt ◽  
R. L. Margolis ◽  
M. W. Becher ◽  
E. Aylward ◽  
M. L. Franz ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cag Repeat ◽  
Pathological Changes ◽  
Repeat Number

scholarly journals Premanifest and Early Huntington’s Disease

2014 ◽  
Author(s):  
Edward J. Wild ◽  
Sarah J. Tabrizi
Keyword(s):  
Clinical Trials ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Clinical Setting ◽  
Disease Onset ◽  
Multidisciplinary Care ◽  
Subjective Symptoms ◽  
Hd Gene ◽  
Stochastic Event ◽  
Made In

The traditional view that individuals carrying an expanded Huntington’s disease (HD) gene undergo phenoconversion, a stochastic event that takes them from symptom-free to symptomatic, is now disputed among clinicians, HD researchers, and patient and family advocates. Disease onset is officially declared when neurologic abnormalities that are unequivocally due to HD are diagnosed, but subjective symptoms and objective signs emerge gradually, and it is more helpful to consider and openly discuss a prodromal period, often as long as 10 years, which can provide a helpful framework for discussion and management. Considerable progress has been made in defining the neurobiologic processes that underlie the development of HD in humans and measures that can predict this for the purpose of conducting clinical trials, but these measures have not yet been validated sufficiently to make them useful in the clinical setting. This chapter discusses the multidisciplinary care of patients with premanifest, prodromal, and early manifest HD.

scholarly journals Insight Into the Emerging Role of Striatal Neurotransmitters in the Pathophysiology of Parkinson’s Disease and Huntington’s Disease: A Review

2019 ◽  
Vol 17 (2) ◽  
pp. 165-175 ◽  
Author(s):  
Sumit Jamwal ◽  
Puneet Kumar
Keyword(s):  
Parkinson’S Disease ◽  
Parkinson's Disease ◽  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuronal Death ◽  
Critical Role ◽  
Altered Level ◽  
Excitotoxic Neuronal Death

Alteration in neurotransmitters signaling in basal ganglia has been consistently shown to significantly contribute to the pathophysiological basis of Parkinson’s disease and Huntington’s disease. Dopamine is an important neurotransmitter which plays a critical role in coordinated body movements. Alteration in the level of brain dopamine and receptor radically contributes to irregular movements, glutamate mediated excitotoxic neuronal death and further leads to imbalance in the levels of other neurotransmitters viz. GABA, adenosine, acetylcholine and endocannabinoids. This review is based upon the data from clinical and preclinical studies to characterize the role of various striatal neurotransmitters in the pathogenesis of Parkinson’s disease and Huntington’s disease. Further, we have collected data of altered level of various neurotransmitters and their metabolites and receptor density in basal ganglia region. Although the exact mechanisms underlying neuropathology of movement disorders are not fully understood, but several mechanisms related to neurotransmitters alteration, excitotoxic neuronal death, oxidative stress, mitochondrial dysfunction, neuroinflammation are being put forward. Restoring neurotransmitters level and downstream signaling has been considered to be beneficial in the treatment of Parkinson’s disease and Huntington’s disease. Therefore, there is an urgent need to identify more specific drugs and drug targets that can restore the altered neurotransmitters level in brain and prevent/delay neurodegeneration.

scholarly journals Multimodal treatment strategies in Huntington’s disease

2021 ◽  
Vol 5 (2) ◽  
pp. 072-082
Author(s):  
Dutta Rajib
Keyword(s):  
Early Intervention ◽  
Neurodegenerative Disease ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Multimodal Treatment ◽  
Disease Onset ◽  
Genetic Diagnosis ◽  
Treatment Strategies ◽  
Emotional Lability ◽  
Made In

Huntington’s disease (HD) is an incurable neurodegenerative disease that causes involuntary movements, emotional lability, and cognitive dysfunction. HD symptoms usually develop between ages 30 and 50, but can appear as early as 2 or as late as 80 years. Currently no neuroprotective and neurorestorative interventions are available. Early multimodal intervention in HD is only possible if the genetic diagnosis is made early. Early intervention in HD is only possible if genetic diagnosis is made at the disease onset or when mild symptoms manifest. Growing evidence and understanding of HD pathomechanism has led researchers to new therapeutic targets. Here, in this article we will talk about the multimodal treatment strategies and recent advances made in this field which can be used to target the HD pathogenesis at its most proximal level.

Conduct disorder and affective disorder among the offspring of patients with Huntington's Disease

1983 ◽  
Vol 13 (1) ◽  
pp. 45-52 ◽  
Author(s):  
Susan E. Folstein ◽  
Mary Lousie Franz ◽  
Barbara A. Jensen ◽  
Gary A. Chase ◽  
Marshal F. Folstein
Keyword(s):  
Conduct Disorder ◽  
Psychiatric Disorder ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Affective Disorder ◽  
Genetic Counselling ◽  
Early Onset ◽  
Affective Illness ◽  
Hd Gene ◽  
Early Manifestation

SynopsisThe rate of occurrence of conduct disorder and affective illness was studied for a sample of 112 offspring of 34 Huntington's Disease (HD) patients. Psychiatric disorder in the offspring was assessed as a function of: (1) age of the parent at the onset of symptoms of HD; (2) family disorganization; and (3) psychiatric disorder in either parent. The findings indicated an increased frequency of conduct disorder in disrupted families, most especially in those where the HD parent had an early onset of symptoms and the non-HD parent showed psychiatric disorder. Affective disorder in the offspring was most strongly associated with the presence of similar symptoms in the HD parent. Affective disorder, but not conduct disorder, may be an early manifestation of the HD gene. The implication of these findings for genetic counselling is discussed.

Cognitive Impairments in Cerebellar Degeneration: A Comparison With Huntington’s Disease

2004 ◽  
Vol 16 (2) ◽  
pp. 176-184 ◽  
Author(s):  
Jason Brandt ◽  
Iracema Leroi ◽  
Elizabeth O’Hearn ◽  
Adam Rosenblatt ◽  
Russell L. Margolis
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Impairments ◽  
Cerebellar Degeneration
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