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 ___ Neurological Disorders: Huntington's Disease ___ |
Huntington's Disease - Brain Regions and their Dysfunctions
Huntington's disease"The disease results from an expanded sequence of CAG repeats in the huntingtin gene and leads to a movement disorder with associated cognitive and systemic deficits." S"Several mechanisms have been identified that mediate neuronal dysfunction and death; these include neuronal aggregation of the mutated protein, transcriptional dysregulation, excitotoxicity, altered energy metabolism, impaired axonal transport, and altered synaptic transmission." S |
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| Affected Region | Dysfunctions |
| Parietal lobe | Induced dysphoric mood was associated with a widespread reduction of activity within the parietal lobes S |
| Insular lobe | Thinning of the gray matter in the insula and of subinsular white matter S |
| Temporal lobe | Thinning of the gray matter in the middle temporal gyri and of right temporal lobe white matter S |
| Hippocampus | Induced dysphoric mood was associated with an increase of activity in the posterior hippocampus S |
| Striatum |
Adenosine A(2A) receptors in the striatum play an important modulatory role of
glutamatergic transmission to the GABAergic enkephalinergic neuron, which function is particularly
compromised in the early stages of Huntington's disease
S
Induced dysphoric mood was associated with an increase of activity in the striate and extrastriate cortex S |
| Caudate nucleus |
Decreased activation in the left caudate nucleus
during finger tapping of the right hand and high-resolution T1-weighted images:
the decreased output of the motor basal ganglia-thalamo-cortical circuit
leads to a compensatory recruitment of accessory motor pathways
(right supplementary motor area and supramarginal gyrus and left intraparietal sulcus)
S
Smaller caudate nucleus volumes predicted lower dorsal-lateral prefrontal activity during induced dysphoric mood S |
| Globus pallidus | Significant grey matter volume loss in the globus pallidus externa S |
| Putamen | Significant grey matter volume loss in the bilateral putamen S |
| Thalamus | Induced dysphoric mood was associated with a reduction of activity within the thalamus (left thalamus: increased activity) S |
| Superior colliculus | Anomalous regulation of distractor-related activity (mediated by connections between basal-ganglia-thalamo-cortical circuitry and the SC) S |
| Red nucleus | SLI (somatostatin-like immunoreactivity) and NPYLI (neuropeptide Y-like immunoreactivity) are significantly increased in the red nucleus S |
| Substantia nigra | Significant grey matter volume loss within the left ventral midbrain in the region of the substantia nigra S |
| Cerebellum | Induced dysphoric mood was associated with a widespread reduction of activity within the cerebellum S |
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