Basal Ganglia

An Inclusive Overview

Neurosciences
Posterior Coronal View
 Motor (Putamen) Circuit
❖ Motor circuit is for executing learned patterns of
movement

❖ Circuit:
- Premotor + supplementary motor + somatosensory
areas → putamen (bypassing caudate) → globus
pallidus internus → VA, VL nuclei of thalamus →
primary motor + premotor + supplementary motor
cortices

❖ Thus, the putamen circuit has its inputs mainly

from the parts of the brain adjacent to the primary
motor cortex but not much from the primary
motor cortex itself

❖ Then its outputs do go mainly back to the primary

motor cortex or closely associated premotor and
supplementary cortex
 Cognitive (Caudate) Circuit
❖ The prefrontal (cognitive) circuit is probably important in
cognitive processes involving the frontal lobes

❖ Input is primarily from the head of the caudate, and

output reaches the prefrontal cortex

❖ Circuit:
- Cerebral cortex → caudate nucleus → globus pallidus
internus → VA, VL nuclei of thalamus → prefrontal +
premotor + supplementary motor areas of the cerebral
cortex

❖ Almost none of the returning signals pass directly to the

primary motor cortex

❖ Instead, the returning signals go to the accessory motor

regions in the premotor and supplementary motor areas
that are concerned with putting together sequential
patterns of movement lasting 5 or more seconds instead
of exciting individual muscle movements
 Parkinson’s Disease
❖ Parkinson disease (PD), also known as idiopathic
parkinsonism, is a neurodegenerative disease and
movement disorder characterized by resting tremor,
rigidity and hypokinesia due to progressive degeneration
of dopaminergic neurons in the substantia nigra

❖ Parkinson disease is by far the most common cause of

the parkinsonian syndrome, accounting for
approximately 80% of cases

❖ The dopaminergic tract is predominantly affected in

Parkinson disease, and histologically, it is characterized
by nigrostriatal dopaminergic degeneration leading to
neuronal loss in the substantia nigra pars compacta

❖ The mainstay of treatment is medical

❖ In patients with refractory symptoms, deep brain

stimulation may be useful
 Huntington's Disease
❖ Huntington disease (HD), also known as Huntington chorea, is
an autosomal dominant neurodegenerative disease
characterized by a loss of GABAergic neurons of the basal
ganglia, especially atrophy of the caudate nucleus and
putamen (dorsal striatum)

❖ Huntington disease is clinically characterized by progressive

unintentional choreoathetoid movements, subcortical type
dementia, behavioral changes, and psychosis which starts in
midlife

❖ On imaging, it is classically characterized by atrophy of the

caudate nucleus with concomitant enlargement of the frontal
horns of the lateral ventricles

❖ The mutation responsible is on chromosome 4p16:3 and

consists of a CAG trinucleotide repeat

❖ No treatment is currently generally available

❖ The adult-onset form is slower in its course and inevitably leads
to death in 14-15 years, whereas the juvenile form has a more
rapidly progressive course, with death occurring in 7-8 years
 Wilson's Disease
❖ Wilson disease is an autosomal recessive disorder that occurs in 1 of
30,000 individuals

❖ The abnormal gene, the ATP7B (adenosine triphosphate) gene, is

located on chromosome 13

❖ The defective protein, adenosine triphosphatase (ATPase), is involved in

the transport and incorporation of copper into ceruloplasmin and the
vesicular compartment near the canalicular membrane for further bile
excretion

❖ Although a neurologic disorder, it affects multiple organs, with the liver

being the most common and earliest affected

❖ Neurologic manifestations include dysarthria, dystonia, rigidity, wing

beating tremor, and choreoathetosis

❖ The Kayser-Fleischer ring, the classic ophthalmologic sign of the

disease, is a yellow-brown discoloration of the Descemet membrane,
best demonstrated by slit-lamp examination

❖ The copper-chelating agent D-penicillamine has been considered the

gold standard of therapy
 Sydenham Chorea
❖ Sydenham chorea (SC) is a neurological disorder of childhood
resulting from infection via Group A beta hemolytic streptococcus
(GABHS), the bacterium that causes rheumatic fever

❖ SC is characterized by rapid, irregular, and aimless involuntary

movements of the arms and legs, trunk, and facial muscles

❖ It affects girls more often than boys and typically occurs between 5
and 15 years of age

❖ Some children will have a sore throat several weeks before the
symptoms begin, but the disorder can also strike up to 6 months
after the fever or infection has cleared

❖ Due to better sanitary conditions and the use of antibiotics to treat

streptococcal infections, rheumatic fever, and consequently SC, are
rare in North America and Europe

❖ The disease can still be found in developing nations

https://www.ninds.nih.gov/disorders/all-
disorders/sydenham-chorea-information-page ❖ Most children recover completely from SC, although a small number
will continue to have disabling, persistent chorea despite treatment
Differentiation and Diagnosis of Tremor - AAFP
 Tremor Clinical Features Treatment
• Bilateral action tremor of the hands, usually • Propranolol (Inderal)
without leg involvement • Primidone (Mysoline)
• Possible isolated head tremor without • Surgery
Essential
dystonia
• Usually no other neurologic signs
• Relieved with alcohol in many cases
• Resting tremor (4-6 Hz) that decreases with • Dopamine agonists
voluntary movement • Anticholinergics
Parkinson's • Usually involves legs & hands • Surgery
• Facial involvement less common • Deep brain
stimulation
• Usually associated with ataxia, dysmetria, • Treat underlying
or gait disorder cause
Cerebellar • Tremor increases steadily as the hand • Deep brain
reaches its target stimulation
• Low amplitude (10-12 Hz) not visible under • Treat underlying
normal conditions cause
• Acute onset with increased sympathetic • Reassurance
Physiologic activity (eg, drugs, hyperthyroidism,
anxiety, caffeine)
• Usually worse with movement & can
involve the face and extremities
Differentiation and Diagnosis of Tremor - AAFP
 References
➢ Netter’s Nervous System – 2nd Edition
➢ Guyton & Hall Medical Physiology – 14th Edition
➢ Purves Neurosciences – 6th Edition
➢ Neuroanatomy Through Clinical Cases – 2nd Edition
➢ Physeo YouTube Channel
➢ Boards & Beyond
➢ Amboss Website
➢ Calgary Guide Website
➢ KSU Medical Cloud