DISORDERS OF THE

BASAL GANGLIA
GROUP 6
 INTRODUCTION
 Basal ganglia are scattered masses of gray matter submerged in
subcortical substance of cerebral hemisphere.
 The components of the basal ganglia include;
1. Corpus striatum
2. Substantia nigra
3. Subthalamic nucleus of Luys.
FUNCTIONS

1. Voluntary movement
2. Postural control
3. Control of muscle tone
4. E.t.c
 BASAL GANGLIA DISORDERS

 Classified as hyperkinetic or hypokinetic movements.

 Include;
1. Huntington chorea
2. Parkinson
3. Wilson disease
4. Hemiballismus
5. Kernicterus
6. Athetosis
7. Chorea
 COMMON DISORDERS

PARKINSON DISEASE
 AKA paralysis agitans or parkinsonism
 Characterised by hypokinesia (akynesia and bradykinesia).rigidity, tremor at rest
 Pathophysiology: result from degeneration of dopaminergic neurons in pars compacta
of substantia nigra.
 As a result there is reduced dopaminergic effect on the GABA secreting neurons in the
corpus striatum.
 Reduction in dopaminergic effect leads to under-stimulation of the motor cortex hence
hypokinesia,
 HUNTINGTON DISEASE
 An inherited progressive neural disorder due to the degeneration of neurons
secreting GABA in corpus striatum and substantia nigra.
 There is mutation of the gene that codes for a protein called huntington(a
protein whose function is not yet known) Autosomal dominant at
chromosome4
 Characterized by abnormal movement -chorea, hypotonia and dementia
 The abnormal movements of Huntington’s disease are believed to be caused by
loss of most of the cell bodies of the GABA-secreting neurons in the caudate
nucleus and putamen and of acetylcholine-secreting neurons in many parts of
the brain
 Mostly in middle age
 dementia in Huntington’s disease results from the loss of acetylcholine-
secreting neurons, perhaps especially in the thinking areas of the cerebral
cortex.
WILSON DISEASE

 AKA hepatolenticular degeneration

 Rare disorder of copper metabolism
 It is a genetic autosomal recessive disorder characterised by yellow
kayser-Fleischer rings in the eyes
 motor disturbances which include wing beating tremor or
asterixis ,dysarthria unsteady gait and rigidity.
 KERNICTERUS
 Form of brain damage in infants due to severe jaundice
 Develops due to high bilirubin content after the survives anaemia in erythroblastosis fetalis
 The bilirubin enters the brain and causes permanent brain damage as the brain barrier is not
fully developed
 Affected parts of the brain are basal ganglia, hippocampus, geniculate bodies, cerebellum and
cranial nerve nuclei.
 FEATURES

 As the brain damage starts, the baby becomes lethargic and sleepy.
 As the disease progresses,the baby develops hypertonia and opisthotonus
 As the disease advances,the signs also advances which include; inability to suckle milk,
irritability and crying, bicycling movements, choreoathetosis, spasticity, fever and coma.
 HEMIBALLISMUS
 Disorder characterized by violent involuntary abnormal movements on one side of the
body mostly the arm.
 Rare movement disorder
 Occurs due to degeneration of subthalamic nucleus of Luys
• The subthalamic nucleus is a diancephalic gray matter portion of the basal ganglia
• It’s the only portion of the ganglia that produces an excitatory neurotransmitter,
glutamate.
• Stilmulates the SNr-Gpi complex
• With time  chorea
 Ballismus, chorea, Athotosis and Dystonia

 These should not be thought of as separate entities amenable to

specific definition but rather as a SPECTRUM of movements that
blend into one another

Ballismus Chorea Athetosis Dystonia

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