PHYSIOLOGY

OF

BASAL GANGLIA
By Dr. Mudassar Ali Roomi (MBBS, M.Phil.) Assistant Professor Physiology

BASAL GANGLIA
These are masses of grey matter present in the white matter of cerebral hemisphere. These include 5 nuclei:
1. 2. 3. 4. 5. Caudate nucleus Putaman nucleus Globus pallidus Substantia nigra Sub thalamic nucleus

BASAL GANGLIA
Caudate + putaman = corpus neostriatum. Globus palidus makes the paleostriatum. Caudate is separated from putaman by internal capsule. Putaman + globus pallidus = lentiform / lenticular nucleus.

CONNECTIONS OF BASAL GANGLIA:

(2 important circuits)
1. PUTAMAN CIRCUIT 2. CAUDATE CIRCUIT

PUTAMAN CIRCUIT:
RECIEVES FIBERS FROM:
Pre motor area Supplementary motor area & Somatosensory areas

SENDS FIBERS TO:

Globus pallidus V.A.T.N. & V.L.T.N. Primary motor area, supplementary motor area & pre motor areas.

CAUDATE CIRCUIT
RECIEVES FIBERS FROM:
Cerebral cortex (including: pre motor & supplementary motor area)

SENDS FIBERS TO:

GLOBUS PALLIDUS V.A.T.N. & V.L.T.N. pre motor & supplementary motor areas & to prefrontal cortex. Note: no fiber from this circuit go to primary motor area.

NEURO TRANSMITTERS IN BASAL GANGLIA

1) CORTICO-STRIATE FIBERS release ACETYLCHOLINE at their nerve endings (cholinergic fibers). 2) NIGRO-STRIATE FIBERS (fibers which pass from substantia nigra caudate & putaman ) secrete DOPAMINE at their nerve endings (dopaminergic fibers).

NEURO TRANSMITTERS IN BASAL GANGLIA:

3) fibers which pass from caudate & putaman globus pallidus & substantia nigra secrete GABA (Gamma Amino Butyric Acid) at their nerve endings. 4) fibers which pass from brain stem basal ganglia secrete NOREPINEPHRINE, SEROTONIN & ENKEPHALIN at their nerve endings.

 Here 3 neuro transmitters are important: ACETYLCHOLINE excitatory DOPAMINE & GABA inhibitory

FUNCTIONS OF BASAL GANGLIA:

As ACCESSORY motor system. Do not function independently but with the help of CEREBRAL CORTEX & CORTICO-SPINAL SYSTEM.

1) CONTROL OF COMPLEX & SKILLED MOVEMENTS: BASAL GANGLIA with the help of CEREBRAL CORTEX & CORTICO-SPINAL SYSTEM control complex & skilled movements: WRITING, STITCHING, PLAYING BASKET BALL, HAMMERING THE NAIL & CUTTING DESIGN WITH SCISSORS. In damage to basal ganglia disturbed writing appears that person is learning to write.

2) COGNITIVE CONTROL OF MOTOR ACTIVITY:

Basal ganglia + sensory input & information stored in the brain COGNITIVE CONTROL of motor activity. Cognitive control = ? It is pattern & sequence of events to achieve a specific aim or goal, e.g., person walking on a road sudden sight of a wild animal sequence of movements to save life (controlled by Basal ganglia).

3) CONTROL OF EXTENT & TIMING OF MOVEMENTS:

Basal ganglia helps to decide, that for HOW LONG the movements will occur & HOW RAPID the movement will be (EXTENT OF MOVEMENT). e.g. while writing: RANGE: x OR X SPEED: rapid: or slow:
Basal ganglia function in close cooperation with posterior parietal cortex.

OTHER FUNCTIONS OF BASAL GANGLIA

Control of muscle tone Control of automatic associated movements e.g. movements of arms while running

LESIONS OF BASAL GANGLIA: 1) ATHETOSIS:

Continuous, slow writhing movements affecting hand, arm, face or may be neck. It is due to damage to GLOBUS PALLIDUS.

2) CHOREA:
Rapid dancing movement affecting hand, arm or some other part of body. Due to damage in CAUDATE & PUTAMAN. 2 types of Chorea:
1) HUNTINGTONS CHOREA 2) SYDENHAM / RHEUMATIC CHOREA (complication of rheumatic fever).

HUNTINGTONS CHOREA:
Hereditary disorder. Features manifest in 3rd or 4th decade of life. There is degeneration of GABA secreting neurons in CAUDATE & PUTAMAN. As GABA is inhibitory, due to loss outburst of activity in GLOBUS PALLIDUS & SUBSTANTIA NIGRA dancing movements. Here (chorea + dementia): Not due to loss of GABA but due to damage to cholinergic neurons in cerebral cortex.

3) HEMIBALISMUS:
involuntary, violent movement affecting 1 side of body / 1 limb. Here is damage to SUBTHALAMIC NUCLEUS. ***

4) PARKINSONS DISEASE (PARALYSIS AGITANS)

1ST discovered by JAMES PARKINSON. CAUSE: degeneration of DOPAMINE secreting neurons in SUBSTANTIA NIGRA DOPAMINE deficiency in CAUDATE & PUTAMAN ( by 50% or even less) No release of DOPAMINE from NIGRO STRIATE fibers.

 NORMALLY: ACETYLCHOLINE = DOPAMINE or EXCITATORY INFLUENCE = INHIBITORY INFLUENCE (on caudate & putaman). In this disease, due to dopamine deficiency this balance is disturbed excitation of caudate & putaman features of Parkinsonism.

CAUSES OF PARKINSONISM:
1) IDIOPATHIC: (Cause ?) In old age dopamine secretion & dopamine receptors decrease. 2) TRAUMA: TO BASAL GANGLIA: Mohammad Ali (boxer)

 3) COMPLICATION OF TREATMENT WITH PHENOTHIAZINE DERIVATIVES: Increased dose over long duration.

4) COMPLICATION OF INFLUENZA: During 1st world war, epidemic of influenza complication Parkinsonism (in many complicated cases).

FEATURES OF PARKINSONISM:
*IMBALANCE BETWEEN EXCITATORY & INHIBITORY INFLUENCE OF ACETYLCHOLINE & DOPAMINE (respectively) DUE TO LOSS OF DOPAMINE.

1) AKINESIA OR BRADYKINESIA:
Inability to initiate movement. or patient is very slow to initiate movement.

2) RIGIDITY: LEAD PIPE RIGIDITY:

When a limb is passively flexed feeling of bending of a lead pipe continuous resistance. Rigidity due to increased motor neuron discharge to both AGONISTS & ANTAGONISTS (Tug of war between them).
LEAD PIPE

3) TREMORS: (PILL ROLLING TREMORS)

REGULAR, RHYTHMIC, ALTERNATE CONTRACTION of AGONISTS & ANTAGONISTS. Tremors involve: fingers & hand & may involve tongue & lips. STATIC TREMORS (at rest) Absent in sleep. Worst in emotional state & when patient is conscious that someone is watching him.

 Due to rigidity back is flexed, arms are flexed & adducted & knees are bent. In severe cases marked rigidity patient can be moved like a statue !

4) GAIT:
Short steps. Unable to stop the movement. Person chases his own shadow.

5) FACIAL EXPRESSION:
MASK LIKE FACE. Loss of facial expression.

6) DECREASED ASSOCIATIVE MOVEMENTS:

DECREASED swinging of arms while walking.

7) SUPERFICIAL ABDOMINAL REFLEX:

Present.

8) TENDON JERKS:
Difficult to be elicited due to rigidity.

9) BABINSKI SIGN:
Not present.

10) *MUSCLE PARALYSIS & *SENSORY LOSS:

ABSENT (like in cerebellar disease).

BASIC PRINCIPLES OF TREATMENT:

There is dopamine deficiency, so we give LEVO-DOPA (it can cross blood brain barrier & in brain: levo-dopa dopamine Anti cholinergic drugs inhibit over activity of Acetylcholine. Sometimes drugs fail. Treatment is then electro-coagulation of thalamic nuclei (VLTN & VATN are important).

WILSONS DISEASE OR HEPATO-LENTICULAR DEGENERATION: (another lesion of basal ganglia)

Due to HEREDITORY AUTOSOMAL RECESSIVE DISORDER of COPPER METABOLISM. In these patients, plasma Ceruloplasmin level is low copper deposition in body tissues: (liver & lentiform nucleus) hepatic cirrnosis & effects due to damage to lentiform nucleus (PUTAMAN + GLOBUS PALLIDUS).