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Basal

Nuclei
Connections
And

Clinical Correlations
Clinical Case 1
David is a 66-year-old man, previously healthy, who was
brought to a neurologist by his daughter because of
increasing difficulty in walking. His daughter noticed that
for the past year, he had difficulty getting out of a chair and
took a lot of time to begin to walk. When he did walk, he
walked with a slow, shuffling gait. In addition, she had
noticed some changes in his face, and that he had been
drooling excessively. His signature on checks became
progressively smaller from the beginning of his name to the
end, and he had developed a new tremor. She brought him
in to make sure this wasnt just aging. The neurologist
examined David and noticed immediately that David facial
expression was masklike, with few eye blinks. When asked
to write a sentence, the letters became progressively
smaller toward the end of the sentence
Case Cont
His speech was soft and monotonous, and he had a slow,
resting pill-rolling tremor in both of his hands. He had
very little spontaneous movement, and his arms, legs,
and trunk were stiff. When the neurologist tried to flex
his arm, he felt many catches, similar to a cogwheel.
There was no weakness, sensory problems, or
abnormalities in his reflexes. When asked to walk, David
took many tries to rise from his chair. When he finally
stood up, his posture was stooped and flexed. His gait
was slow, his feet shuffled when he walked, and his arms
didnt swing with his steps. The neurologist told David
daughter that she was correct that this wasnt aging and
explained to her all of the details about a new medication
that David needed to take.
Clinical Case 2
A 55 year-old Tennis player noticed the abrupt
onset of involuntary movements on his right side.
His right arm would make large, violent, flailing
or ballistic movements that interfered with his
game. The most likely site of the damage was
the:
Left substantia nigra
Left globus pallidus
Right globus pallidus
Right subthalamic nucleus
Left subthalamic nucleus
Clinical Case 3
Hyperkinetic syndromes such as ballism,
Athetosis and chorea are related to a
problem of thalamic disinhibition related
to which of the following
neurotransmitters.
Dopamine
Gamma aminobutyric acid
Acetylcholine
Dopamine and gamma aminobutyric acid
Substance pand enkephalin
Clinical Case 4
77-year-old man is finding that he has more difficulty
moving about for the past year. He is annoyed by a
tremor in his hands, but the tremor goes away when
he performs routine tasks using his hands. His friends
remark that he seems more sullen and doesn't smile
at them, but only stares with a fixed expression on his
face. He has not suffered any loss of mental ability.
Which of the following diseases is he most likely to
have?
Alzheimer disease
Parkinson disease
Cerebellar Disease
None Of above
asal Ganglia Gross Features
It is a nuclear mass located
within the depths of each
cerebral hemisphere.
Corpus Striatum
Claustrum
Amygdaloid body
The corpus striatum is the
largest component of the basal
ganglia.
Divided into three components.
It includes the caudate nucleus,
the putamen and the globus
pallidus.
The caudate nucleus (CD) is a
large C-shaped grey matter
located medial to the internal
capsule and related throughout
its length to the surface of the
lateral ventricle.
Basal Ganglia Gross Features
It has an head that
bulges into the lateral
wall of the frontal horn
of the lateral ventricle.
The head tapers to form
the body located along
the lateral wall of the
ventricle.
The body in turn
becomes further
(Continued) attenuated
to form the tail which
courses along the roof of
the temporal horn of the
ventricle and terminates
at the amygdaloid Amygdaloid Nu
nucleus.
Basal Ganglia Gross Features

The PU and CD together comprise


the neostriatum (neo = new) which
is sometimes simply abbreviated
striatum.
Histologically, the structure of the CD
and PU are similar.
They contain the inhibitory
neurotransmitter gamma-amino
butyric acid (GABA).
Basal Ganglia Gross Features
The paleostriatum (paleo =
ancient ) or "pallidum" is the
forerunner of the mammalian corpus
striatum and consists of the globus
pallidus (GP) only.

The GP is situated medial to the


putamen and is separated from it by
a thin lamina of myelinated fibers
called the external medullary
lamina.
A similar lamina (medial medullary
lamina) divides the GP into a lateral
or external division (GPe) and a
medial or internal (GPi) division,
each of which has its own peculiar
set of connections.
The GP is traversed by numerous
myelinated fibers which give it its
characteristic pale appearance in
stained sections and from which its
name derives.
Basal Nuclei Connections
Dopamine and Cholinergic Effect
Basal Ganglia Connections

Primary Supplementary
STRIATUM
Motor Area Motor Area
(Putamen)
(M I) (SMA)

pyramidal
tract
ansa
lenticularis
THALAMUS PALLIDUM
(VLo, VApc, CM) (GPi)
lenticular
LMN fasciculus
Basal Ganglia
Connections

Primary Prefrontal STRIATUM


Motor Area Association (Caudate
(M I) Cortex Nucleus)

pyramidal
tract

THALAMUS SNr
(Substantia Nigra,
(VLm, VAmc, MD)
pars reticulata)
LMN
Basal Ganglia (Microcircuitary)
Connections

glutamate
Motor Striatum
Cortex +

+
glutamate

glutamate
-- GABA
+

VA-VL -
GP, SNr
complex GABA
Organization of the motor system

Level
Cerebral cortex
Cerebral cortex motor
motor areas
areas

1. The elements 1
Basal
Basal Thalamus
Thalamus
Level Gangli
Gangli
aa
2. Three levels 2
Cerebellu
Cerebellu Brain Stem
Brain Stem
mm
3. Feedback loops
Muscle
Muscle
between and Spinal Cord
Spinal Cord contraction
contraction
within levels. and
and
Movement
Movement
Level
3
Sensory
Sensory
receptors
receptors
Disorders Of Basal Ganglia
Hyperkinetic
Chorea, Athetosis & Ballism
Hypokinetic
Akinesia &Bradykinesia
SYDENHAMS
SYDENHAMS CHOREA
CHOREA

Clinical Feature
- Complication of
Rheumatic Fever
- Fine, disorganized , and
random movements of
extremities, face and
tongue
- Accompanied by
Muscular Hypotonia
- Typical exaggeration of
associated movements
during voluntary activity
- Usually recovers
spontaneously
Principal Pathologic Lesion: Corpus Striatumin 1 to 4 months
HUNTINGTONS CHOREA

Clinical Feature
- Predominantly autosomal dominantly
inherited chronic fatal disease
(Gene: chromosome 4)
- Insidious onset: Usually 30-50
- Choreic movements in onset
- Frequently associated with
emotional disturbances
- Ultimately, grotesque gait and sever
dysarthria, progressive dementia
ensues.
Principal Pathologic Lesion:
Corpus Striatum (esp. head of
caudate nucleus) & Cerebral
Cortex
HEMIBALLISM
HEMIBALLISM

Clinical Feature

- Usually results from CVA


(Cerebrovascular Accident)
involving subthalamic nucleus
- sudden onset
- Violent, writhing, involuntary
movements of wide excursion
confined to one half of the body
- The movements are continuous
and often exhausting but cease
during sleep
- Sometimes fatal due to exhaustion
- Could be controlled by
phenothiazines and stereotaxic
Lesion: Subthalamic Nucleussurgery
Parkinsons PD
Disease
Disease of mesostriatal
dopaminergic system

Muhammad Ali in Alanta Olympic normal


Parkinsons Disease - Paralysis
Agitans

Substantia
Nigra,
Pars Compacta
(SNc)
DOPAminergic
Neuron
Clinical Feature (1)

Slowness of Movement
- Difficulty in Initiation and Cessation
of Movement
Parkinsons Disease
Paralysis Agitans

Clinical Feature (2)


Resting Tremor
Parkinsonian Posture
Rigidity-Cogwheel Rigidity