Basal Ganglia For Residents
Basal Ganglia For Residents
Basal Ganglia For Residents
•ANATOMY
•CONNECTION
•PHYSIOLOGY
•MOVEMENT DISORDERS
•PARTS:
CAUDATE
NUCLEUS,
PUTAMEN
GLOBUS PALLIDUS
•ROLE IN MOTIVATION
DOPAMINE PATHWAY FROM SUBSTANTIA NIAGRA TO
CAUDATE NUCLEUS AND PUTAMEN
Wilson's disease
A NEURODEGENERATIVE DISORDER
CHARACTERISED BY TRIAD:
•RESTING TREMOR
•RIGIDITY
•AKINESIA / BRADYKINESIA
•ETIOLOGY
• IDIOPATHIC
• OXIDATIVE STRESS
• MITOCHONDRIAL DYSFUNCTION
• EXCITOTOXICITY
• CALCIUM INDUCED INJURY
• TOXINS LIKE MPTP
• VIRAL PANDEMICS
• DRUG INDUCED
GENETIC ASSOCIATIONS
• PARK 1 TO PARK 11
• ALPHA SYNUCLEIN,
• PARKIN
• LRRK2
MALE> FEMALE
MEAN AGE 55 YRS
RESTING TREMOR FREQUENCY 4 – 6 HZ
RIGIDITY
BRADYKINESIA
FESTINEAN SHORT SHUFFLING GAIT
EXCESS SALIVATION
SEBORRHEA
CONSTIPATION
FATIGUE
DEPRESSION
ANXIETY
APATHY
PSYCHOSIS
BEHAVIORAL DISTURBANCES
COGNITIVE IMPAIRMENT AND DEMENTIA
MOTOR SYMPTOMS
•DOPAMINE PRECURSOR: LEVODOPA
•PERIPHERAL DECARBOXYLASE INHIBITOR: CARBIDOPA,
BENSERAZIDE
•DOPAMINERGIC AGONIST:BROMOCRIPTINE, ROPINIROLE,
PRAMIPEXOLE
•MAO B INHIBITOR: SELEGILINE
•COMT (-):ENTACAPONE, TOLCAPONE
•DOPAMINE FACILITATOR:AMANTADINE
SURGICAL MODALITIES
•HIGH FREQUENCY DEEP BRAIN
STIMULATION
•PALLIDOTOMY
•THALAMOTOMY
•PROGRESSIVE SUPRANUCLEAR PALSY
•CORTICOBASAL DEGENERATION:
•ENCEPHALITIS LETHARGICA
AD, NEURODEGENERATIVE DISORDER
MIDLIFE ONSET
CAG REPEAT EXPANSION MUTATION IN
HUTINGTIN GENE ON CH 4
LOSS OF INTRASTRIATAL GABA ERGIC AND
CHOLINERGIC NEURONS
MOTOR: CHOREA, ATHETOSIS INVOLVES LIMBS
AND TRUNK BUT ALSO RESPIRATORY,
LARYNGEAL, PHARYNGEAL AND ORAL
MUSCULATURE
HEPATOLENTICULAR DEGENERATION
AR
MUTATION IN GENE ATP7B
ABNORMAL ACCUMULATION OF Cu IN
LIVER,BRAIN AND OTHER TISSUES
NORMAL: IN WILSONS DISEASE:
40% TO 50% OFCu
ABSORBED IN STOMACH AND DEFECTIVE BILIARY EXCRETION
DUODENUM
TRANSPORTED TO LIVER, Cu ACCUMULATION IN LIVER
LOOSELY BOUND TO ALBUMIN
TOXIC LIVER INJURY
FREE Cu DISSOCIATES AND
TRANSFERRRED TO Cu SPILLS IN CIRCULATION
HEPATOCYTES BOUND WITH @2
GLOBULIN FORMS Eg: K F RINGS IN EYES
CERRULOPLASMIN, WHICH IS
ENDOCYTOSED IN LIVER AND
DEGRADED IN LYSOSOMES, TO
EXCRETE IN BILE
ONSET IN CHILDHOOD OR ADOLESCENCE
JAUNDICE OR HEPATOSPENOMEGALY
RIGIDITY OR TREMOR
DYSTONIC POSTURES/ SPASTIC RIGIDITY
ATHETOID WRITHING MOVEMENTS
CONVERSION DISORDER LIKE MENIFESTATIONS
FLAPPING TREMOR OF WRIST- WING BEATING
NO FACIAL EXPRESSION
EPILEPTIC SEIZURES , PSYCHIATRIC SYMPTOMS
CT AND MRI SHOW VENTRICULAR DILATATION,CORTICAL
ATROPHY, ENLARGEMENT OF CISTERNS AROUND BRAIN
STEM
HYPODENSE AREA OF BASAL GANGLIA
S CU LEVELS, CERRULOPLASMIN LEVELS
ABNORMAL KFT, LIVER BIOPSY, GENETIC TESTING
D- PENICILLAMINE
TRIENTINE
TETRATHIOMOLYBDATE
ZN SALTS
CLASSIFICATION DSM IV
TREATMENT:SWITCHING TO ATYPICAL
ANTIPSYCHOTICS, CLOZAPINE, BZD, BETA
BLOCKERS, SELEGILINE, 5 HT 2 ANTAGONIST
RITANSERIN, VIT B6, MIRTAZAPINE 15 MG
AT LEAST OF 2 MONTHS AFTER EXPOSURE TO DOPAMINE
BLOCKING AGENT OR 1 MONTH IF PATIENT IS > 60 YRS,
PATIENT OFF ORAL MED FOR 8 WEEKS AND 4 WEEKS FOR
LONG ACTING INJECTABLES, PRESENT FOR 4 WEEKS
SPASMODIC TORTICOLLIS
BLEPHAROSPASM
OROMANDIBULAR DYSTONIA
LARYNGEAL DYSTONIA
TICS ARE STEREOTYPED, RAPID, RECURRING
MOTOR MOVEMENTS OR VOCALIZATIONS THAT
ARE NON RHYTHMIC,INVOLUNTARY OR
SEMIVOLUNTARY AND SUDDEN IN ONSET
MOTOR TICS
◦ SIMPLE
◦ COMPLEX
VOCAL TICS
◦ SIMPLE
◦ COMPLEX
INCREASED INCIDENCE OF ADHD AND OCD
•OBSENE WORDS(COPROLALIA)
ATTENTION DEFICIT
HYPERACTIVITY
DISORDER
Striatum
Frontal Cortex
+ D1 D2
+ +
Direct - - Indirect
Pathway Pathway
- GPI &
GPE
Thalamus SNr
+ -
Subthalamic
Nucleus
(Alexander et al,1986)
Imaging studies
Structural: increases or decreases in size of the
orbitofrontal cortex, striatum, or thalamus
Increased activity in caudate, orbitofrontal cortex,
cingulate cortex
Dysfunction of cortical/basal ganglia loop
◦ Huntington’s, Tourette’s, maybe Parkinson’s, Sydenham’s
chorea also exhibit OCD
Baxter et al., 1998
SSRIs
Adjunctive antipsychotics (dopamine antagonists)
Behavior therapy
Psychosurgery
CINGULLOTOMY AND DBS
Athymhormic syndrome, or psychic akinesia,
Is a rare neurological syndrome characterized by extreme passivity,
apathy, blunted affect, and a profound generalized loss of self-
motivation and conscious thought. For example, a patient with this
syndrome might sustain severe burns on contact with a hot stove,
due to lacking the will to move away despite experiencing severe
pain. The existence of such symptoms in patients after damage to
certain structures in the brain has been used to support a physical
model of motivation in human beings, wherein the limbic loop of
the basal ganglia is the initiator of directed action and thought.