•INTRODUCTION

•ANATOMY

•CONNECTION

•PHYSIOLOGY

•MOVEMENT DISORDERS

•NEURO PSYCHIATRIC IMPLICATIONS

A STRUCTURE IN THE BRAIN ( COLLECTION OF
NUCLEI ) WHICH IS CONCERNED WITH THE
PLANNING AND ORGANIZATION OF VOLUNTARY
MOVEMENT THROUGH FEEDBACK CIRCUITS TO
THE MOTOR AND PREMOTOR CORTEX.
•DERIVED FROM THE TELENCEPHALON

•PARTS:

CAUDATE NUCLEUS CORPUS STRIATUM

PUTAMEN
GLOBUS PALLIDUS LENTIFORM NUCLEUS
SUB THALAMIC NUCLEI
SUBSTANTIA NIAGRA
Head of
caudate
Internal
capsule
Lentiform
nucleus Putame
n
thalamus
Globus
pallidus
 EMBRYONAL ORIGIN OF ALL ADULT GABA
INTERNEURONS

 REACHES NEOCORTEX THROUGH TANGENTIAL

MIGRATION
•FUNCTIONING WITH THE CORTICOSPINAL
SYSTEM TO CONTROL COMPLEX PATTERN
OF MOTOR ACTIVITY

•EXECUTING LEARNED PATTERNS OF

MOVEMENT

•COGNITIVE CONTROL OF MOTOR

ACTIVITY

•CHANGE THE TIMING AND TO SCALE THE

INTENSITY OF MOVEMENT
 MOTOR SENSORY CEREBRAL
CEREBRAL CORTEX, THALAMUS,
CORTEX BRAIN STEM

CAUDATE
NUCLEUS,
PUTAMEN

GLOBUS PALLIDUS

CRANIAL NERVE MOTOR

NUCLEI
ANTERIOR HORN CELLS

FINAL COMMON PATHWAY TO MUSCLES

•EYE MOVEMENT

•ROLE IN MOTIVATION
 DOPAMINE PATHWAY FROM SUBSTANTIA NIAGRA TO
CAUDATE NUCLEUS AND PUTAMEN

 GABA PATHWAY FROM CAUDATE NUCLEUS AND

PUTAMEN TO GLOBUS PALLIDUS AND SUBSTANTIA
NIAGRA

 ACETYLCHOLINE PATHWAY FROM CORTEX TO

CAUDATE NUCLEUS AND PUTAMEN

 OTHERS LIKE NOREPINEPHRINE, SEROTONIN,

ENKEPHALIN

 MULTIPLE GLUTAMATE PATHWAYS

 Movement disorders are a group of diseases and
syndromes affecting the ability to produce and control
movement
 Attention-deficit hyperactivity disorder (ADHD)
 Athymhormic syndrome (PAP syndrome)
 Athetosis
 Cerebral palsy: basal ganglia damage during second
and third trimester of pregnancy
 Chorea
 Dystonia
 Fahr's disease
 Foreign accent syndrome (FAS)
 Huntington's disease
 Obsessive-compulsive disorder
 Other anxiety disorders
 Parkinson's disease
 PANDAS
 Sydenham's chorea
 Tourette's disorder
 Tardive dyskinesia, caused by chronic antipsychotic
treatment

 Wilson's disease
A NEURODEGENERATIVE DISORDER
CHARACTERISED BY TRIAD:

•RESTING TREMOR

•RIGIDITY

•AKINESIA / BRADYKINESIA
•ETIOLOGY

LOSS OF DOPAMINERGIG NEURONS IN SN

• 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:

•FRONTO TEMPORAL DEMENTIA

•MULTIPLE SYSTEM ATROPHY

•DEMENTIA WITH LEWY BODIES

•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

 COGNITIVE: DIFFICULTY IN SWITCHING FROM

ONE TASK TO OTHER, TRACKING MULTIPLE TASK
SIMULTANEOUSLY, POOR WORK OR SCHOOL
PERFORMANCE, MMSE SCORE DECREASE BY 1 TO
2 PTS EACH YR

 PSYCHIATRIC: DEPRESSION, MANIA, DELUSION,

OCD LIKE, ADJUSTMENT PROBLEMS,
PERSONALITY CHANGES
•ATROPHY OF CAUDATE AND
•PUTAMEN

•PCR, GENETIC COUNSELLING

 CHOREA: LOW DOSES OF ANTIPSYCHOTICS
LIKE HALOPERIDOL, BZD,DOPAMINE
DEPLETING AGENTS LIKE TETRABENZINES,
RESERPINE, AMANTADINE

 DEPRESSION: SSRI ,TCA , MAOI, ECT

 MOOD ELEVATORS LIKE LI AND

DIVALPROATE
 ANTIOXIDANTS COENZYME Q10, DBS
 MISS XYZ ,17 YR OLD GIRL DEVELOPED EMOTIONAL
LABILITY, DIFFICULTY WITH HANDWRITING AND DETERIORATION IN
SCHOOL PERFORMANCE. TAKEN TO PSYCHIATRIST FOR ADOLESCENT
ADJUSTMENT PROBLEMS. CHLORPROMAZINE PRESCRIBED INCREASED
HER TREMORS. LFT WAS ABNORMAL THOUGHT TO BE DUE TO THE
MEDICINE. LATER DIAGNOSIS OF SCHIZOAFFECTIVE DISORDER WAS
MADE. SHE FINALLY SHOWED EPS WITH EXCESS DROOLING OF SALIVA,
MASK LIKE FACIES, DYSTONIA. KF RINGS WERE SEEN IN SLIT LAMP AND
CU LEVELS WERE LOW.

 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

•NEUROLEPTIC INDUCED PARKINSONISM

•NMS
•NEUROLEPTIC INDUCED ACUTE DYSTONIA
•NEUROLEPTIC INDUCED ACUTE AKATHISIA
•NEUROLEPTIC INDUCED TARDIVE DYSKINESIA
•MEDICATION INDUCED POSTURAL TREMOR
•MEDICATION INDUCED MOVEMENT DISORDER NOS
 SAME AS IDIOPATHIC PARKINSONISM

 DUE TO DEPLETION OF NIGROSTRIATAL DOPAMINE ACTIVITY, DUE TO

DEPLETION OF DOPAMINE IN PRESYNAPTIC STORES(RESERPINE),
DOPAMINE BLOCKING AGENTS(ANTI PSYCHOTICS) ATYPICAL
CALCIUM CHANNEL BLOCKING AGENT, CINNARAZINE

 TREATMENT: SUBSIDES WITH DOSE REDUCTION OR STOPPAGE OF THE

DRUG.

 SWITCHING FROM HIGH POTENCY TOLOWER POTENCY D2 BLOCKING

AGENT OR QUITIAPINE, ARIPRIPAZOLE

 ALSO ANTICHOLINERGIC AGENTS THP, BENZTROPINE

 AMANTIDINE
 DHEA
 MUSCLE RIGIDITY, TEMPERATURE>38C IN PATIENTS ON
ANTIPSYCHOTIC MEDICATION
 DIAPHORESIS, TACHYCARDIA, INCREASED BP, DYSPHAGIA,
INCONTINENCE, TREMOR,CONFUSION TO COMA, MUTISM,
LEUCOCYTOSIS,LAB EVIDENCE OF MUSCLE INJURY, LIVER
ENZYME ELEVATION
 PATHOLOGY:HYPODOPAMINERGIC STATE, ABRUPT
DISCONTINUATION OF ANTI PSYCHOTIC MEDICATION
 BLOCKADE OF DOPAMINE RECEPTORS IN CORPUS
STRIATUM CAUSING MUSCLE RIGIDITY AND HEAT
GENERATION
 ACUTE DYSTONIA 10 TO 15 % MEN RECEIVING
ANTIPSYCHOTICS

 ABNORMAL POSITIONING OF HEAD AND NECK, SPASM OF JAW

MUSCLES, IMPAIRED SWALLOWING, SPEAKING, BREATHING,
THICKENED OR SLURRED SPEECH, TONGUE PROTRUSION,
DEVIATED EYES, ABNORMAL POSITIONING OF LIMBS

 WITHIN MINUTES OF RECEIVING INJECTABLE HIGH POTENCY

ANTI PSYCHOTICS OR WITHIN DAYS OF STARTING ORAL ANTI
PSYCHOTICS OR REDUCING MEDICINE USED TO TREAT EPS

 ACUTE SATURATION OF D2 RECEPTORS , SIGMA 1 AND SIGMA 2

 TREATMENT:IV/ IM BENZTROPINE OR DIPHENHYDRAMINE

 DEVELOPS WITHIN 1ST FOUR WEEKS OF STARTING
OR INCREASING THE DOSE OF MEDICATION OR
DECREASING MEDICINE USED TO TREAT EPS, ALSO
WITH VARIOUS ANTIDEPRESSANTS(SSRI)

 FIDGETING, SWINGING OF LEGS,ROCKING FROM

FOOT TO FOOT WHILE STANDING, PACING, LIFTING
THE FEET AS IF MARCHING IN PLACE

 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

 INVOLUNTARY CHOREIFORM, ATHETOID OR RYTHMIC

MOVEMENTS OF TONGUE, JAW OR EXTREMITIES

 TREATMENT:DISCONTINUATION OF DRUGS,VIT B6,

ONDANSETRON, DONEPEZIL,CYPROHEPTADINE, VIT E,
CLONAZEPAM, GABAPENTIN, LEVITIRACETAM, DBS
 THEY ARE CERTAIN GROUP OF DISORDERS
IN WHICH SUSTAINED MUSCLE SPASMS
INVADE MUSCLE GROUPS, CAUSING
WRITHING OR TWISTING MOVEMENTS OR
DISTORTING BODY AND LIMBS INTO
CHARACTERISTICS POSTURES
 DYSTONIA MUSCULORUM DEFORMANS

 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

 ONSET USUALLY IN LATE CHILDHOOD

 ACTIVATION OF THE CORTICO-STRIATAL-
THALAMIC LOOP DURING TICS (STERN, 2005)
REDUCTION IN VOLUME AND OTHER
ABNORMALITIES IN BASAL GANGLIA
 Increase in dopaminergic activation
 IMBALANCE: TOO MUCH ACTIVATION OF
DIRECT COMPARED TO INDIRECT

DA antagonists (particularly D2 antagonists)

reduce tics
Amphetamine can trigger tics
This is problematic for individuals with both
ADHD and Tourette’s syndrome
 GILLES DE LA
TOURETTE SYNDROME

•PRESENCE OF MULTIPLE MOTOR AND ONE OR

MORE VOCAL TICS AT ILLNESS

•MANY TIMES A DAY

•FOR MORE THAN 1 YR

•OBSENE WORDS(COPROLALIA)
 ATTENTION DEFICIT
HYPERACTIVITY
DISORDER

•MOST COMMONLY DIAGNOSED

•IT IS A BEHAVIORAL AND NEUROCOGNITIVE

CONDITION CHARACTERISED BY
DEVELOPMENTALLY INAPPROPIATE AND
IMPAIRING LEVELS OF GROSS MOTOR
OVERACTIVITY, INATTENTION AND IMPULSIVITY
 ATTENTIONAL DIFFICULTIES
 IMPULSIVITY
 HYPERACTIVITY
 BEHAVIORAL
 COGNITIVE
 EMOTIONAL
 SOCIAL
 HYPERACTIVITY CIRCUIT: CSTC LOOP AND
PREFRONTAL CORTEX

 IMPULSIVITY CIRCUIT: CSTC LOOP AND

ORBITAL FRONTAL CORTEX
 DEXMETHYLAMPHETAMINE
 DEXTROAMPHETAMINE
 METHYLPHENIDATE
 ATOMOXETINE
 Obsessions: persistent thoughts
 Compulsions: persistent behaviors

 Individual is aware that the behavior or obsession is

unreasonable or excessive
OCD Spectrum Disorders
❖Behavioral Regulation Through Frontal-Subcortical Circuits

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.

 The syndrome is believed to be due to damage to areas of the basal

ganglia or frontal cortex, specifically the striatum and globus
pallidus, responsible for motivation and executive functions.It may
occur without any preexisting psychiatric condition.
 PANDAS
 is an abbreviation for Pediatric Autoimmune
Neuropsychiatric Disorders Associated with
Streptococcal infections. This diagnosis is used to describe
a set of children who have a rapid onset of obsessive-
compulsive disorder (OCD) and/or tic disorders such as
Tourette syndrome (TS), following group A beta-hemolytic
streptococcal (GABHS) infections such as "strep throat"
and scarlet fever.[1] The proposed link between infection
and these disorders is an autoimmune reaction, where
antibodies produced by the infection interfere with neuronal
cells.[2]
 FAHRS DISEASE: IDIOPATHIC BASAL GANGLIA
CALCIFICATION
 PSYCHOGENIC MOVEMENT DISORDER