BASAL GANGLIA & PARKINSONISM Flashcards
Basal Ganglia is a MISNOMER
Basal Nuclei
Structural & Functional Components of BASAL GANGLIA
Parts of Substantia Nigra
- Pars Reticulata: Homologous to GP internus
- Pars Compacta
Wilson’s Pencil
Thin Myelinated (OR) Unmyelinated axons which are afferents & afferents passing through Straitum
⭐ Direct Pathway in Basal Ganglia does
⭐ Indirect Pathway in Basal Ganglia does
🧠⚡INdirect ⚡
⭐ Direct Pathway in Basal Ganglia does
🎯 Stimulates MOVEMENT
⭐ Indirect Pathway in Basal Ganglia does
🎯 INhibits MOVEMENT
Functions of BASAL GANGLIA
⭐ Inhibitory at REST ⭐
✨ TIMING & SCALING the intensity of movement
✨ Planning & Programming movement
✨ Cognitive component of Motor Activity
✨ Tone adjustment
✨ Movement Enhancer
2 EXTRA THINGS PRESENT in INDIRECT PATHWAY than DIRECT PATHWAY
- Globus pallidus Externus
- Subthalamus
Glutamate is EXCITATORY neurotransmitter, Released in BASAL GANGLIA by
- Cerebrum
- Subthalamus
- Thalamus
GABA is INHIBITORY neurotransmitter, Released in BASAL GANGLIA by
Corpus Straitum
✨ Caudate nucleus
✨ Putamen
✨ Globus pallidus
⭐ DOPAMINE is Released in BASAL GANGLIA
⭐ ACETYLCHOLINE is Released in BASAL GANGLIA
⭐ DOPAMINE is Released in BASAL GANGLIA
🎯 Substantia Nigra
⭐ ACETYLCHOLINE is Released in BASAL GANGLIA
🎯 Putamen
Direct pathway in BASAL GANGLIA
Indirect pathway in BASAL GANGLIA
Nigrostraital Pathway
Releases Dopamine
✨ D1 Receptor: Direct Pathway: Stimulate Movements
✨ D2 Receptor: Indirect Pathway: Inhibit Movements
Athetosis is DUE TO LESION in
Globus Pallidus
Athetosis
Slow
INVOLUNTARY
IRREGULAR
FORCEFUL
WRITHING movement
HANDS, ARM, NECK & FACE
Chorea is DUE TO LESION in
Caudate Nucleus
(OR)
Putamen
Chorea
Dancing like movement
Involuntary
Flicking movement
Hand, Face & Shouldera
Hemiballismus is DUE TO LESION in
Subthalamus
⬇️
Sudden Flailing movement of Entire limb
Parkinson’s disease is DUE TO LESION in
Substantia Nigra
Neurological Manifestation of WILSON DISEASE DUE TO
Copper Deposition in Lentiform nucleus
Only EXCITATORY NUCLEUS OF BASAL GANGLIA
Subthalamus
🚦DIFFERENTIAL DIAGNOSIS🚦 of HYPOKINETIC MOVEMENT
- Parkinson’s
- Non-Parkinson’s etiology
✨ Hypothyroidism
✨ Catatonia
⚡⚡ MOST COMMON NEURODEGENERATIVE DISEASE
⚡⚡ 2nd MOST COMMON NEURODEGENERATIVE DISEASE
⚡⚡ MOST COMMON SUBCORTICAL NEURODEGENERATIVE DISEASE
⚡⚡ MOST COMMON NEURODEGENERATIVE DISEASE
🎯 ALZHEIMER’S DISEASE
⚡⚡ 2nd MOST COMMON NEURODEGENERATIVE DISEASE
🎯 PARKINSON’S DISEASE
⚡⚡ MOST COMMON SUBCORTICAL NEURODEGENERATIVE DISEASE
🎯 PARKINSON’S DISEASE
Age of Parkinson’s presentation
≥ 65 yrs
If age of presentation of Parkinson’s DISEASE is ≤ 60yrs, known as
Familial Parkinson’s Disease
⚡⚡ MOST COMMON CAUSE of FAMILIAL PARKINSON’S
🧬 MODE OF INHERITENCE 💉
PARK8
⭐ AD
⭐ LRRK2 mutation
FAMILIAL Forms of PARKINSON’S
Function of Alpha Synuclein
Lipid Binding Protein ASSOCIATED with Synapses
Substantia Nigra is Pigmented DUE TO:
Presence of NEUROMELANIN Granules
⬇️
Synthesis of Dopamine
Lewy Body
Accumulation of Alpha Synuclein
Synuclein deposition occurs 1st in
Lower BRAINSTEM (MEDULLA)
⬇️
PONS
⬇️
MIDBRAIN
⬇️
SUBSTANTIA NIGRA
Prion like spread within Brain is ASSOCIATED with
PARKINSON’S DISEASE
✨ Alpha Synuclein aggregates can be released from 1 neuron & taken uup by another
Absent Swallow Tail Sign is seen in
MRI: PARKINSON’S DISEASE
MRI Signs of PARKINSON’S DISEASE
- Loss of Pigmentation in SUBSTANTIA NIGRA
- ABSENT SWALLOW TAIL SIGN
- B/L HYPERINTENSE GREY MATTER on upper Midbrain
Lewy Bodies can be seen in
- Other Pigmented nuclei in Brainstem
- Dorsal Nucleus of Vagus
- Nucleus BASALIS
- Other Cortical ASSOCIATION Areaa
Primary & Secondary PARKINSON’S Disease
🧠⚡2° causes: We Will Have DPT² ViaL⚡
- WILSON’s disease
- Whipple’s DISEASE
- Head Trauma: Dementia pugilistica
- Drug induced
- Post-encepahlitic
- Toxins: Manganese, MPTP, CO, Cyanide
- Tumour / Thyroid Function ⬇️ ⬇️
- Vascular
- Liver Failure
Symptoms of PARKINSON’S DISEASE
🧠⚡Be SMART⚡
ASYMMETRICAL
➕
1. Bending Forward (OR) Tilt
2. Shuffling Gait
3. Mask like facies
4. Akinesia / Bradykinesia
5. Rigidity
6. Tremors
Earliest Symptom of PARKINSON’S DISEASE
Tremor
⬇️
Akinesia
⬇️
Axial Skeleton: Gait & Posture
⬇️
Sleep, ANS, Sensory
⬇️
Cognitive Symptoms
Clinical Phenotypes of PARKINSON’S DISEASE
- Tremor Predominant
🎯 Younger patient (60-70yrs)
🎯 BETTER Prognosis
🎯 SLOWER DISEASE PROGRESSION - Akinetic Rigid Syndrome
🎯 Older patient > 70yrs
🎯 POOR Prognosis
🎯 FASTER DISEASE PROGRESSION
⚡⚡ MOST COMMON Clinical Phenotype of PARKINSON’S DISEASE
Tremor Predominant
⚡⚡ MOST COMMON PRESENTING SYMPTOM OF PARKINSON’S DISEASE
Tremors
Charcteristic Features of TREMORS in PARKINSON’S DISEASE
✨ Pill Rolling Tremors
- Resting Tremor
- UL Distal
- Frequency: 5-6 Hz
- Asymmetrical: U/L ➡️➡️ B/L
- Worsens with Emotional Stress
- Disappears on VOLUNTARY ACTIVITY & SLEEP
- Brought ON by concentration
- Most patient t have Combination of Action ➕ Rest Tremors
Types of TREMORS
- Resting Tremors
- Intentional tremors or Kinetic
- Postural
Frequency of different types of TREMORS
🧠⚡REPs increasing⚡
- Resting Tremors in PD: 4-6Hz
- Essential Tremors: 8-10Hz
- Physiological Tremors: 12Hz
Progression of Involvement of Limb in Tremors of PARKINSON’S DISEASE
I/L arm
⬇️
I/L Leg
⬇️
C/L Leg
⬇️
C/L Arm
Difference between PD Tremor & Essential Tremors
✨ Relieving Factors for PARKINSON’S DISEASE TREMOR
✨ Relieving Factors for ESSENTIAL TREMOR
✨ Relieving Factors for PARKINSON’S DISEASE TREMOR
🎯 L-DOPA
🎯 Dopamine agonists
🎯 anticholinergic
✨ Relieving Factors for ESSENTIAL TREMOR
🎯 ALCOHOL
🎯 PROPRANOLOL
🎯 PRIMIDONE
🎯 TOPIRAMATE
🎯 GABAPENTIN
🎯 CLONAZEPAM
Target Site for DEEP BRAIN STIMULATION in:
⭐ PARKINSON’S DISEASE
⭐ DYSTONIA
⭐ DEPRESSION
⭐ OCD
⭐ ESSENTIAL TREMORS
⭐ PARKINSON’S DISEASE
🎯 SUBTHALAMUS
🎯 GPi
⭐ DYSTONIA
🎯 GPi
⭐ DEPRESSION
🎯 ANTERIOR LIMB OF INTERNAL CAPSULE
🎯 CINGULUM
⭐ OCD
🎯 ANTERIOR LIMB OF INTERNAL CAPSULE
⭐ ESSENTIAL TREMORS
🎯 VENTRAL INTERMEDIATE NUCLEUS
Wheel Chair Sign seen in
PARKINSON’S DISEASE
✨ Rising from Wheel Chair: Difficult
GLABELLAR TAP SIGN seen in
PARKINSON’S DISEASE
🎯 Repetitive Tapping (at 2Hz) over Glabella produces Sustained BLINK RESPONSE
Myerson’s Sign
GLABELLAR TAP sigb
Characteristic features of BRADYKINESIA in PARKINSON’S DISEASE
- Facial Hypomimia (Expressionless facies)
- Automatic Movements ⬇️⬇️ (Arm Swinging)
- Small Hand writing
- Wheel Chair Sign
- GLABELLAR TAP sign
- Difficulty in tying Shoelaces, buttoning up & Difficulty in ROLLING OVER in Bed
- Hypophonic Voice (Slow & Soft) Flat monotonus & stuttering
⭐ Narrow Based Gait seen in
⭐ Broad Based Gait seen in
⭐ Narrow Based Gait seen in
🎯 PARKINSON’S DISEASE
⭐ Broad Based Gait seen in
🎯 PARKINSON Plus syndrome
💎 Cerebellar disease
Abnormal Pull Test seen in
PARKINSON’S DISEASE
⬇️
Pt falls backward
Froment’s sign seen in
⭐ Meaning
PARKINSON’S DISEASE
⭐ Activity induced ⬆️ in C/L Limb Rigidity
Rigidity vs Spasticity
Difference BETWEEN PARKINSON’S DISEASE DEMENTIA & LEWY BODY DEMENTIA
PARKINSON PLUS ➕ SYNDROME
🧠⚡PCM Do⚡
Group of disorder that presents with PARKINSONISM Features (BRADYKINESIA & RIGIDITY) but are different in underlying clinical manifestations & pathology
- Progressive Supranuclear Palsy
- Cortico-basal Degeneration
- Multi-system Atrophy
- Diffuse Lewy Body DEMENTIA
Steele Richardson Olszewski Syndrome
Progressive Supranuclear Palsy
Suspect PARKINSON plus syndrome if
- No response to Levodopa
- Absense of Rest Tremors
- Severe & Early ANS features
- Cortical abnormality: Apraxia
- Supranuclear gaze palsy
- Predominant Cerebellar (OR) Pyramidal findings
- Early ONSET REPEATED FALLS
- Symmetrical BRADYKINESIA
- EARLY DEMENTIA & HALLUCINATION
UK BRAIN BANK CRITERIA is used to diagnose
Parkinson’s disease
Synucleinopathies
🧠⚡P²REM = Love⚡
- Parkinson’s disease
- Pure Autonomic Failure
- REM Behavioural Disorders
- Multi system atrophy
- Lewy Body DEMENTIA
In PARKINSON’S & LEWY BODY DEMENTIA, Synuclein aggregates in
In MULTI SYSTEM ATROPHY, Synuclein aggregates in
In PARKINSON’S & LEWY BODY DEMENTIA, Synuclein aggregates in
🎯 Neurons
In MULTI SYSTEM ATROPHY, Synuclein aggregates in
🎯 Glial Cells
⭐ Hyper-phosphorylated Tau Proteins are aggregated in
⭐ Hypo-phosphorylated Tau Proteins are aggregated in
⭐ Hyper-phosphorylated Tau Proteins are aggregated in
🎯 ALZHEIMER’S DISEASE
⭐ Hypo-phosphorylated Tau Proteins are aggregated in
🎯 PICK’S DISEASE
SUBCORTICAL TAUPATHIES
- Progressive Supranuclear Palsy
- Corticobulbr Degeneration
TAUPATHIES
🧠⚡PNS C And D⚡
- Progressive Supranuclear Palsy
- Pick’s disease
- Post-encephalitic PARKINSON’S DISEASE
- Pantothenate Kinase ASSOCIATED Degeneration
- Niemann Pick DISEASE type C
- SSPE
- CBD
- Chronic Traumatic Encephalopathy (Dementia Pugilistica)
- ALZHEIMER’S disease
- Down’s Syndrome
🌸 TYPES of MSA Multi System Atrophy
🧠⚡ PAC⚡
- MSA-P: PARKINSON like presentation
- MSA-A: Autonomic Features Predominant
- MSA-C: Cerebellar Features Predominant
Shy Dagger Syndrome
MSA-A
SHY DAGGER SYNDROME
🧠⚡SHY? Parked Cerebellum⚡
- Systemic Atrophy
- Hypotension
- Y = Urinary Incontinence / Urgency ➕ other autonomic features
- Symptoms of PARKINSON’S DISEASE
- Cerebellum problems: Coordination & Speech
LEWY Body DEMENTIA
🧠⚡
Lewyhas halLEWYcinations.
Lewy is slowy.
Lewy is sleepy.
Lewy is slippy.
lEwy also has an E
⚡
🧠⚡ Lewy is slow-y, sleepy, slippy and sees things (halLEWYcinations)⚡
✨ Visual hallucinations are common
✨ Slow movements for Parkinson’s disease
✨ REM sleep abnormalities like drowsiness.
✨ DLB is associated with frequent falls.
✨ eosinophilic intracytoplasmic alpha synuclein aggregates.
Essential TREMORS
More common in males
Family history often present
It’s an action tremor (not resting)
Tremor begins in hands>arms>head»legs (very rarely are legs involved)
Rx: 1) Non-selective beta-blocker (Propranolol)
2) Primidone (anticonvulsant)
Alcohol improves the symptoms.
🧑🏻⚕️ Clinical Features of MSA
- Cranio-cervical dystonia
- High Pitched Dysarthria
- Pyramidal involvement ➕
- Autonomic features ➕
- Cerebellar features ➕
Radiological findings of MSA
IN MSA-C
1. Cruciform Hyperintensity @ Pons
2. Hot cross Bun sign
in MSA-P
3. PUtamen Ring Hyperintensity
PSP
🧠⚡BEEF HAFS⚡
- Broad Based Gait
- Extension Hypertonia: Retrocollis
- Eyelid Apraxia: Down Gaze Palsy
- Emotional Incontinence
- Frequent Falls
- Facial Dystonia: Procerus (OR) Surprise sign
- Hummingbird (OR) Penguin Sign on MRI
- Applause Sign ➕
- Frontal Release Sign ➕
- Spastic Dysarthria
PARKINSON plus syndrome with EARLY FREQUENT FALLS
PSP
Flexion Hypertonia seen in
Extension Hypertonia seen in
Flexion Hypertonia seen in
🎯 PARKINSON’S DISEASE
Extension Hypertonia seen in
🎯 PROGRESSIVE Supranuclear PALSY
Procerus sign (OR) Surprise sign seen in
Progressive Supranuclear Palsy
Applause sign
⭐ Seen in
When you ask to applause, the patient don’t STOP, NORMAL person stops
⭐ Seen in: Progressive Supranuclear Palsy
How to differentiate BETWEEN Progressive Supranuclear Palsy & Pick’s Disease
Applause test ➕ in PSP
Cause of development of HUMMINGBIRD SIGN on MRI
Atrophy of MID-BRAIN
Dysarthria: TYPES
- FLACCID
- SPASTIC
- CEREBELLAR
Difference BETWEEN PSP & CBD on H&E
CBD: Tufted ASTROCYTES
CBD: ASTROCYTE PLAQUES
PARKINSONISM Motor Features
➕
CORTICAL Features ➕
➕
MRI: CORTICAL ATROPHY
Corticobasal Degeneration
🧑🏻⚕️ Clinical Features of CORTICOBASAL DEGENERATION
- Apraxia, Agnosia
- Alien Limb Phenomena
- Asymmetrical DYSTONIC Contractions
- Dystonia
Alien Limb Phenomena seen in
⭐meaning
CORTICOBASAL DEGENERATION
⭐ Without own’s knowledge, limb assumes a position in space
⬇️
Doing something, you don’t know about
Neuroprotective Drugs used in PARKINSON’S DISEASE
- MAO-B ⛔
✨ Selegeline - Glutamate antagonist
✨ Riluzole
Drugs causing PARKINSONISM
🧠⚡ NA FART⚡
- Neuroleptics
- Amiodarone
- Flunarizine
- Alpha Methyl DOPA
- Reserpine
- Tetrabenazine
- Metoclopramide
- Lithium
- Cinnarizine
Treatment of PARKINSON’S DISEASE
🧠⚡ Cold SALAD⚡
Symptoms of PARKINSON’S that do NOT IMPROVE with Levodopa
🧠⚡CAP Falls ⚡
- Cognitive impairment: DEMENTIA ➕ Sleep d/o
- ANS Symptoms
- Postural instability
- Falls
Levodopa benefits which symptoms
🧠⚡TRAp ⚡
- Tremors
- Rigidity
- BRADYKINESIA (OR) Akinesia
🤢😳SIDE EFFECTS🥴😵 of LEVODOPA
Peak Dose Dyskinesia with LEVODOPA
Choreiform movement, Athetosis & Ballismus
- Young ♀️ with Severe Disease
- With HIGHER Dose of Levodopa
- Rhythmic contractions Predominant in Lower Limb
Wearing Off Phenomena
On Off Phenomena seen with
Levodopa
⬇️
Unpredictable fluctuations
✨ ON: Dyskinesia ➕
✨ OFF: Dystonia
PISA SYNDROME seen in
Seen in PARKINSON’S DISEASE pt treated with LEVODOPA
✨ In OFF Phase