Basal Ganglia Disorder Degeneration of dopaminergic neurons in the substantia nigra pars compacta. Low Dopamine Intraneuronal proteinaceous inclusions [ α-synuclein] in cell bodies and axons (Lewy bodies) Cardinal Motor Features Bradykinesia Tremors Rigidity Gait imbalance
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Basal Ganglia Organization Two pathways Direct & Indirect Direct = Excitatory Indirect = Inhibitory Dopaminergic neurons that project from the substantia nigra to the putamen normally have two effects Stimulate the D1 dopamine receptors inhibit D2 receptors
Basal Ganglia Organization
Dopamine denervation Loss of dopaminergic tone Increased firing of neurons in the STN and GPi Development of parkinsonian features
Bradykinesia Slowness of initiation of voluntary movement with a progressive reduction in speed and range of repetitive actions, such as voluntary finger-tapping
Rigidity Resistance to externally imposed ("passive") joint movements Leadpipe rigidity is sustained resistance to passive movement throughout the whole range of motion, with no fluctuations. Cogwheel rigidity is jerky resistance to passive movement as muscles tense and relax. Tremor superimposed on this hypertonia that results in intermittent increase in tone during the movement.
Rig idity vs Spasticity More resistance in one direction the other direction It is velocity dependent (i.e. more noticeable with fast movements Same resistance in all directions Not velocity dependent – does not vary with speed of movement of muscle groups involved
Postural instability A disturbance in balance that impairs the ability to maintain an upright posture when standing and walking Festinating gait feeling like being stuck in place, when initiating a step or turning, and can increase the risk of falling.
Tremors Rhythmic oscillation of body due to intermittent muscle contractions Promitent at REST . Pathophysiology behind this???????
NON MOTOR Mood disorders Sleep disturbances Orthostatic hypotension GIT disturbances Sexual dysfunction Cognitive impairment Anosmia OTHER MOTOR FEATURES Micrographia Mask like faces Reduced eye blinking Hypophonia Dysphagia Hypommia
Parkinsonism is a generic term that is used to define a syndrome manifest by rigidity, bradykinesia, tremor, and postural instability. PARKINSONISM VS PARKINSON DISEASES
CLASSIFICATION PARKINSONISM Idiopathic Atypical secondary Hereditary/Neurodegenrative PARKINSON PSP Drug SNCA GENE Mutation Corticobasal Syndrome Infection GLUCOCEREBROSIDASE MSA Tumor Wilson disease No response to levodopa Trauma Dementi a with Vascular Lewy bodies Toxins (MPTP)
TREATMENT Balance between dopaminergic and acetylcholnergic Dopaminergic drugs L DOPA
TREATMENT Balance between dopaminergic and acetylcholnergic Dopaminergic drugs L DOPA Dopa precursor of dopamine Dopa can 't cross blood brain Barrier. Decarboxylase inhibtor ( Carbidopa)
T o prevent its peripheral metabolism to dopamine the development of nausea, vomiting, and orthostatic hypotension Due to activation of dopamine receptors in the area postrema (the nausea and vomiting center) Not protected by the BBB. Carbidopa can't cross blood brain barrier
Deep brain stimulation (DBS) Target areas for DBS or lesions include the thalamus, globus pallidus, or subthalamic nucleus. DBS involves the implantation of a medical device called a neurostimulator, which sends electrical impulses to specific parts of the brain. DBS is recommended for people who have PD with motor fluctuations and tremor inadequately controlled by medication, or to those who are intolerant to medication, a
Physical t herapies and Exercises ANDY WRIGHT
A 64-year-old male presents to your office because he has had two falls within the last month. He states that he loses his balance when he tries to turn or stop suddenly while walking. Recently, he says, it has been taking him quite a while to get himself out of bed. He also complains of hand tremors that started last year in his left hand, but that now have been affecting both hands. Which of the following is the best tool to confirm his diagnosis? A Physical examination B. Lumbar puncture C. CTscan of the head D. Electroencephalography E. Nerve conduction studies
To date, there are no imaging or laboratory tests that can be used to confirm this diagnosis with any greater accuracy than physical examination. EEG is typically used in the assessment of patients with seizure disorders, sleep disorders and metabolic disorders affecting the CNS. Peripheral nerve conduction studies may yield unremarkable or non-specific results. Such studies are typically used to assess for peripheral neuropathy
Brain CT scan findings may be unremarkable or non-specific in patients with PD. No imaging study is required in patients with a typical PO presentation MRI may be useful to exclude items on the differential diagnosis, such as a multi-infarct state, normal pressure hydrocephalus, etc.
Diagnosis?
Robin Williams Dementia with lewy body
What can you do ??????
To get invol ved in research Still don't know the exact pathophysiology behind this