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48 Cards in this Set

  • Front
  • Back
what are the 5 componenents of the basal ganglia?
1. Putamen
2. Globus Pallidus
3. Caudate Nucleus
4. Subthalamic Nuclei
5. Substantia Nigra
What 2 components of the basal ganglia compose the lentiform nucleus?
1. Putamen
2. Globus Pallidus
What are the two parts of the the corpus striatum?
1. Lentiform nucleus (Putamen, Globus Pallidus)
2. Caudate nucleus
What are the 6 types of abnormal movement?
1. tremor
2. chorea
3. athetosis or dystonia
4. ballismus
5. myoclonus
6. tics
The type of tremor seen with Parkinson disease is called?
Resting tremor (present at rest, disappears or is reduced with movement.)
Benign essential tremor is what type of tremor?
Postural - strong familial link
What drugs are associated with worsening physiologic tremor?
1. Valproic acid
2. Lithium
3. Bronchodilators
4. TCA's
what type of movement abnormality is asterixes classified as?
myoclonus - intermittent cessation of extensor action reveals underlying flexor myoclonus resulting in flapping up and down o hand at wrist.
Damage to what brain structure results in intention tremor?
superior cerebellar peduncle
what is the typical frequency of a resting tremor such as that seen with Parkinson disease
4-6 Hz
Which disease can cause, resting, postural, and intention tremors?
Wilsons disease (diffuse brain damage leads damage to multiple areas of the movement circuitry
What is rapid, irregular, muscle jerks that occur involuntarily and unpredictably in different parts of the body?
Chorea
Sudden dipping or lurching to one side during gate is characteristic of which movement abnormality?
Chorea
Damage to what two brain structures can cause chorea?
Caudate nucleus and putamen
What are two potentially curable causes of chorea?
Thyrotoxicosis and polycythemia vera
What are the two important hereditary causes of chorea?
Wilsons and Huntington's Disease
What is sydenham chorea?
Chorea caused by acute Rhematic fever. Usually clears within several months of antibiotic treatment, but can sometimes appear later in life.
What are common iatrogenic causes of chorea?
Antipsychotic meds and Parkinson treatment (levodopa or other dopaminergic drugs
What is unilateral chorea that is especially violent because the proximal muscles of the limbs
are involved?
Hemibalismus
What causes hemibalismus?
vascular damage to the contralateral subthalamic nucleus - usually clears within several weeks
Abnormal movements that are slow, sinuous, and writhing are called?
Athetosis - if these movements are sustained the may be regarded as a postural dystonia
Which diseases can be associated with athetosis/dystonias?
Parkinsons, Huntingtons, and Wilsons
What are the common iatrogenic causes of athetosis/dystonias?
Dopaminergic drugs and antipsychotics
Sudden, rapid, twitch-like movements are known as?
Myoclonus
What is an important infectious cause of myoclonus?
Creutzfeldt-jakobs disease
Segmental myoclonus can be caused by damage to what brain structures?
Actually damage caused to any of the central nervous system structures can cause this (cortex, brainstem, spinal cord)
what is epilepsia partialis continua and what causes this disorder?
repetitive focal epileptic discharge occuring from the contralateral sensorimotor cortex leading to segmental myoclonus. Caused by hyperosmolar nonketotic hyperglycemia
Treatment of myoclonus?
Depakote 250-500mg TID, clonazepam .5mg up to 12mg daily. For myoclonus associated with anoxic brain injury, 5-HT precursor to serotonin is very effective (combine with carbodopa to avoid peripheral metabolism).
Familial essential tremor has what sort of inheritance pattern?
AD
what is treatment of familial essential tremor?
Propranolol 40-120 mg daily. Also high frequency thalamic stimulation can be helpful in severe cases which do not respond to other approaches.
"lower body Parkinsons" or primary disturbances of gait with only other minor parkinsonian symptoms may be caused by?
Vascular disease of white matter
what is Myerson sign?
sustained blink relfex after tapping bridge of nose on someone with Parkinsons disease
Common cause of falls in persons with parkinsons disease related to therapy with dopaminergic agents?
Orthostatic hypotension - always get orthostatic BP's in office
neuronal degeneration with the
The presence of neurofibrillary tangles in the midbrain, pons, basal ganglia, and dentate nuclei of the
cerebellum is hallmark for what movement disorder?
PROGRESSIVE SUPRANUCLEAR PALSY
What movement disorder does this discribe? Gait disturbance with early falls, supranuclear ophthalmoplegia, pseudobulbar palsy, axial dystonia with
or without extrapyramidal rigidity of the limbs, and dementia.
progressive supranuclear palsy
This is characterized by prominent failure of voluntary vertical gaze, with later paralysis of horizontal gaze?
Supranuclear ophthalmoplegia
What movement disorder presents this way? Neck often assumes an extended posture (axial
dystonia in extension), with resistance to passive flexion.
progressive supranuclear palsy
midbrain atrophy
(hummingbird sign)?
progressive supranuclear palsy
Apraxia and clumsiness are more common in this movement disorder rather than extrapyramidal deficits (tremors, dyskinesia, rigidity) often seen with Parkinsons
Corticobasal degeneration
Alien limb syndrome (involuntary, semi-purposeful, movement of limb without owners recognition)
Corticobasal degeneration
this disease is characterized by a mutation involving a gene located on chromosome 4p16.3
Huntings disease and huntingtin
how is huntingtons inherited?
AD with complete penetrance
what is the characteristic mutation which causes huntingtons?
expansion of a CAG trinucleotide repeat that codes for
a polyglutamine tract
Biochemical studies have shown that the concentrations of the inhibitory neurotransmitter GABA, its biosynthetic enzyme glutamic acid decarboxylase (GAD), and acetylcholine and its biosynthetic enzyme choline acetyltransferase are all reduced in the basal ganglia of patients with this disease?
Huntingtons
what is action of drugs such as reserpine and tetrabenazine?
reduce dopaminergic inhibition of striatal output neurons by depleting dopamine at a nerve terminals
mutations in the gene localized in the region of chromosome 13q14–21 causes what disease?
Wilsons
"face of the giant panda" sign in
the midbrain and sometimes a "face of the miniature panda" in the pontine tegmentum?
Wilsons disease
what is treatment for Wilsons disease?
Chelating agent penicillamine to remove copper from serum