Neuro Clinical Correlations

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flucas
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19060
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Neuro Clinical Correlations
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2010-05-24 01:51:17
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movement disorders neuroscience neuroanatomy Neuro Clinical Correlations HUBIO Felicia Lucas project neuro
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Neuro Clinical Correlations (HUBIO 532)
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  1. What are some examples of involuntary movements?
    • myoclonus (jerks)
    • tics
    • tremor (oscillations; alternating contract/relax of agonist/antagonist muscles) (action or resting)
    • postural tremor (evident when arms are stretched out or when wrists are dorsiflexed; tremor may be absent when limb is supported; feet almost never involved)
    • chorea (more purposeful than myoclonus)
    • hemiballismus (unilateral movements of limbs)
    • dystonia (twisting, abnormal posture, muscle spasms)
    • athetosis (slow, sinuous, writhing movements)
    • fasciculations (twitches of individual somatic motor units; can be benign)
    • myokymia (undulating contractions of muscle; peripheral nerve damage)
    • hemifacial spasm (repetitive contractions on one side of part or allof the muscles innervated by the seventh cranial nerve; irregular)
  2. What are some diseases associated with involuntary movements (i.e. dyskinesia)?
    • Parkinsonism
    • Huntington's disease
    • Torsion Dystonia (genetic; involves trunks & limbs)
    • Tardive Dyskinesia (dyskinesia as a side effect of medications, do not immediately recover; e.g. antipsychotic drugs like haloperidol)
    • Wilson's Disease (genetic; retain copper in system)
    • Tourette's Disease
    • (epilepsy)
    • Essential/Familial Tremor Disease (resting tremors; postural tremors)
    • Writer's cramp (focal dystonia)
    • spasmodic torticollis (focal dystonia)
    • blepharospasm
  3. True or False: Parkinsonism is associated with resting tremors.
    True
  4. True or False: Certain toxins (e.g. MPTP) and drugs (Phenothiazines, metoclopramide) can cause symptoms similar to those seen with Parkinson's Disease.
    True
  5. What are some typical syptoms and signs of Parkison's Disease?
    • resting tremors that improve with movement
    • bradykinesia (slowness of movement)
    • rigidity
    • balance problems
    • decrease in substantia nigra (midbrain)
    • Lewy bodies (aggergates of protein) in neurons in brain
    • (Parkinson's disease is not the same as Parkinsonism)
  6. What is the neurotransmitter transmitted in the nigrastriatal fibers? Where do they run?
    • dopamine
    • the nigrastriatal fibers run from the sustantia nigra to the striatum of the basal ganglia
  7. What are the characteristics of Huntington's Disease?
    • autosomal dominant
    • mutation in Huntington gene (CAG expansion)
    • no present effective treatment
    • symptomatic treatment for chorea and dementia (e.g. neuroleptic drugs such as haloperidol)
    • sx: dementia, chorea, athetosis, enlarged frontal horns of lateral ventricales in brain, cortical atrophy, atrophy of caudate nuclei
  8. What is the typical treatment for Parkinson's Disease?
    L-dopa (e.g. Sinemet)
  9. What are the characteristics of Wilson's Disease?
    • rare, but important not to miss (KF ring)
    • autosomal recessive mutation (hepatic copper transporting ATPase)
    • hepatic and cerebral forms
    • free Cu (total Cu: 3x ceruloplasmin) is high (>15-20 ug/dl)
    • 24 hr urine Cu is high
    • sx: increasing dystonia, chorea, and/or tremors; Kaiser-Flasher ring (copper ring in inner layer of cornea); hyperintensities in basal ganglia (MRI image); copper deposits in liver, kidneys, and cornea
    • tx: remove liver
  10. What are some examples of dystonias?
    • cervical dystonia (torticollis: neck, focal dystonia)
    • generalized dystonia
    • Writer's cramp
    • oromandibular dystonia (Meige syndrome)
    • spasmodic dysphonia
    • Tardive dyskinesia
  11. What would you NOT expect to see on an MRI scan of a brain from a person with advanced Huntington's Disease?

    A. enlarged lateral ventricles
    B. atrophied caudate nuclei
    C. generalized cortical atrophy
    D. enlarged caudate nuclei
    D. enlarged caudate nuclei
    (this multiple choice question has been scrambled)
  12. True or False: Fasciculations are symptomatic of cervical spondylosis with rediculopathy and with amyotrophic lateral sclerosis (ALS)
    True
  13. True or False: Myokymia is often decreased or absent in a patient with multiple sclerosis.
    False
  14. What type of consequences might you see with a lesion in the central tegmentum of the brainstem?
    • involuntary movements
    • palatal myoclonus (jerks of pharyngeal/palatal muscles)
  15. True or False: Hemifacial spasms are often caused by pressure of an artery (posterior inferior cerebellar artery) that is located close to the root entry nerve of the ipsilateral cranial facial nerve (CN VII).
    True
  16. True or False: Hemifacial spasms are different from tics because patients with tics often have the ability to concentrate and inhibit the movements while patients with hemifacial spasms often do not have this ability.
    True
  17. What is FALSE about tics?

    A. they go away during sleep
    B. they are characteristically sterotyped and can mimic coordinated movements
    C. they typically involve facial muscles but can spread to the neck, shoulders, trunk, and limbs
    D. they often present with slow, gradual onset
    E. a person can often concentrate and inhibit the movements
    D. they often present with slow, gradual onset
    (this multiple choice question has been scrambled)
  18. All of the following are potential symptoms of which involuntary movment disease?

    multiple motor and vocal tics
    symptoms usually appear between the ages of 2-25 years old
    patients may snort, grimace, hiccough or emit explosive sounds
    corprolalia (expression of vulgar words)
    Tourette's syndrome
  19. Which statment about myoclonus is FALSE?

    A. myoclonus is a possible consequence of encephalitis
    B. myoclonus is not a possible consequence of uremia
    C. can be caused by hyperexcitability of nervous system at any level from spinal cord to cerebral cortex
    D. characterized by sudden brief jerks of muscles or muscle groups
    E. muscle jerks may be segmental or generalized
    B. myoclonus is not a possible consequence of uremia
    (this multiple choice question has been scrambled)
  20. Which of the following can cause diseases/conditions with myoclonus?

    a. mutations in mitochondrial gene
    b. degenerative disorders of cortical neurons
    c. anoxic-ischemic damage to CNS, especially serotoninergic centers
    d. metabolic conditions (e.g. uremia)
    e. encephalitis
    f. all of the above
    g. more than one but not all of the above
    f. all of the above

    • a. mutations in mitochondrial gene --> MERRF (myoclonic epilepsy with red ragged fibers)
    • b. degenerative disorders of cortical neurons --> progressive myoclonic epilepsy; Baltic myoclonus
    • c. anoxic-ischemic damage to CNS, especially serotoninergic centers --> generalized myoclonus
    • d. metabolic conditions (e.g. uremia)
    • e. encephalitis
  21. True or False: Intentional tremors are commonly associated with damage to the cerebellar outflow systems.
    True
  22. What is Benedikt's syndrome and what causes it?
    • Benedikt's syndrome: severe intentional tremors in extremities because of damage to contralateral red nucleus in midbrain
    • cause: infarct due to occlusion of a branch of the basilar artery
  23. What symptom would be caused by an infarction in the posterior cerebral artery resulting in damage to the subthalamic nuclei?
    contralateral hemiballismus
  24. Chorea is generally associated with damage to which part of the brain? What is an example of a disease associated with chorea?
    • basal ganglia, esp. striatum
    • Huntington's disease
  25. Which condition is associated with basal ganglia damage secondary to kernicterus (bilirubin disorder)?
    athetosis (slow, twisting, writhing movements; alternation of flexion/extension, supination/pronation)
  26. What are some examples of focal dystonias?
    • blepharospasm
    • writer's cramp
    • spasmodic torticollis
  27. A likely location of a single lesion which causes both left facial and right arm/leg paralysis (alternating hemiplegia) is in the:
    leftside pons
  28. At what level is the unilateral lesion that would cause the following: Loss of pain and temperature sensation on ONE side of the body AND loss of vibration and position sense on the OPPOSITE side of the body.
    spinal cord
  29. At what level would the unilateral lesion be to cause the following: Loss of fine touch and vibration sense on ONE side of the body AND fasciculations of the tongue on the side OPPOSITE to the paralysis.
    medulla
  30. At what level would the unilateral lesion be to cause the following: Weakness and hyperreflexia on ONE side of the body AND a homonymous hemianopsia IPSILATERAL to the paralysis.
    cortex
  31. At what level would the unilateral lesion be to cause the following: Loss of pain and temperature sense on ONE side of the body AND some loss of pain and temperature sense on the OPPOSITE side of the face.
    medulla
  32. At what level would the unilateral lesion be to cause the following: Weakness and hyperreflexia on ONE side of the body AND loss of pupillary accommodation in the CONTRALATERAL eye.
    pons
  33. Myokymia is associated with damage to what part of the nervous system?
    peripheral motor nerve
  34. Fasciculations are associated with damage to which part of the nervous system? What diseases are associated with fasciculations?
    • spinal cord; nerve roots; anterior horn cells
    • (i.e. lower motor neurons)
    • cervical spondylosis, ALS
  35. Intentional tremors are associated with damage to which part of the nervous system? What is an example of a disease associated with intentional tremors?
    • cerebellum (esp. dentate nucleus)
    • Benedikt's syndrome
  36. Resting tremors are associated with damage to which part of the nervous system?
    substantia nigra
  37. Slow speech with telegraphic content, impaired writing, dysarthria, and hemiparesis are characteristics of which type of aphasia?
    nonfluent aphasia
  38. When a person has impaired comprehension and can speak in complete sentences but often has paraphasic errors and jargonisms, they are said to have which type of aphasia?
    fluent aphasia
  39. Anomias, or disorders of naming, are associated with damage to which part of the brain?
    (dominant) temporal lobe
  40. What type of aphasia is associated with damage to the arcuate fasciculus and what are its particular symptoms?
    • damage to the arcuate fasciculus --> conduction aphasia
    • disorder of repitition (e.g. "no ifs, ands, or buts" said repeatedly)
  41. Infarction or transection of the anterior corpus callosum may lead to what?
    apraxia (inability to carry out learned, purposeful movements) of LEFT extremities
  42. A patient presents with "alexia without agraphia" (unable to read but able to write) and right homonymous hemianopsia but is able to name and recognize objects. What areas of the brain would you suspect to be damaged? If the brain damage resulted from arterial occlusion, which artery is most likely involved?
    • damage: left visual cortex; posterior corpus callosum
    • artery: posterior cerebral artery
  43. Which is FALSE about memory loss (i.e. amnestic syndrome)?

    A. memory loss often results from damage to the hippocampus
    B. damage to the medial thalamus does not usually result in memory loss
    C. memory loss usually implies bilateral lesions in the brain
    D. damage to the temporal lobe unilaterally does not usually result in memory loss
    E. herpes simplex encephalitis is known to result in memory loss
    B. damage to the medial thalamus does not usually result in memory loss
    (this multiple choice question has been scrambled)
  44. What are some common causes of isolated amnestic syndrome?
    • thiamine deficiency (Wernicke-Korsakoff syndrome)
    • bilateral infarcts of medial temporal lobes
    • bilateral infarcts of dorsomedial thalamus
    • bilateral temporal lobectomy
    • carbon monoxide poisoning
    • herpes simplex encephalitis
    • temporal lobe contusions
    • thalamis tumors
    • vascular complications (e.g. embolus to "top of basilar")
    • trauma
  45. What are some general symptoms associated with lesions in the right hemisphere?
    • inattention to left side
    • denial of disability/existence of left side
    • "cortical" sensory loss: bilateral simultaneous stimulation, stereognosis, 2-pt. discrimination, figure-writing
    • not usually language loss (associated more with left hemisphere damage)
    • inability to perform visual-constructional tasks (e.g. copying a drawing)
    • loss of precision in doing fine movments with left hand
    • deficits in spatial visualization, especially on left side
  46. Damage to which cerebral hemisphere (left or right) is associated with language-loss syndromes (i.e. aphasias)?
    damage to left cerebral hemisphere
  47. True or False: Even in left-handed individuals the left cerebral hemisphere appears to be more important (than the right) for speech.
    True
  48. True or False: Damage to the thalamus may result in both amnesia AND aphasia.
    True
  49. Damage to which areas of the brain might result in speech disturbances?
    • (left) temporal lobe
    • left cerebral hemisphere
    • Broca's area (frontal lobe)
    • Wernicke's area (temporal lobe)
    • primary and supplementary motor areas
    • connections between cortical areas (i.e. association bundles; commissural bundles)
    • left thalamus
  50. Electrical stimulation of the primary and supplementary motor areas (in either hemisphere) typically result in which type of speech alteration(s)?
    • speech arrest
    • vocalization
  51. Electrical stimulation of the thalamic nuclei in the dominant hemisphere typically result in which type of speech alteration(s)?
    • arrest of speech
    • inability to name objects
  52. What are the basic types of aphasia?
    • expressive
    • receptive
    • fluent
    • nonfluent
    • anomic (nominal) temporal lobe
    • conduction
    • Wernicke's
    • isolation areas
  53. Lesions around the operculum of the inferior frontal lobe (Broca's area) often result in which type of aphasia?
    nonfluent (expressive, motor, verbal) aphasia
  54. Persistent aphasias are associated with damage to which particular part of the cerebral cortex?
    insular cortex and adjacent white matter
  55. What types of aphasia are associated with cortical damage to the parietal lobe or temporal-parietal area?
    fluent (sensory, receptive) aphasia
  56. What are the 4 basic types of fluent aphasia and what areas of the brain are they associated with?
    • Wernicke's: posterior superior temporal lobe
    • intentional areas: areas involved with intact speech
    • anomic temporal lobe: angular gyrus or lower (i.e. mid to inferior temporal lobe)
    • conduction: suprasylvian-parietal lobe
  57. What are the 4 basic types of fluent aphasia and is their comprehension or repition impaired, or both?
    • Wernicke's: comprehension impaired, repitition impaired
    • conduction: comprehension normal, repitition impaired
    • anomic temporal lobe: comprehension normal, repitition normal
    • isolation areas: comprehension impaired, repitition normal
  58. What type of brain damage results in "general aphasia" and what are some associated symptoms?
    • general aphasia: damage to both Broca's (frontal lobe) and Wernicke's areas (posterior superior temporal lobe)
    • symptoms: nonfluent aphasia combined with loss of comprehension and repetition, possibly writing difficulties
  59. What are the cortical areas involved in speech and speech perception?
    • facial area of motor cortex
    • Broca's Area (operculum of frontal lobe)
    • Wernicke's Area (posterior superior temporal lobe)
    • transverse temporal gyrus
    • planum temporale
    • angular gyrus
    • visual association area
    • visual cortex (parietal lobe)
  60. What is the correct sequence (first to last) of the cortical areas involved in naming a seen object?

    a. visual cortex, motor cortex (facial area), angular gyrus, Wernicke's area, Broca's area
    b. visual cortex, Wernicke's area, Broca's area, angular gyrus, motor cortex (facial area)
    c. visual cortex, angular gyrus, motor cortex (facial area), Wernicke's area, Broca's area
    d. visual cortex, angular gyrus, Broca's area, Wernicke's area, motor cortex (facial area)
    e. visual cortex, angular gyrus, Wernicke's area, Broca's area, motor cortex (facial area)
    • e. visual cortex, angular gyrus, Wernicke's area, Broca's area, motor
    • cortex (facial area)
  61. What is the correct sequence (first to last) of the cortical areas involved in understanding the spoken name of an object?

    A. transverse temporal (Heschl's) gyrus, Wernicke's area, angular gyrus, visual association area
    B. transverse temporal (Heschl's) gyrus, angular gyrus, Wernicke's area, visual association area
    C. transverse temporal (Heschl's) gyrus, visual association area, angular gyrus, Wernicke's area
    D. transverse temporal (Heschl's) gyrus, Wernicke's area, visual association area, angular gyrus
    A. transverse temporal (Heschl's) gyrus, Wernicke's area, angular gyrus, visual association area
    (this multiple choice question has been scrambled)
  62. True or False: The visual association cortex is involved in naming a seen object; it is not involved in understanding the spoken name of an object.
    False
  63. Electrical stimulation of the nulcei in the left thalamus results in what types of behavioral disruption?
    • interuption of speech
    • inability to name objects
  64. A patient who has undergone a surgery to sever the corpus callosum will not be able to readily name an object that is placed in which half of the visual field?
    • left visual field
    • (the right cerebral hemisphere, which would "identify" the object in the left visual field, cannot communicate with the language centers in the left cerebral hemisphere)
  65. What are the three basic types of memory and how are they each tested?
    • immediate recall: repeat numbers, recall names of objects
    • short-term memory: recall names of objects or events of the recent past even after a distraction
    • long-term memory: recall events remote in time
  66. Thiamine deficiency related to amnestic syndromes is often associated with damage to which particular part of the diencephalon?
    mammillary bodies (hypothalamus)
  67. What are some major cortical and subcortical structures that are affected by an occlusion (e.g. thrombus, embolus) in the posterior cerebral artery?
    • amygdala
    • amygdaloid nuclei
    • hippocampus
    • uncus
    • parahippocampal cortex
  68. What is the medical definition of a seizure?
    abnormal, excessive, paroxysmal (sudden and transient), synchronous discharge of a population of neurons, with a change in behavior and/or perception
  69. What are the phases of an epileptic's life?
    • aura: brief period at start of seizure
    • ictus: sequence of events that make seizure
    • postictal: period immediately following seizure
    • interictal: all the rest of epileptic's life
  70. What are the different types of generalized seizures?
    • tonic-clonic (grand mal)
    • absence-petit mal
    • myoclonic
    • atonic
  71. Which cerebral lobe is the most common origination site for focal seizures?
    temporal lobe
  72. True or False: Any process that cuases destruction or irritation of the cerebral cortex can lead to a focal seizure.
    True
  73. How do the EEg's of a generalized seizure and a focal seizure compare?
    • generalized seizure: simultaneous discharge can be seen over the entire brain
    • focal seizure: abnormal electrical dishcarge is seen in just one area of the brain, it may spread to other areas
  74. What marking on an EEG is diagnostic of epilepsy?
    • interictal epileptiform discharge
    • (paroxysmal depolarizing shift, PDS)
  75. What is the PDS (paroxysmal depolarizing shift) and what are some possible mechanisms?
    • PDS: underlies interictal epileptiform discharge; synchronization of electrical activity in many neurons
    • possible mechanisms of PDS:
    • intrinsic membrane properties (increased/decreased polarization)
    • synaptic (increased/decreased excitation, pre/post synaptic)
  76. Epilepsy is associated particularly with dysfunction of which type of neurotransmitter?
    GABA (inhibitory neurotransmitter)
  77. What is the general definition of cerebral palsy?
    • i.e. static encephalopathy
    • a non-progressive syndrome of delayed and abnormal motor development due to dysfunction of the central nervous system
    • children may have spastic weakness, involuntary movements
    • cognitive impairment and epilepsy may also be present
    • numerous causes, mostly idiopathic
  78. What are the clinical features of intellectual disability (mental retardation)?
    • sub-average intellectual abilities (<70 IQ)
    • limitations in adaptive functioning
    • non-progressive syndrome
    • must be differentiated from degenerative disorders like dementia (i.e. loss of cognitive function)
  79. Which statement about intellectual disability (mental retardation) is FALSE?

    A. males and females are equally afflicted with intellectual disabilities
    B. intellectual disability is over-represented in lower socioeconomic classes
    C. intellectual disability may result from congenital, metabolic, toxic, cytogenetic, or single gene disorders
    D. it is a non-progressive syndrome that is distinct from degenerative cognitive disorders
    E. most people with intellectual disabilities suffer from a mild form
    A. males and females are equally afflicted with intellectual disabilities (more men than women, 1.5:1)
    (this multiple choice question has been scrambled)
  80. What are the 3 general patterns of abnormal motor signs (e.g. spasticity, weakness) associated with cerebral palsy?
    • diplegia
    • hemiplegia
    • quadriplegia
  81. What are the 4 classic signs of "floppy baby syndrome" (i.e. hypotonia)?
    • paucity of voluntary movement
    • unusual postures
    • decreased resistance to passive movement of a joint
    • increased joint mobility
  82. True or False: Upper motor neuron damage may present as hypertonia in an adult and as hypotonia in an infant.
    True
  83. What are the 2 patterns of hypotonia and how do they compare?
    • paralytic hypotonia: children are weak, associated with peripheral nerve damage
    • non-paralytic: children are floppy but have adequate strength, associated with CNS damage
  84. What are some possible causes of intellectual disability (mental retardation)?
    • chromosomal alterations (e.g. trisomy 21)
    • single gene disorders
    • prenatal, perinatal, postnatal injuries to CNS
    • abnormal brain development
    • malnutrition
    • environmental causes (abuse, neglect, under-stimulation)
    • most etiology is idiopathic
  85. Which statment about myopathies is true?

    A. thyroid myopathy and polymyositis are examples of genetic myopathies
    B. all myopathies are genetic
    C. proximal muscles generally atrophy before distal muscles
    D. Duchenne's dystrophy is an example of a channelopathy
    C. proximal muscles generally atrophy before distal muscles
    (this multiple choice question has been scrambled)
  86. Myasthenia gravis is the most common representation of which class of disorders?
    disorders of the neuromuscular junction
  87. Which statment about peripheral nerve disorders is FALSE?

    a. pronounced weakness in distal muscles is associated with polyneuropathies
    b. when sensory fibers are affected by peripheral nerve disorders, sensory loss follows a "glove and stocking" distribution
    c. toxins, such as arsenic, can cause polyneuropathies
    d. diabetes mellitus is an example of a disease associated with mononeuropathies
    • d. diabetes mellitus is an example of a disease associated with
    • mononeuropathies
  88. Dermatomal distribution of pain, tingling, weakness, loss of reflex, and sensory loss are symptoms of what type of neurological disorder?
    nerve root disease
  89. What are some clinical manifestations of lateral medullary plate syndrome and what causes it?
    • cause: infarction in dorsolateral medulla (occlusion of vertebral or posterior inferior cerebellar artery)
    • symptoms:
    • loss of pain/temperature sensation on face (ipsilateral) (CN V)
    • loss of pain/temperature sensation on half of body (contralateral) (spinothalamic tract)
    • clumsiness of extremities (ipsilateral) (cerebellum/inferior cerebellar peduncle)
    • Horner's syndrome (ipsilateral) (descending sympathetic fibers)
    • hoarseness, palate/posterior pharynx weakness (ipsilateral) (nucleus ambiguus)
  90. Ipsilateral 3rd nerve palsy and contralateral tremors are associated with damage to which area of the brain?
    midbrain
  91. Ipsilateral CN VI and CN VII palsies with contralateral hemiparesis are associated with damage to which area of the brain?
    pons

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