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
What would you NOT expect to see on an MRI scan of a brain from a person with advanced Huntington's Disease?
C. enlarged caudate nuclei
True or False: Fasciculations are symptomatic of cervical spondylosis with rediculopathy and with amyotrophic lateral sclerosis (ALS)
True or False: Myokymia is often decreased or absent in a patient with multiple sclerosis.
What type of consequences might you see with a lesion in the central tegmentum of the brainstem?
palatal myoclonus (jerks of pharyngeal/palatal muscles)
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 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.
What is FALSE about tics?
E. they often present with slow, gradual onset
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)
Which statment about myoclonus is FALSE?
B. myoclonus is not a possible consequence of uremia
Which of the following can cause diseases/conditions with myoclonus?
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)
True or False: Intentional tremors are commonly associated with damage to the cerebellar outflow systems.
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
What symptom would be caused by an infarction in the posterior cerebral artery resulting in damage to the subthalamic nuclei?
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
Which condition is associated with basal ganglia damage secondary to kernicterus (bilirubin disorder)?
athetosis (slow, twisting, writhing movements; alternation of flexion/extension, supination/pronation)
What are some examples of focal dystonias?
A likely location of a single lesion which causes both left facial and right arm/leg paralysis (alternating hemiplegia) is in the:
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.
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.
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.
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.
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.
Myokymia is associated with damage to what part of the nervous system?
peripheral motor nerve
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
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)
Resting tremors are associated with damage to which part of the nervous system?
Slow speech with telegraphic content, impaired writing, dysarthria, and hemiparesis are characteristics of which type of aphasia?
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?
Anomias, or disorders of naming, are associated with damage to which part of the brain?
(dominant) temporal lobe
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)
Infarction or transection of the anterior corpus callosum may lead to what?
apraxia (inability to carry out learned, purposeful movements) of LEFT extremities
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
Which is FALSE about memory loss (i.e. amnestic syndrome)?
E. damage to the medial thalamus does not usually result in memory loss
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
vascular complications (e.g. embolus to "top of basilar")
What are some general symptoms associated with lesions in the right hemisphere?