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71 Cards in this Set

  • Front
  • Back
Midline cerebrellar lesion
truncal ataxia--inability to stand upright
tumor in 4th ventricle (medulloblastoma)

Anterior cerebellar lobe lesion
gait ataxia--still present with eyes open
anterior vermis syndrome--common in alcoholics
Posterior cerebellar lobe lesion
incoordination of voluntary movements of limbs
intention tremors
dysdiadochokinesia--difficulty with rapis alternating movements
impaired speech
athetoid
slow, rotating movements of distal muscles
choreiform
brisk, fast, dance-like
ballistic
fast, flailing, and rotary movements of proximal extremities
dystonia
sustained/repetitive muscular contractions or cramping

tic
brief, abrupt, spasmodic twitching and other stereotyped movement

can be suppressed
akathisia
movements acted out by an inner feeling for the need to move

can be suppressed

often induced by anti-psychotics
myoclonus
lightening-like intermittant muscle jerks
Parkinson's Disease
lesion in substantia nigra
dopamine
akinesia, bradykinesia, festination, anosmia, rigidity, cognitive/emotional disorders, resting tremor

herbicides, pesticides, and other chemicals

treatment = L-DOPA with Cardopa
Huntington's Chorea
hyperkinesia--choreiform and athetoid movements, hypotonia, and dementia

dopamine > ACh and GABA

major target--indirect (D2) striatalpallidal pathwat--lose the "braking" system
Hemiballismus
hyperkinesia--ballistic movements in proximal muscles

caused by inclusion of posteromedial central a.

target: subthalmic nucleus--braking system

dopamine > ACh and GABA
Dystonia
involuntary, sustained, cramping muscle contraction resulting in abnormal posture or repetitive twisting movements
Basal Ganglia and eye movements
Oculomoter loop through basal ganglia faciliatates and supresses eye movement
-GABA neurons in the pars reticulata project to VA and superior colliculi resulting in an inhibitory effect on eye movement

Results in eye fixation and abnormal eye movements
Tardive Dyskinesia
exhibit choreoathoid movements similar to those seen in huntington's
--may interfere with GABA (so too much dopamine)

seen in schizophrenic patients treated with older antipsychotic drugs
Cerebral Palsy
syndrome due to hypoxic or ischemic events in utero

results in various motor system impairments: paralysis, spasticity, rigidity, athetosis, and/or dystoni
Gilles De La Tourette
hereditary, males

multiple tics, rarely include corporalea

dopamine reuptake deficit and decreased serotonin in striatum
Obsessive Compulsive Disorder
unable to suppress repeated execution of a motor task because the striatal-frontal lobe fails to process completion of the goal

associated with Strep A
Restless Leg Syndrome
characterized by low iron in substantia nigra and low dopamine levels
rubella infection (weeks 7-8)
internal ear defects--congenital deafness
Craniofacial Dystosis
1st arch syndrome
Treacher-Collins
hypoplasia of mandible and maxilla
DiGeorge Syndrome
results from neural crest deficiency

affects Arch 1, Pouches 3 & 4
-thymus and parathyroids fail to develop resulting in immune deficiency
-wide eyes, low ears, small jaw

Rubella infection (weeks 4-6)
congenital cataracts, glaucoma
Kallman Syndrome
ineffective migration of ectoderm and neural crest cells into the hypothalamus (to become GRH cells) and base of skull (olfactory epithelium)

results in anosmia and hypogonadism
cleft lip and primary palate
failure of fusion between maxillary processes and medial nasal processes
cleft secondary palate
failure of fusion between lateral palatal shelves and septum
bilateral cleft lip and primary palate
failure of both maxillary processes to fuse with both medial nasal processes
cleft lip and primary/secondary palate
failure of fusion between medial nasal processes and maxillary processes AND the lateral palatal shelves, nasal septum, and primary palate
oblique facial cleft
failure of maxillary processes to fuse with lateral nasal processes

exposed nasolacrimal duct
transverse cleft (macrostomia)
wide mouth

failure of maxillary processes to fuse with mandible
midline cleft
failure of medial nasal processes to merge resulting in midline cleft lip and nasal groove
hypertelorism
excess neural crest cell production in the frontonasal prominence
holoprosencephaly
mutation in SHH resulting in defective determination of embryonic midline and right-left axis
Diabetes Insipidus
lesions that destroy supraoptic and paraventricular nuclei or disrupt the supraopticohypophyseal tract--low ADH

results in polyuria (excessive urination), polydipsia (excessive thirst), hypernatremia (high Na)
Syndrome of Inappropriate Secretion of ADH (SIADH)
due to excess secretion of ADH

results in hyponatremia

treatment = restrict water
Wernicke-Korsakoff's Syndrome
due to thiamine deficiency

causes degeneration of mamillary bodies (and DM) which inhibits the conversion of short term memory into long term memory
Craniopharyngioma
tumor in the hypothalamus

occurs in children and older males

results in obesity problems and precocious puberty
Hypothalami Hamartomas
mass of normal tissue in the wrong location

associated with gelastic seizures (laughing episodes) in children
auditory agnosia
superior temporal gyrus (left or right)

inability to recognize familiar sounds
tactile agnosia (astereognosis)
supramarginal gyrus lesion (left or right)

inability to recognize object by touch on contralateral side
apperceptive agnosia
diffuse bilateral damage to occipital cortex (2nd degree)

difficulty with visual perception
(impaired shape matching and copying ability)
associative agnosia
diffuse bilateral damage to occipital cortex (2nd or 3rd degree)

inability to access the meaning of what is percieved
(preserved copying ability of objects they can't recognize)
graphesthesia
intraparietal sulcus

inability to recognize writing on skin by touch
right parietal lobe (2-3) lesion
contralateral neglect--lack of awareness on one side of body
sensory extinction--loss of perception on one side (usually left) when both sides are stimulated at the same time
asomatognosia--lack of recognition of one's own body one side
dressing apraxia--inability to dress
due to impaired sense of orientation and sequence

parietal lesions (right and left)
constructional apraxia--inability to copy, draw, or construct simple figures
dyscalculia--inability to perform arithmetic calculations

left parietal lesions
ideomotor apraxia: impaired performance of skilled motor acts, despite intact sensory, motor and language function
--voluntary automatic dissociation

ideational apraxia: impaired ability to carry out sequence of actions for a complex task
expressive (broca's) aphasia
lesion in left broca's area

non-fluent speech (w/ writing impaired)
intact comprehension
poor repetition

*frustrated--aware of disability
receptive (wernicke's) aphasia
lesion in left wernicke's area

fluent speech
impaired comprehension
poor repetition

*not as frustrated
conduction aphasia
lesion in arcuate fasciculus

fluent speech
intact comprehension
poor repetition

*aware of errors but difficult to correct

transcortical motor aphasia (adynamia)
lesion in MCA-ACA watershed area (frontal lobe)

inability to initiate speech (writing impaired)
intact comprehension
good repetition
transcortical sensory aphasia
lesion in MCA-PCA watershed area

fluent speech
impaired comprehension
good repetition (echolalia)
mixed transcortical aphasia
watershed areas of MCA-ACA AND MCA-PCA

impaired speech
impaired comprehension
good repetition
global aphasia
occlusion of left internal carotid or MCA
--damages the whole left hemisphere

virtually complete loss of language
visual aphasia
lesion in angular gyrus

word blindness (alexia)

inability to read
speech, writing, comprehension are unimpaired
motor aprosodia
lesion in right equivalent of broca's area

monotone speech without inflection
sensory aprosodia
lesion in right equivalent of wernicke's area

inability to comprehend speech inflections
prosopagnosia
2 and 3 zone temporal lesions:
occipito-temporal, fusiform gyrus
(bilateral or right sided)

face blindness
inability to visually recognize faces
lesion in right temporal association cortex
defects in episodic memory
-experiences, specific events
lesion in left temporal association cortex
defects in semantic memory
-facts, general knowledge
lesion of medial temporal lobe (hippocampus)
anterograde amnesia

impaired ability to form new memories
lesion of lateral temporal lobe
retrograde amnesia

impaired ability to recall the past
temporal lobe epilepsy
seizure disorder preceded by olfactory hallucinations "uncinate fits"

characterized by: anterograde amnesia, automatisms, and perseverations
lesion in amygdala
placidity (loss of protective response), loss of associative learning, psychic blindness (revert to oral inspection), pedophilia
Kluver-Bucy syndrome
bilateral ablation of anterior temporal lobes including the amygdala and hippocampus

results in psychic blindness, placidity, deviant or hypersexual behavior, anterograde amnesia
Limbic lobe lesions
changes in behavior and personality
--excessive uncontrollable laughter, crying or other emotions
--panic reactions
clinical depression
decreased hippocampus

decreased dopamine, serotonin, and norepinephrine in frontal and limbic areas
acute stress
locus ceruleus releases NE which stimulates hippocampus and amygdala resulting in enhanced activity and memory (flashbulb memory--AMPA)

hypothalamus stimulate sympathetics to release more NE
chronic stress
results in release of CRH from hypothalamus which stimulates pituitary to release ACTH which stimulates adrenal cortex to release cortisol

results in diminished activity and volume of hippocampus and enhanced stimulation of amygdala which causes fear and anxiety--amy and hippo stimulate hypo continuing the cycle
schizophrenis
decreased dopamine in mesolimbic system and increased dopamine in mesocortical system (frontal and cinculate lobe)

results in widened sulci in prefontal cortex, shallow left superior temporal gyrus, and enlarged ventricles
Pick's disease
pick intracellular inclusion bodies in frontal and temporal lobes

results in loss of inhibitions (rude, verbally, sexually aggressive)