Behavioral Neurology Flashcards
Aphemia
Apraxia of speech characterized by complete articulatory failure in the presence of preserved writing, comprehension and oropharyngeal function.
Lesion in the left inferior frontal gyrus (Broca’s area)
Broca’s aphasia
Expressive aphasia. Nonfluent speech; can’t name or repeat. Written language also affected. Comprehension intact. Due to a lesion in the posterior part of the dominant inferior frontal gyrus
Wernicke’s Aphasia
Receptive aphasia. Comprehension impaired. Speech is fluent but nonsensical. Lesion in posterior portion of dominant superior temporal gyrus.
Conduction aphasia
Impaired repetition. Speech is fluent but not perfect and patients can comprehend spoken word. Naming an writing are affected. Lesion in the arcuate fasciculus (fibers connecting Broca’s and Wernicke’s areas).
Global aphasia
Expressive and receptive aphasia due to a lesion in the dominant perisylvian area, involving Broca’s and Wernicke’s areas. Patients have nonfluent speech, poor comprehension, poor repetition and naming.
Transcortical motor aphasia
Expressive aphasia but can repeat (unlike Broca’s aphasia). Due to a lesion in the dominant supplementary motor areas, the connections between the SMA and Broca’s area or above or anterior to Broca’s area. May be due to stroke involving watershed territory between ACA and MCA.
Transcortical sensory aphasia
Receptive aphasia but can repeat. Lesion at the junction of the temporal, parietal and occipital lobes.
Transcortical Mixed Aphasia
Nonfluent speech and poor comprehension with repetition
Alexia without agraphia
aka pure word blindness. Lesion of the medial region of the dominant occipital lobe or medial and inferior occipitotemporal regions and splenium of the corpus callosum. Can also occur with lesion of periventricular white matter around the occipital horn of the lateral ventricle in the dominant hemisphere
Alexia with agraphia
Lesion of the dominant angular gyrus
Balint’s Syndrome
Oculomotor apraxia, optic ataxia, simultagnosia and issues with depth perception.
Due to bilateral parietal-occipital damage
Auditory Verbal agnosia/pure word deafness
Can hear sounds but can’t understand words or repeat. Can read and write.
Localization: deep dominant superior temporal lesion or bilateral lesions in the midportion of the superior temporal gyrus.
Alien Limb Syndrome
Patient may not recognize their own limb or may feel can’t control it. May have involuntary movement of limb.
Due to lesion of corpus callosum, ACA infarct, basal ganglionic degeneration.
Anton’s Syndrome
Cortical blindness that patient is not aware that they have.
Lesion in bilateral occipital lobes.
Capgras Syndrome
Patients have delusion that people around them have been replaced by imposters.
Charles Bonnet Syndrome
Patients with vision loss see things in the space where vision was lost. Complex visual hallucinations, usually colored patterns, images of people, animals , plants or inanimate objects. Patients are typically elderly with normal cognition and know they are having hallucination.
Ganser Syndrome
Patients give approximate but wrong answers.
Gerstmann’s Syndrome
Finger agnosia, agraphia, acalculia, right-left confusion..
Due to a lesion of the angular gyrus of the dominant hemisphere. Brodmans’ area 39 (parietal lobe)
Common cause is infarction of the inferior division of the MCA
Kluver-Bucy Syndrome
Patients hyperoral, hypersexual and placid.
Lesion in bilateral anterioral temporal lobe.
Wernicke Encephalopathy
Confusion, ataxia, nystagmus.
Due to thiamine deficiency. Alcoholics with thiamine deficiency and get glucose before thiamine may have this triggered.
Anosodiaphoria
lack of concern about one’s impairment, specifically used in association with indifference to paralysis.
Anosognosia
denial/lack of awareness of one’s impairment; often seen with right parietal lesions.
Susac’s Syndrome
Microangiopathy involving the brain, retina, and cochlea that usually present in young women.
Susac’s syndrome is associated with a triad:
1. BRAO
2. Encephalopathy
3. Sensorineural hearing loss
Brain MRI may resemble MS. There are multiple areas of high T2 involving white and gray matter. Corpus callosum often involved
Whipple’s Disease
Due to gram positive bacillus (Tropheryma whippelii).
Dx Triad: dementia, supranuclear gaze palsy, myoclonus
Pathognomonic: oculomasticatory and skeletal myorhythmias
May also have convergence-divergence nystagmus
akinetic mutism
seen with medial frontal lesions, specifically of the anterior cingulate gyrus
asomatognosia
a category of neglect in which a patient denies ownership of a limb contralateral
to a lesion of the supramarginal gyrus of the parietal lobe (usually nondominant)
Alzheimer Genetics
Presenilin 1-chr 14 (aggressive AD)
Presenilin 2-chr 1
Apolipoprotein E4 on chr 19: modifies risk–3x higher
Amyloid Precursor Protein (APP)–chro 21
Types of Memory–Duration
Immediate–amount of info. someone can keep in conscious awareness w/o active memorization. Test with Forward Digit Span.
Working–test by manipulation of info retained in immediate memory
Recent–ability to register and recall specific items after a delay of minutes to hours–usually impaired first in AD and tested by three-word recall
Remote–historical life events and long-known info.
Types of Memory–Content
Declarative–facts and events, aka explicit
- -Episodic-specific events and contexts (most impaired early in AD)
- -Semantic–facts such as vocabulary and concepts
Nondeclarative— aka implicit; memory of skills and other acquired behaviors
Procedural
FDG-PET in Dementias
AD-bilateral parietotemporal hypometabolism
FTD-fontal and anterior temporal
Huntingtons-head of caudate
Diffuse Lewy Body–occipital
PD-no hypometabolism
PSP–global metabolic reduction in regions including the anterior cingulate, basal ganglia (esp. caudate and putamen), thalamus and upper brainstem
Orbitofrontal lesions
OCD traits, disinhibition, emotional lability, hypersexuality, anxiety, depression, impulsiveness, antisocial behavior
Familial Frontotemporal dementia genetics
17q21
chromosomes 3 and 9 also
3 variants of FTD
Behavioral Variant (most common): personality change, abulia, apathy, social withdrawal, social disinhibition, impulsivity, lack of insight, poor personal hygiene, stereotyped or ritual behaviors, hyperphagia, suddenly new artistic abilities or hobbies, emotional blunting, loss of empathy, mental rigidity, distractibility, impersistence, perseverative behavior and impaired organizational skills
Progressive nonfluent aphasia: characterized in early stages by anomia, word-finding difficulty, impaired object naming, effortful speech with preserved comprehension.
Behavior and social interaction remain unaffected until late in the disease at which point the patient becomes globally aphasic.
Semantic dementia/progressive fluent aphasia: progressive speech disturbance with normal fluency but impaired comprehension, anomia, and semantic paraphasias. May clinically resemble a transcortical sensory aphasia
Lesion Dorsomedial nucleus of the thalamus
Abulia, anterograde amnesia, social disinhibition and motivation loss.
Lesion of anterior nucleus of the thalamus
dysfunction of limbic relay and memory formation
Lesions causing akinetic mutism
Patient generally has preserved awareness with open eyes but remains immobile, mute and does not respond to commands
- bilateral globus pallidus interna
- bilateral ACA infarcts to the frontal lobes
- lesions to the medial frontal lobes
silver-staining, spherical aggregations of tau proteins in neurons
Pick bodies of Pick’s disease
globose neurofibrillary tangles in subcortical nuclei and tufted astrocytes
PSP
Memantine
low-to-moderate affinity noncompetitive NMDA receptor antagonist that inhibits glutamate stimulation and thus theoretically limits overactivation and toxicity to remaining cholinergic neurons.
Acetylcholinesterase inhibitors used in tx of AD
Donepezil: pure acetylcholinesterase inhibitor
Rivastigmine: combined acetylcholinesterase and butyrylcholinesterase antagonist–>both result in limiting break down of acetylcholine.
Galantamine: combined acetylcholinesterase inhibitor and allosteric nicotinic modulator
Pathology of Alzhemier’s disease
Amyloid plaques
intraneuronal neurofibrillary tangles
granulovacuolar degeneration: neuronal intracytoplasmic granule containing vacuoles
amyloid angiopathy: amyloid deposition in walls of small and medium sized arteries
Hirano bodies: cytoplasmic inclusions composed mainly of actin and actin-associated proteins
familial CJD
AD inheritance
prion protein gene (PRNP) coding for PrP located on chromosome 20p.
Prosopagnosia
inability to recognize faces
localize to bilateral lesion of the temporo-occipital regions (bilateral fusiform gyri}–bilateral PCA infarct or AD
Foix-Chavany-Marie Syndrome aka anterior opercular syndrome
severe dysarthria, bilateral voluntary paralysis of lower CN’s with preserved involuntary and emotional innervation.
Assoc. w/ bilateral anterior opercular syndrome
Ideational apraxia
impairment in the sequence of motions needed to carry out a specific movement (told to pour water into cup and drink but “drinks” before pouring water)
seen in patients with bifrontal or biparietal dysfunction, as occurs in neurodegenerative disorders
ideomotor apraxia
use of a body part as an object during pantomime, as well as unusual movements and postures
lesion in dominant parietal cortex, in or around the area of the superior marginal and angular gyrus.
conduction apraxia
impairment in imitation of movement
localization not well-defined
conceptual apraxia
misconception of the function of objects in the environment–using fork to eat soup
seen in diffuse neurodegenerative processes, lesions in nondominant hemisphere
disassociation apraxia
inability to execute a movement on command but normal ability to imitate
occurs in L hand in L hemisphere language dominant patients with L MCA infarct
dressing apraxia
localizes to right parietal lobe; often occurs with neglect syndrome
lesions to dorso-lateral prefrontal cortex
personality changes, lack of ability to plan or to sequence actions or tasks
DLPFC also involved in verbal and design fluency, organizational skills, problem-solving, abstract thinking, shifting sets/topics and creative reasoning
lesions to cingulate cortex
akinetic mutism, apathy and abulia, anterograde and retrograde amnesia and confabulation
Tests of Executive function
Continuous Performance Task Controlled Oral Word Association Digit vigilance test Figural fluency test Halstead category test Paced Auditory Serial Addition test Wisconsin card sorting task--patient arranges cards based on specific concept Symptol digit modalities test
Trail making tests
Part A-simple processing speed and attention
Part B–patient connects consecutive numbers and letters–set shifting and working memory in addition to Part A stuff.
Ideomotor apraxia
impaired control of skilled movements–incorrect posture when using tool but know what tool should be used for
using handle of hammer instead of head
Ideational apraxia
inability to correctly order a series of movements to perform an action, making multistep actions confusing
