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Q1=What is the general role the basal ganglia play in motor activity?
A1=* Translates the desire to move into action ^* Planning, initiation, and termination of movements, especially those with a complex cognitive dimension.
Q2=Be able to identify and label components of the basal ganglia on a diagram.
A2=* Striatum (caudate nucleus and putamen) (aka corpus striatum, neostriatum) ^* Lenticular nucleus (putamen and globus pallidus) ^* Globus pallidus (internal segment = medial segment; external = lateral) (aka pallidum) ^* Substantia nigra (pars compacta and pars reticulata) ^* Subthalamic nucleus ^* (Also part of the basal ganglia circuitry are the neocortex, ventral anterior, ventral lateral, and centromedian nuclei of the thalamus)
Q3=What are the primary afferent structures of the basal ganglia, the major sources of inputs to the afferent structures, and the neurotransmitters involved?
A3=* Striatum is primary afferent structure ^* Inputs to striatum: neocortex (except primary visual and auditory cortices); substantia nigra pars compacta; and centromedian nucleus of the thalamus ^* Neurotransmitters: neocortex and thalamus = excitatory glutamate; substantia nigra = dopamine (excitatory and inhibitory)
Q4=What is the direction of information flow through the basal ganglia?
A4=* Striatum project to internal and external globus pallidus segments and project to substantia nigra pars reticulata ^* External globus pallidus project to subthalamic nucleus ^* Subthalamic nucleus project to internal globus pallidus
Q5=What are the primary efferent structures of the basal ganglia and their neurotransmitters?
A5=* Internal segment of globus pallidus and substantia nigra pars reticulata project to centromedian, ventral anterior, and ventral lateral thalamic nuclei, which then project to the pre-central gyrus and frontal lobe. ^* Neurotransmitters: internal globus pallidus = inhibitory GABA; substantia nigra = GABA
Q6=What are major diseases of the basal ganglia and the consequent changes in basal ganglia circuitry and function?
A6=* Huntington's - bilateral degeneration of striatal neurons; hyperkinetic and eventually distonia; autosomal dominant gain-of-function mutation ^* Parkinson's - bilateral substantia nigra degeneration; hypokinetic
Q7=What are various basal ganglia diseases?
A7=o General = movement disorders ^o Hyperkinetic = involuntary, spontaneous, uncontrollable, and relatively rapid ^o Hypokinetic = inability or slowness to initiate voluntary movements; increased muscle tone, paucity of spontaneous movements ^o Distonia = spontaneous assumption of unusual fixed postures lasting seconds to minutes
Q8=What are the four basal ganglia fundamentals from Haines?
A8=o Damage to, or disorders affecting, the basal ganglia lead to movement disorders and possibly changes in cognition, perception, and mentation. ^o Anatomically and functionally segregated into parallel circuits that process different types of information. ^o Function primarily through disinhibition (release of inhibition). ^o Diseases of the basal ganglia can be described as disruptions of the neurochemical interactions between basal nuclei.
Q9=What are the definitions of the following terms:  bradykinesia, rigidity, tremor, postural instability, chorea, athetosis, ballism, dystonia, tics, and tardive disorders?
A9=* Bradykinesia - extreme slowness of movements and reflexes ^* Rigidity - resistance to passive movements (by examiner) ^* Tremor - rhythmic, regular, involuntary movement, usually of the limb ^* Postural instability - tendency to fall due to delayed proximal relfexes ^* Chorea - disease featuring involuntary, uncontrollable, purposeless movements of body and face, often with marked incoordination of the limbs ^* Athetosis - marked by continuous slow movements, expecially of the extremities; related to chorea, but with more writhing, sinuous movements ^* Ballism - extreme proximal chorea with characteristic swinging, jerking limb movements ^* Dystonia - disordered fixed postures of limbs ^* Tics - involuntary movements or vocalization characterized by brief, frequent, irregular, and purposeless contractions of several functionally related muscle groups ^* Tardive disorders - twitching of the face and tongue and involuntary motor movements of the trunk and limbs, due to prolonged use of antipsychotic drugs
Q10=What are the cardinal features of Parkinsonism?
A10=* Tremor, rigidity, bradykinesia, impaired postural reflexes (40% get dementia)
Q11=What is the primary differential diagnosis of Parkinson's disease?
A11=* Infectious: von Economo's encephalitis, a sequelae only experienced during great influenze epidemics, can cause a Parkinson-like syndrome ^* Vascular: ischemic injury to substantia nigra pars compacta ^* Drug-induced: reserpine, alpha-methyl dopa, some antipsychotics ^* MPTP use: homemade Demerol derivative was actually MPTP, which is converted to MPP+ by MAO, which leads to substantia nigra death ^* Other: benign essential tremor, progressive supranuclear palsy, multisystem atrophy, olivopontocerebellar atrophy
Q12=What are the cardinal features of essential tremor?
A12=* Postural and terminal movement (e.g. writing, holding a spoon, etc) tremor most prominent in the upper extremities ^* Begins unilaterally, eventually bilateral because it gets progressively worse ^* PET shows overly-excited cerebellum ^* Treatment: beta-blockers, anticonvulsants, surgery to destroy the VIM thalamus to destroy connection to cerebellum
Q13=What are the cardinal features of Huntington's disease?
A13=* Personality change, chorea, dementia, dysarthria, abnormal eye movements, athetosis, dystonia, rigidity
Q14=What are the cardinal features of Wilson's disease?
A14=* Dystonic hands and face, tremor, dysarthria, rigidity ^* Young person with dystonia has WD unless proven otherwise
Q15=What are the cardinal features of Ballism?
A15=* Involuntary movement affecting the proximal limb musculature, manifested as jerking, flinging movements of the extremity ^* Usually one-sided = hemiballismus
Q16=What are the cardinal features of Tourette's syndrome?
A16=* Multiple motor and/or phonic tics which fluctuate in type and frequency, present for more than one year ^* Tic types: face > head/shoulder > arm >> leg/trunk
Q17=What are the cardinal features of dystonias?
A17=* Fixed postures of limbs or muscle groups ^* Types: generalized - childhood onset; focal/segmental - adult onset
Q18=What are the cardinal features of tardive disorders?
A18=* "the great mimic" ^* dyskinesia (usually oral/buccal - chewing, tongue thrusting), chorea, dystonia, tics, akathisia (Stedman's - akathisia = syndrombe characterized by an inability to remain in a sitting posture, with motor restlessness and a feeling of muscular quivering) ^* caused by long-term neuroleptics; limit dosing to prevent, can't do much when they start
Q19=What are the pathologies of Ballism and Parkinson's, Huntington's, and Wilson's diseases?
A19=* Ballism - caused by a lesion of, or near, the contralateral subthalamic nucleus ^* Parkinson's - substantia nigra pars compacta neuronal cell death (<50% = symptomatic) ^* Huntington's - degeneration of the striatum (interneurons not affected); striatum to globus pallidus externa is degenerated ^* Wilson's - copper transport disease causing liver cirrhosis, hemolytic anemia, renal disease, and the optic Kayser-Fleischer ring
Q20=What are the genetics of Huntington's disease, Wilson's disease, and generalized dystonia?
A20=* Huntington's - trinucleotide CAG repeat (Gln) in translated region ^* Wilson's disease - 13q recessive ^* General dystonia (full-body) - 9q32-34
Q21=What are the basic drugs used to treat Parkinson's disease and the rationale for their use?
A21=* Levodopa (l-dopa) - dopamine is not being provided by the nigrastriatal pathway, so supply l-dopa, which is converted to dopamine (dopamine not given directly because it can't cross the blood-brain barrier) ^* Pergolide and bromocriptine - dopamine receptor agonists ^* Amantadine - glutamate antagonist ^* Benztropine, trihexyphenidyl, diphenhydramine - dopamine re-uptake inhibitor ^* Tolcapone, entacapone - COMT inhibitor prevents degradation ^* Carbidopa - AAAD inhibitor (doesn't cross blood-brain barrier) prevents degradation ^* Deprenyl - MAO B inhibitor
Q22=What are the main mechanism of action and key usage of the anti-Parkinson drugs?
A22=* Covered in previous objective ?
Q23=What are the important side effects of the anti-Parkinson's drugs, especially early-occurring and late-occurring effects of levodopa and dopamine agonists?
A23=* Levodopa - early = anorexia, nausea, vomiting, orthostatic hypotension (tolerance develops); late = dyskinesia (abnormal involuntary movements, esp. oral/facial; possibly due to changing dosing regimine) and psychiatric (hallucinations and confusion, esp. when not eating) ^* Pergolide - dyskinesias, decreased prolactin, nausea, mental changes, hallucination, orthostatic hypotension ^* Tolcapone - nausea, dyskinesia, diarrhea, abdominal pain, constipation ^* Benztropine - constipation, urinary retention, blurred vision
Q24=How is levodopa absorbed and metabolized?
A24=* Absorbed by transport system for aromatic amino acids ^* Absorption affected by rate of gastric emptying (direct proportion) and gastric pH ^* Metabolized in GI tract, liver, and brain by L-aromatic amino acid decarboxylase (AAAD) and COMT
Q25=What drugs are contraindicated in the treatment of Parkinson's disease and why?
A25=* Antipsychotic drugs (block dopamine receptors and worsen Parkinson's) ^* Antiemetic drugs (some are antipsychotic drugs) ^* Monoamine oxidase A inhibitors (accentuates peripheral actions of l-dopa and may cause life-threatening hypertensive crises) ^* Reserpine - depletes dopamine in striatum ^* MPTP is metabolized to MPP by MAO B, which is a toxic to substantia nigra
Q26=What is the required time course and location of lesions necessary to make a diagnosis of Multiple Sclerosis?
A26=* At least two symptomatic lesions in the CNS separated in space and time
Q27=What are the three diagnostic tests most useful in Multiple Sclerosis and in which situations are these tests most useful?
A27=* MRI (magnetic resonance imaging) - primary exam; high sensitivity for plaques ^* EP (evoked potential) - assess visual pathways, brainstem transmission, or spinal cord conduction; used to verify lesions ^* LP (lumbar puncture) - demonstrate oligoclonal inflammatory response in CNS (demonstrate elevated IgG)
Q28=What are the pathological findings in Multiple Sclerosis and progressive multifocal leukoencephalopathy?
A28=* Multiple sclerosis ^o Gross - sclerotic plaques in white matter of periventricular regions, optic nerves, brainstem, cerebellum, and spinal cord; proceeds to atrophy ^o Microscopic - oligodendroglial cell degeneration, astrocyte proliferation, perivenular inflammation (immune system reaction against myelin); axonal preservation; no peripheral myelin or Schwann cell damage ^* Progressive multifocal leukoencephalopathy ^o Viral (papova virus) inclusion bodies in oligodendroglial cells and asymmetric cystic demyelination ^o Parietal and occipital lobes most affected, with occasional involvement of cerebellum and brainstem
Q29=What are the three most common clinical presentations of Multiple Sclerosis?
A29=* Intranuclear opthalmoplegia - IMPORTANT; fairly specific to MS; nystagmus in abducting eye and failure of concomitant adduction ^* Optic neuritis - subacute vision loss; 75% recover; progressive ^* Transverse myelitis - subacute inflammatory myelopathy (sensory changes, bladder problems, paraparesis, and spasticity); possibly asymmetric
Q30=What three agents are useful for symptomatic management of Multiple Sclerosis?
A30=* Antispasmodic - lioresal (BACLOFEN), benzodiazepines, and tizanidine ^* Anti-fatigue - amantadine ^* Anti-depressant - prozac ^* Urinary frequency/urgency - Ditropan, lioresal, tricyclics
Q31=What are two currently proposed or used treatments that may change the course of Multiple Sclerosis?
A31=* Beta interferon (1a = AVONEX; 1b = BETASERON) ^* Glatiramer acetate (COPAXONE) ^* Mitoxantrone ^* IVIg
Q32=How are inherited and acquired demyelinating diseases different in terms of pathology and clinical presentation?
A32=* Aquired ^o Acute disseminated encephalomyelitis - {seen after immunization or viral infection}; symptoms = rapid onset, lethargy, coma; perivascular inflammation, vascular necrosis, surrounding demyelination of brain and spinal cord ^o Progressive multifocal leukoencephalopathy - {seen with immunocompromised}; viral (papova virus) inclusion bodies in oligodendroglial cells and asymmetric cystic demyelination; symptoms = dementia, weakness, ataxia (motor findings often unilateral) ^* Inherited ^o Adrenoleukodystrophy - X-linked peroxisomal disorder leading to accumulation of very long chain fatty acids; 4-8 years old; diffuse white matter and some peripheral demyelination; symptoms = personality changes, abnormal spastic gait, cortical blindness and deafness ^o Metachromatic leukodystrophy - deficiency of arylsulfatase A; symptoms = regression of motor milestones, dementia, optic atrophy; accumulation of sulfated lipids ^o Krabbe's disease - glactosyl-ceraminidase deficiency; accumulation of lipids in white matter macrophages; symptoms = progressive spasticity, dementia, extensor posturing, seizures; peripheral myelin may be affected; infantile form more severe than later form ^o Pelizaeus Merzbacher - aka sudanophilic leukodystrophy; symptoms = spasticity, intellectual regression in toddlers; fatty acid components of central myelin are deficient
Q33=What are gross and microscopic changes in the appearance of the brain in patients with hypoxia, poisoning, metabolic and nutritional disorders, alcoholism, multiple sclerosis, and viral, bacterial, and fungal diseases?
A33= 
Q34=What is selective vulnerability in the brain and why does it tend to produce symmetric lesions?
A34=* Selective vulnerability is due to certain cells or neuroanatomic regions being more sensitive to toxic agents or to metabolic abnormalities than other regions. ^* Symmetry is due to global effects on a bilaterally symmetric organ.
Q35=Which cells and neuroanatomic structures are vulnerable to specific abnormalities and what are those abnormalities? (specific abnormality - cell most affected; gross region affected)
A35=* Toxic/metabolic ^o Anoxia - large pyramidal and purkinje neurons; Sommer's section of hippocampus, cerebral cortex, cerebellum ^o Severe hypoglycemia - large pyramidal neurons; Sommer's section of hippocampus, cerebral cortex ^o Carbon monoxide - neurons; globus pallidus (THINK PINK) ^o Vitamin B1 deficiency - vessels and glia; mamillary bodies, periaqueductal grey (ALCOHOL, WERNICKE'S, AND LEIGH'S ENCEPHALOPATHY) ^o Hepatic encephalopathy - astrocytes; deep grey matter ^o Methyl mercury - granular neurons; cerebellum ^o Thallium - long axons; dorsal columns of cord, PNS ^o Lead - capillary endothelium; whole brain edema (BBB) ^* Toxic/metabolic and demyelinating ^o Lead - myelin; PNS ^o Triethyl-tin - myelin; multiple bilateral white matter regions ^o Vitamin B12 deficiency - myelin; corticospinal and dorsal columns of cord (subacute combined degeneration; also presents with pernicious anemia) ^o Central pontine myelinolysis - myelin; central pons (vacuolar demyelination; associated with overcorrection of hyponatremia) ^o Adrenoleukodystrophy - myelin, adrenal, and gonadal cells; multiple bilateral white matter regions and bilateral adrenals (Xq28) ^o Metachromatic leukodystrophy - myelin; multiple bilateral white matter regions (arylsulfatase on chr 22) ^o Krabbe's disease - myelin (globoid cells); multiple bilateral white matter regions (aka globoid cell leukodystrophy; high cerebroside levels; 14q31) ^* Demyelinating ^o Multiple sclerosis - myelin; disregard for anatomical boundaries (STRESSED ASYMMETRY; more common in northern north America than southern) ^o Allergic encephalomyelitis - myelin; perivenous white matter (*perivenous demyelination) ^o Progressive multifocal leukoencephalopathy - myelin; deep cerebral white matter; disregard for anatomic boundaries
Q36=What are the different manifestations of infectious diseases in the nervous system and the associated organisms?
A36=* Encephalitis ^o Viral - herpes simplex = frontal and temporal lobes; targets neurons ^o Prions - Creutzfeldt-Jakob disease (spongiform encephalopathy) ^o Bacteria - invasion with trauma, sinusitis, or sepsis; frequently focal and suppurative ^* Meningitis ^o Bacteria - typically involves subarachnoid space, but can be subdural; Eschericia coli in newborns, Haemophilus influenza in children, and meningococcus and pneumococcus in adolescents and adults; fever, malaise, headaches, and stiff neck ^* Abscess ^o Chronic and focal bacterial infection in brain; rare situation where significant collagen deposition occurs in brain; symptoms include space-filling lesions in CNS, headache, drowsiness, coma ^* Granulomas and granulomatous meningitis ^o Caused by fungi, mycobacterium, or sarcoid; possible brain tissue involvement, but more likely meningeal, especially at the base of the brain; hyphal fungi like Mucor and Aspergillus can invade vascular walls leading to infarct and hemorrhage
Q37=What are the various categories of diseases based on their targets in the brain and body and their pattern of lesions?
A37=* Toxic/metabolic - neurons, vessels, glia ^* Toxic/metabolic and demyelinating - astrocytes ^* Demyelinating - astrocytes
Q38=What is the classic triad for basal ganglia destruction?
A38=* Carbon monoxide = globus pallidus ^* Wilson's disease = putamen ^* Huntington's disease = caudate nucleus
Q39=What are the limbic lobe and the limbic system?
A39=* Lobe = anatomic entity ^* System = bilateral system with three functional divisions, all which are reciprocally connected
Q40=What are the components of the limbic system?
A40=* Limbic forebrain ^o Limbic lobe = subcallosal area (rostral) => cingulate gyrus => parahippocampal gyrus (caudal) ^o Orbital frontal cortex (OFC; frontal cortex just superior to roof of orbit) ^o Hippocampus ^o Amygdala nucleus ^o Olfactory structures ^o Anteriomedial thalamus nuclear groups ^o Ventral striatum (poorly understood basal ganglia; nucleus accumbens) ^* Limbic brainstem (aka reticular formation) ^* Preoptic area (POA) - hypothalamus
Q41=What are the structural and functional relationships between the limbic system, the cerebral cortex, and the visceroendocrine periphery?
A41=* Control primarily from hypothalamus and preoptic area (POA) ^* Somatic and visceral functions are controlled via tracts through reticular formation, not directly ^* Hypothalamus connects to pituitary gland ^* Limbic system mediates associations from cerebral cortex (e.g. primary sensory cortex projects to association cortex which projects to limbic system, which will mediate associative memories that underlie motivational states and emotional expression)
Q42=Where is the hippocampal formation and what are the important associated connections?
A42=* Location - deep to parahippocampal gyrus; part of wall of inferior horn of lateral ventricle; behind amygdala and extends back to below the splenium of the corpus callosum (vestigal hippocampal formation overlies the corpus callosum) ^* Hippocampal formation = dentate gyrus + hippocampus proper + subiculum ^* Connections - olfactory and associative cortex information => entorhinal cortex => dentate gyrus => hippocampus proper => subiculum => fibria (part of fornix)
Q43=What are the central connections of the olfactory system, particularly the extrathalamic projections to limbic structures?
A43=* Lateral stria of olfactory tract sends connections to primary olfactory cortex at the end of the anteromedial temporal lobe (odor detection, not discrimination) and to dorsal medial nucleus of thalamus, which then proceed to the orbital frontal cortex (limbic system; odor discrimination) ^Lateral stria of olfactory tract also sends branches to amygdala (limbic system) and entorhinal cortex (limbic system), both of which send branches into the preoptic area-hypothalamus (limbic system)
Q44=Where is the amygdaloid nucleus and what are the important associated connections?
A44=* Rostral to hippocampus ^* Two nuclei - corticomedial nucleus (visceral input; taste and olfaction) and basolateral nucleus (somatic input from association areas) ^* Circuits ^o Amygdalofugal bundal - both amygdala nuclei contribute fibers; sends fibers to hypothalamus and ventral striatum ^o Stria terminalis - main connection from corticomedial amaygdala to the hypothalamus and preoptic area ^o (unnamed) - primary outflow tract from basolateral amygdala sends branches to the dorsalmedial thalamus and the orbital frontal center
Q45=What are the anatomical boundaries of the hypothalamus?
A45=* Preoptic area is anterior ^* Hypothalamic sulcus is superior ^* Ventral thalamus is lateral boundary ^* Third ventricle is medial boundary ^* Line between mammillary body (protuberance of ventral thalamus) and the posterior commissure forms the posterior boundary ^* Neurohypophesis is inferior projection of hypothalamus
Q46=What are the important features about the development of the hypothalamus and its relationship to the other major components of the diencephalon?
A46=???
Q47=What are the major nuclear groups located within the medial and lateral areas of the hypothalamus?
A47=* most nuclei are in medial area - specifics not tested ^* most fibers are in lateral area - specifics not tested
Q48=What are the afferent and efferent connections of the hypothalamus?
A48=* Afferent - posterior input via reticular formation of brainstem; superior via hippocampal amygdala; anterior via limbic forebrain; retina ^* Efferent - neurohormonal output to pituitary; brainstem outflow to reticular formation; anterior thalamus
Q49=What role does the hypothalamus play in the functional hierarchy of the limbic system?
A49=* Control center for the integration of visceral, somatic, and neuroendocrine function; combines autonomic action with appropriate action of the somatic nervous system (e.g. rage reaction producing increased heart and respiratory rates, generalized vasoconstriction, and increased skeletal muscle tone)
Q50=What are the important features about the neocortex cellular anatomy?
A50=* Six layers (1 = superficial; 6 = deep) ^* Two principle neuron types: granule (aka stellate; local circuit) and pyramidal (projection to subcortical and other cortical areas) ^* For sensory cortices, thalamic afferent axons innervate the 4th layer ^* Layers 3 and 5 contain pyramidal neurons and are the primary output layers
Q51=What is the organization of a cortical column and its importance?
A51=* All neurons in a column receive information from the same receptive field
Q52=What are the locations of the primary sensory cortices (auditory, visual, somatosensory, olfactory, gustatory)?
A52=* Auditory - transverse temporal gyrus (temporal lobe) ^* Visual - superior and inferior lips of the calcarine sulcus (occipital lobe) ^* Somatosensory - postcentral gyrus (parietal lobe) ^* Olfactory - frontal lobe ^* Gustatory - 
Q53=What is the laterality of the various sensory representations?
A53=* Ipsilateral = olfaction ^* Contralateral = somatosensory (except head) and vision ^* Bilateral = audition, somatosensory (head), and taste
Q54=What is topographic organization and why is it important?
A54=* Relationship of body points (sensory and motor) to regions of brain ^* Functional organization is conserved between people
Q55=What are the spatial and functional relations of the association and primary cortices?
A55=* Association cortices surround primary cortices and heavily interconnect ^* Association cortex types ^o Unimodal - directly connected to local primary cortex ^o Multimodal - receives input from unimodal areas; integrative cortex
Q56=How do projection fibers, association fibers, and commissural fibers relate to the cerebral cortex, corona radiata, corpus callosum, and internal capsule?
A56=* Projection fibers - from pyramidal cells (layer 5) to subcortical structures (thalamus, brainstem, spinal cord) through internal capsule; in transit, known as corona radiata ^* Association fibers - interconnect different areas within a hemisphere; short = adjacent sulci, long = distant sulci/lobes; responsible for parallel, reciprocal, and hierarchical organization of cerebral cortex ^* Commissural fibers - from pyramidal cells (layer 3); connect two hemispheres; corpus callosum (genu {ant} => body => spelnium {post}) and anterior commissure are two connecting tracts; connect homologous and heterologous regions
Q57=What are the location and function of the arcuate and superior longitudinal fasciculi?
A57=* Arcuate fasciculus - interconnects Broca's area (frontal) with Wernicke's area (parietal); motor and perceptual aspects of speech ^* Superior longitudinal fasciculus - occipital and frontal lobe connection
Q58=What is meant by cerebral dominance of cortical functions?
A58=* Numerous functions are performed by cortical areas unilaterally (left hemisphere is predominantly used for speech)
Q59=What are the definitions of aphasia, apraxia, and other deficits of higher cortical function?
A59=* Aphasia - disorder of language production or reception ^* Apraxia - disorder in the execution of learned, skilled motor tasks ^* Amnesia - disorder of memory ^* Alexia - impaired reading ^* Agraphia - impaired writing ^* Anomia - difficulty naming common objects ^* Aprosody - loss of melody and intonation in language (i.e. emotional aspect of language) ^* Agrammatism - omission of words such as "and", "the", "by" ^* Anarthia - disorder of speech production; non-language disorder ^* Agnosia - inability to perceive or understand a primary sensory modality ^* Anosagnosia - lack of awareness of illness or deficit
Q60=How can aphasia be characterized based on testing naming, fluency, comprehension, repetition, reading, and writing?
A60=* Spontaneous speech - articulation, fluency, circumlocutions (e.g. watch = "thing you tell time with"), paraphasic errors (e.g. pog for dog, cat for dog), automatic utterances ^* Naming - controntation naming (e.g. parts of an object, objects, body parts, colors), series naming (e.g. days of week) ^* Auditory comprehension - complex grammatical constructions, commands, yes/no questions ^* Repetition - complex grammatical phrases (e.g. "There are no if's, and's, or's, or but's"), polysyllabic words (e.g. episcopal); single syllable words ^* Reading - reading comprehension, reading aloud ^* Writing - spontaneous writing, writing to dictation, copying
Q61=What are the characteristics of the following types of aphasia:  anomic, motor/Broca's, sensory/Wernicke's, conduction, transcortical motor, transcortical sensory, mixed transcortical, and global.
A61=* Impaired repetition ^o More common (out of impaired and intact repetition) ^o Broca's aphasia - speech is slow, halting, and produced with great effort; short and agrammatic sentences; frustrated patients ^o Wernicke's aphasia - abundant language production; frequent paraphrasic errors ("word salad"); little awareness of, or concern for, the speech difficulty ^o Global aphasia - slow, halting speech without response to conversion; almost always accompanied by hemiplegia unless caused by separate lesions hitting both Broca's and Wernicke's areas ^* Intact repetition ^o All are transcortical aphasias; difficulty with naming; usually cortical watershed infarcts ^o Transcortical motor - nonfluent speech ^o Transcortical sensory - impaired comprehension ^o Transcortical mixed - nonfluent speech and impaired comprehension ^o Anomic - Alzheimer's; fluent speech and normal comprehension
Q62=How are aphasic and apraxic deficits localized?
A62=* Identify if there is a "handedness" to the disorder (e.g. salute with left and right hands)
Q63=Be able to use clinical vignettes and videos of patients with aphasia be used to examine, characterize, and provide syndromic and anatomic diagnoses?
A63= 
Q64=What are seizures and epilepsy?
A64=* Seizure - clinical manifestation of excessive, and/or hypersynchronous, usually self-limited, abnormal activity of neurons in the cerebral cortex ^* Epilepsy - recurrent seizures
Q65=What are the different types of seizures and epilepsy?
A65=* Seizure ^o Reactive - self-limited and only present when noxious stimulus is present ^o Unprovoked - occurs without a known noxious stimulus ^* Epilepsy ^o Generalized nonconvulsive ^* Absence - childhood; unresponsive blank stare; lasts seconds; without aura; abrupt start/stop; ppt'd with hypervent; EEG 3Hz ^* Atypical absence - unresponsive blank stare; gradual start/stop; developmentally delayed children; EEG < 2Hz ^* Tonic - mentally retarded; brief; associated with falls; abrupt tonic limb extension or elevation, abduction and tonic flexion of upper extremities ^* Atonic - mentally retarded; brief; associated with falls; sudden loss of muscle tone in all four extremities, resulting in falls ^o Generalized convulsive ^* Clonic - lose muscle tone, many events; loss of consciousness ^* Myoclonic - singular events; no loss of consciousness; high amplitude; rapid lightning-like jerks causing elevation/flexion of the upper limbs at the shoulder and elbow ^* Tonic-clonic - (aka grand mal); cry => small clonic => moderate clonic => large clonic
Q66=How can epileptic syndromes be classified?
A66=* Localization-related epilepsies ^o Aka partial epilepsies ^o Idiopathic partial epilepsies (genetic) ^* Benign rolandic epilepsy ^* Benign childhood epilepsy with occipital paroysms ^* Familial temporal lobe epilepsy ^* Autosomal dominant nocturnal frontal lobe epilepsy ^o Symptomatic/cryptogenic partial epilepsy ^* Temporal lobe epilepsy ^* Frontal lobe epilepsy ^* Parietal lobe epilepsy ^* Occipital lobe epilepsy ^* Generalized epilepsies ^o Idiopathic ^* Childhood absence epilepsy ^* Juvenile absence epilepsy ^* Juvenile myoclonic epilepsy ^* Epilepsy with grand mal seizures on awakening ^* Benign neonatal familial convulsions ^* Benign myoclonic epilepsy in infancy ^* Epilepsy with seizures precipitated by specific modes of activation - reading epilepsy ^o Symptomatic/cryptogenic ^* Infantile spasms ^* Lennox Gastaut syndrome ^* Epilepsy with myoclonic absences ^* Epilepsy with myoclonic astatic seizures ^* Undetermined epilepsies ^o Landau-Kleffner syndrome ^o Epilepsy with continuous spike waves during slow wave sleep ^o Severe myoclonic epilepsy in infancy
Q67=When should treatment be initiated for seizures?
A67=* With single unprovoked seizure, do not treat, because most will not recur ^* Remote symptomatic causes have a higher recurrence risk, but may also just be observed if MRI and EEG are normal ^* Patients with a remote cause and a history of acute symptomatic seizures should be treated, because they have a 100% recurrence rate ^* Because there is a 20% chance that patients will be refractory to medical treatment, those patients should be considered for surgery
Q68=What are the appropriate anti-epileptic drugs for different types of seizures/epilepsies?
A68=* Partial epilepsy - male = phenytoin (hirsutism); female or adolescent = carbamazepine ^* Avoid valproate in pregnant women and individuals with family history of spina bifida ^* Primary generalized (idiopathic) - valproate ^* Partial seizures and generalized tonic-clonic - topiramate is an add-on ^* Partial seizures - neurontin is an add-on
Q69=What is convulsive status epilepticus and how is it treated?
A69=* Status epilepticus - 1) continuous seizure lasting >30 minutes; or 2) two or more sequential seizures within 30 minutes ^* Treat acutely with lorazepam (rapid onset, but short half-life) first and then phenytoin (slow onset, long half-life) in saline (not D5W, because precipitates) ^* Fosphenytoin is a good replacement for phenytoin because it is less alkaline
Q70=What is the general basis for choosing a particular anticonvulsant for initiating epilepsy therapy?
A70=* Selection based mainly on seizure type, not cause
Q71=What generally is the main difference between a first-line and second-choice anticonvulsant when either drug would be suitable for the patient, but one is generally preferred to start treatment?
A71=* A drug is generally first line because of better relative safety, not necessarily greater efficacy
Q72=What is the general value of, or needs for, monitoring anticonvulsant drug serum levels?
A72=* Checking serum levels allows monitoring for noncompliance (no drug in serum, patient isn't taking meds)
Q73=In the context of long-term drug treatment of epilepsy, what is the most frequent cause of apparent "lack of efficacy?"
A73=* Noncompliance
Q74=What brain neurotransmitter seems to be the "target" of anticonvulsant action, in one way or another, depending on the anticonvulsant?
A74=* Gamma-aminobutyric acid
Q75=What are the normally preferred first and second drug(s) for generalized tonic-clonic seizures, absence seizures, and partial/focal seizures?
A75=* Tonic-clonic seizures - 1 = phenytoin; 2 = phenobarbital ^* Absence seizures - 1 = ethosuximide; 2 = valproic acid ^* Partial/focal seizures - 1 = phenytoin; 2 = carbamazepine
Q76=What are the most serious toxicities or adverse responses, as well as the more common side effects, of phenytoin, carbamazepine, phenobarbitol, valproic acid, ethosuximide, and clonazepam?
A76=* Phenytoin - extensive gingival hyperplasia (UNIQUE); aggrevates absence seizures; CNS (ataxia, vertigo, diplopia, nystagmus); hematologic (folate, vit K def) ^* Carbamazepine - agranulocytosis, aplastic anemia (UNIQUE); dizziness, GI upset; vit K dependent bleeding ^* Phenobarbitol - sedation; vit K dependent bleeding; tolerance is common ^* Valproic acid - GI distress, weight gain (appetite stimulation); hepatotoxicity (IMPORTANT); alopecia (UNIQUE); teratogenic (IMPORTANT); thrombocytopenia ^* Ethosuximide - headache; dizziness; GI distress ^* Clonazepam - sedation; tolerance; seizure exacerbation when drug discontinued
Q77=What anticonvulsant(s) is/are important for long-term treatment of "metabolic autoinduction?"
A77=* Phenytoin and carbamazepine are autoinducers
Q78=Which three anticonvulsants are hepatic inducers and which is a hepatic inhibitor?
A78=* Hepatic inducers: phenytoin, carbamazepine, phenobarbitol (barbiturates, OCP's, rifampin, warfarin, theophyline) ^* Hepatic inhibitor: valproic acid (cimetidine)
Q79=What is the most common mechanism of interactions between common anticonvulsants (e.g. phenytoin and barbiturates, primidone, valproic acid, carbamazepine)?
A79=* Hepatic induction
Q80=What is currently the "most often recommended" pharmacologic approach to managing status epilepticus?
A80=* Initially IV benzodiazepine (e.g. lorazepam (ATIVAN)) ^* Follow-up with IV phenytoin (DILANTIN)
Q81=What are the benefits and limitations of using IV benzodiazepine or phenytoin alone for treating status epilepticus?
A81=* Benzodiazepine - fast-acting, so effects gone quickly, allowing recurrence ^* Phenytoin - enters brain slowly, so delayed onset; very alkaline, so irritating to veins (overcome with newer fos-phenytoin)
Q82=What precautions must be taken, for both the mother and newborn, when phenytoin or phenobarbital is administered to pregnant women?
A82=* Vitamin K and folate supplementation
Q83=What region of the body is the main source of behavior?
A83=* Brain
Q84=How was the fact that the brain is the source of behavior first determined and what techniques have been used to establish brain-behavior linkages?
A84=* First established with naturally-occurring lesions (e.g. war injuries) ^* Other - surgical lesions; electrical stimulation studies; neuropsychological test batteries; biochemical measures (e.g. CSF); neuroendocrine challenge tests; psychophysiological measures (e.g. EEG); brain imaging (e.g. CT, MRI, PET); genetic studies (e.g. gene expression or knock-outs)
Q85=What are behavior and how does abnormal behavior develop?
A85=* Normal - the "final common manifestation" of perceptions, cognitive processes, affective regulation, and behavioral outputs. ^* Abnormal - dysregulation of any of the aspects of normal behavior.
Q86=How are the various behavioral functions (e.g. perception, cognition, affect/mood, and behavior) mediated and regulated in the brain?
A86=* Mediated - various types of message transmission (e.g. electrical and neurochemical) ^* Regulated - genetic influences on neurotransmitter storage and release
Q87=What is the limbic system, its important components, and the normal behaviors regulated by its components?
A87=* Limbic system - collection of nuclei and tracts that are intimately related to behavioral expression in animals and man ^* Components - amygdala, hippocampus, medial forebrain bundle, septal regions, hypothalamus, and anterior cingulate cortex ^* Behaviors - pain/pleasure; reward/reinforcement; aggression; fear; appetite/satiety; taste; autonomic regulation; chronobiological rhythm; sleep; libido; short-term memory/learning; concentration; psychomotor regulation; prosody; separation/bereavement; maternal behavior
Q88=Are limbic networks stable or plastic and why is this important?
A88=* As with other parts of the brain, the limbic networks are plastic and consequently constantly influenced and modified by developmental, psychological, and social experiences.
Q89=Why is the "mind-body" dichotomy outdated and unscientific?
A89=* Because the brain is plastic and...