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NEURO CC Final Review
Clincally-relevant stuff
76
Other
Not Applicable
05/17/2008

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Term

 

 

Hirschsprung's disease/megacolon

Definition
Cause: failure of NC migration=lack of ENS ctrl.
Sx: dilation, hypertrophy, constipation/fecal retention 
Term

 

 

Familial dysautonomia (Riley-Day syndrome) 

Definition
Cause: (AR, Jews only, 1:3700) loss of ANS neurons in sensory ganglia (periphery)
 
Sx: abnormal sweating, BP instability, poor muscle tone in GI tract, sensory loss
Term

 

 

Horner's syndrome 

Definition
Causes: SNS dysfunction somewhere btw. brainstem and carotid plexus; Pancoast's tumor, thyroid carcinoma/goitre, carotid or aortic dissection, MS, Lateral Medullary Syndrome, basically anything involving BS, nerve plexi from T2 to eyes...
 
Sx:
1. miosis
2. ptosis
3. anhidrosis
 
DDx: no #3=SNS dysfxn somewhere btw. carotid plexus and eye/target=ICA thrombosis
Why: SNS to eye wraps around ICA, SNS to facial sweat glands follows External carotid a.
Term

 

 

Autonomic Dysreflexia 

Definition
Cause: partial or complete spinal cord injury=loss of descending (CNS) inhibitory ctrl., altered adrenergicR sensitivity
 
Sx: BP dysregulation (life-threatening), abnormal CV control triggered by noxious (pain, etc.) OR non-noxious stimuli (bladder distension, etc.)
Term

 

 

Lateral vs. medial

 

hypothalamic lesions 

Definition
Lateral=anorexia, weight loss
Medial=obesity, weight gain
 
Note: Prader-Willi (15q deletion, 1:12,000 RARE) causes excessive, chronic hunger +LDs, small stature)
 
Caveat: in genetics, parental imprinting causes PWS when gene is inherited from FATHER, inherited from mother=Angelman (smiley-overly affectionate, LDs, fascination with water (ha), not very verbal, walk like a puppet
Term

 

 

Poikilothermia vs. hyperthermia

 

(hypothalamic lesions) 

 

Definition
Anterior nucleus BILATERAL lesion=hyperthermia
 
Posterior nuc. BILAT=poikilothermia (inability to regulate body temp. at all; anterior nucleus fibers run thru posterior nucleus)
 
Caveat: arcuate nucleus contains key feeding-related neurons (acted upon by leptin and other hormones)
1. AgRP/NPY: increase feeding
2. POMC/CART: decrease feeding 
Term

 

 

Craniopharyngioma 

Definition
Cause: congenital tumor (often calcified) arising from Rathke's pouch (ectoderm diverticulum creating adenohypophysis)
 
Note: most common supratentorial tumor in children
 
Sx:
1. optic chiasm pressure=bilateral hemianopsia
2. pressure on hypo=hypothalamic syndrome:
 
1. DI, obesity/adiposity, somnolence (DDx: OSA), temperature regulation disturbances (seems to be a hallmark of hypothalamic dysfunction)
 
Term

 

 

Kluver-Busy syndrome (KBS)

Definition
Cause: medial temporal (rhinencephalon esp.), amygdala lesions (bilateral)
 
Etiology: trauma (infarcs, anoxia, status epilepticus), Herpes encephalitis, Pick's, Alzhiemer's, subarachnoid hemorrhage
 
Sx:
1. increased oral activity
2. HYPERsexuality
3. hypermetamorphosis: react to/fascination w/ everything one sees
4. Placidity: flat affect, lack of fear & aggression
5. Visual agnosia: inability to recog. familiar objects
6. Bulimia 
Term

 

 

Geschwind Syndrome 

Definition
Cause: (medial) temporal lobe SEIZURE (hyperexcitability)
 
Sx:
1. Philosophical, cosmic preoccupations
2. Altered sexuality (usually HYPO)
3. Hypergraphia: writes a lot (cosmic stuff)
4. Viscosity: interpersonal clingyness/talkativeness, otherwise known as Interictal personality
Term

 

 

Cocaine and heroin:

 

effects on mesolimbic system 

Definition
Cocaine=inhibits DA reuptake
Heroin=increases dopaminergic neuron activity/firing 
 
Compare: amphetamines increase extracellular DA, NE and 5-HT by inhibiting reuptake and inducing vesicular emptying into synapses
 
Mesolimbic system: dopaminergic pleasure/motivation center of CNS
Term

Lesion/stimulation/tumor studies:

1.  Septal nuclei/area

2. Amygdala

3. Hippocampus

4. Cingulate 

 

Definition
1. Septal lesion=
a. Lesion=re-established normal sexuality in cat who was already hypersexual due to amygdala lesion (see: KBS)
b. Stimulation=hypersexuality
c. Lesion=lowered sexuality, more sociability
d. tumor=rage, irritability
...seems like septum is a positive ctrl. for SEX
 
2. Amygdala
Lesion=HYPOemotionality, social isolation (FXN=memory-emotional integration) 
 
3. Hippo
Lesion=declarative memory (facts) amnesia 
 
Caveat: episodic memory (events) + semantic (facts)=declarative/explicit memory
 
Implicit memory=procedural, priming, assoc. learning (behav & operatant conditioning, non-assoc. learning (habits, etc.))
 
4. Cingulate
Lesion=hypersexuality (disinhibition), OCD, anxiety, depression, bulimia, placidity, apathy
 
Caveat: very similar to KBS (except for bolded sx) b/c AC is W/IN the medial temporal lobe (lesioned in KBS).
Term
Clinical applications of cingulate gyrus fxn:
1. Pain
2. OCD
Definition
1. Decreased chronic pain
2. Cingulotomy has been used to treat OCD
Term
 Other Sx of Cingulate lesions/dysfunction 
 
1. Sociopathy correlation
 
2. Bilateral AC+OFC lesion=sx? 
Definition
1. Sociopathy: reduced emotional responses
 
2. (Bilateral AC + OFC lesions): reduced judgement and common sense with preserved memory, IQ.  Reduced ability to read and adapt to social cues 
Term

 

 

Akinetic mutism 

Definition
Cause: Anterior cingulate lesion
 
Sx: tending not to move (akinesis) or speak (mutism) 
Term

 

 

(Gilles de la) Tourette syndrome (GTS)

Definition
Cause: anterior cingulate decreased activity (decreased utilization of glucose in AC and insula)
 
Remember: AC=emotion, motor (PC=memory, visuospatial tasks)
 
Sx:
1. affective vocalizations (emotion + motor)
2. complex coordinated movements (tics) (motor)
3. OCD (emotion) 
 
Tx: disconnect (surgical) AC from Thalamus
 
Term

 

 

Age-Associated Memory Impairment 

Definition
Cause: +50 y/o who score >1 sd below mean for memory task performance and complain of memory problems
 
Caveat: memory STARTS to decline at 18 y/o; at 70-80, performance is only 50% what it was at 18
Term
Compare: cortical vs. subcortical dementia
1. retrieval vs. encoding of memory
2. explicit vs. implicit memory functioning
3. CC/examples 
4. Clinical presentation of each (DDx)
Definition
1.
Cortical=impaired storage/retrieval
Sunbcortical=impaired formation/encoding
 
2.
Cortical=impaired explicit, intact implicit
Subcortical=increased explicit (compensatory), decr. implicit
 
3.  
Cortical=Alzheimer's, Pick's, Creutzfeld-Jakob
Subcortical=MS, hydrocephalus, Parkinson's, Huntington's, microvascular disease 
 
4. DDx:
Cortical=impaired learning, recall, recognition, semantic memory
Subcortical=impaired retrieval, intact encoding as evidenced by intact recognition memory
Term

 

 

Wernicke-Korsakoff syndrome 

Definition
Cause: alcoholism-->thiamine deficiency-->dysfxn of (1) mammillary bodies
(2) thalamus
 
Sx:
1. Acute=Wernicke's encephalopathy:
a. confusion
b. oculomotor
c. ataxia
2. Chronic=Korsakoff's syndrome:
a. chronic anterograde amnesia
b. temorally-graded retrograde amnesia
c. confabulation 
Term

 

 

Alzheimer disease (AD) 

Definition
Cause: neural tangles, cholinergic deficits in hippo, P-F-T lobes (relative sparing: BG, occipital, cerebellum, BS)
 
Sx:
1. Episodic memory impaired the most (events)
2. Semantic memory progressively affected
3. Implicit memory: priming is impaired progressively, procedural can be intact
4. Working memory deteriorates progressively 
Term

Vascular brain disorders: 

1. vessels involed

2. sx 

Definition
1. PCA, thalamic penetrating aa, AComm (aneurysm)
2. ACommA syndrome:
Basal forebrain amnesia, confabulation, personality change 
3. Small vessel disease 
Term

 

 

Ischemic vs. histotoxic cerebral anoxia 

Definition
1. Ischemic=circulatory dysfxn, tissue perfusion not adequate
 
2. Histotoxic=chemical intereference w/ the cellular utilization of oxygen 
Term

 

 

Memory deficits seen in R vs. L hemisphere lesions 

Definition
1. R=LESS severe deficits in working memory-->recognition intact
 
2. L=more severe b/c L hemisphere is involved more with encoding, whereas R hemisphere is utilized more in retrieval ("R&R")
Term

 

 

Transient Global Amnesia 

Definition
Cause: cerebrovascular susceptibility, lasts 3-24 hrs, recurrence: 10% 
 
Sx:
1. sudden onset, profound amnesia, cannot learn new information
2. appear confused, lose track of thoughts, events
3. OK with basic personal info 
Term
Functional/psychogenic amnesias:
 
1. Somatoform Disorder
2. Malingering 
Definition
Hallmarks: amnesia for one's own name(exclusive) selective loss of personal info, test inconsistencies, retrograde>>anterograde amnesia
 
1. Somatoform disorders
Cause: psychological 
Sx: generalized pain, dizziness, depression, nausea...
Examples: hypochondriasis, coversion disorder
 
Conversion disorder: presence of sensorimotor dysfunction that suggests neurological condition 
 
2. Malingering
Cause: NONE (medical)
Sx: fabricating/exaggerating sx for personal GAIN/positive reinforcement 
Term
Experiment: brainstem transection
Effect on sleep-wake cycles/consciousness? 
 
1. Level=midbrain (above RF)
2. Level=caudal medulla (below RF) 
Definition
1. Midbrain=coma
 
2. Caudal medulla=sleep impaired-->AWAKE
 
Meaning: ascending arousal system (brainstem-->higher CNS centers)=RF
 
Caveat:
1. NREM-on center=VLPO (GABA/galanin desc. inhibition of RF arousal system)
2. REM-on center=PPT/LDT (cholinergic), aminergic neurons fall silent (LC, N. Raphe, TMN: 5-HT, NE, Histamine respectively)=disinhibition of PPT/LDT lead to REM
Term
Changes in sleep architecture through life:
1. NREM
2. REM 
3. Total sleep time 
4. REM:NREM ratio* 
Definition
1. NREM peaks in infancy, decreases with age
2. REM= decreases drastically from conception to birth, decreases slightly with age birth-->death
*18% REM 30s-40s (least % throughout life)
3. TST decreases conception-->death
4. LOTS of REM in womb (60:40), normalizes out to the usual 20:80 by age 3-5
Term
Sleep state dissociation disorders:
 
1. NREM
2. REM 
Definition
NREM:
1. Somnambulism
2. Pavor Nocturnus (night terrors)
3. Somniloquy
 
REM:
1. REM Behavioral Disorder: incomplete paralysis during REM
2. Narcolepsy
3. Cataplexy 
Term

 

 

Narcolepsy-Cataplexy 

Definition
Cause:
 
Sx:
1. Excessive Daytime sleepiness (EDS)
2. Hypnagogia: vivid sensory hallucinations while falling asleep (transition period)
3. Sudden shift from WAKE-->REM
4. Cataplexy: sudden loss of/deficit in motor ctrl.
5. Sleep paralysis: transient paralysis upon waking from sleep
 
Caveat: SSRI Discontinuation Syndrome (discontinuation of depression meds=motor deficits)
Term

 

 

Sleep Deprivation signs/Sx 

Definition
DECREASED:
1. alertness
2. body temperature (daily/waking temperature regulation pattern maintained b/c waking body temp. reg. is a distinct system) 
3. TSH, GH, PRL, leptin
4. CNS glucose utilization 
 
INCREASED:
1. co-morbidities (thrombosis, CV diseases, decr. lifespan, fibromyalgia, etc.)
2. Appetite, metabolism, SNS activation
3. Immune: phagocytes, cytokines
4. Ox stress: aminotransferases (ATs) 
Term

 

Pathological Left-Handedness 

Definition
Cause: early brain damage leading to a shift in development of handedness and concomitant atypical (cerebral) dominance patterns (R dominant)
 
Sx: R cerebral dominance, signs of early BD, sinistral 
Term

 

Brain damage

and

Language Cerebral Dominance development 

Definition
1. BD before 1 y/o:
Language spared, often generalized cognitive deficit
 
2. BD 1-5 y/o:
Language spared, crowding often (visual-spatial deficits; normally a R-hemisphere cognition)
 
3. BD >5 y/o:
Language deficits emerge; MEANS: brain plasticity declines with age!
Term
Disorders of speech:
1. Mutism
2. Aphonia
3. Dysarthria
4. Anarthria/aphemia
 
Disorders of LANGUAGE: 
Aphasias (Broca's, Wernicke's, transcorticals, conduction...)
Definition
1. Mutism: absence of speech/tendency to not speak
Causes: organic  (see: ant. cingulate, Tourette's..), choice
 
2. Aphonia: inability to produce vocalizations
Cause: vocal cord damage/dysfxn or periph. n(n).
 
3. Dysarthria: impaired articulation
4. Anarthria/aphemia: inability to articulate
Cause: neuromotor (or damage to white matter tracts underlying Broca's area efferents)
 
Aphasias: acquired linguistic dysfunction due to CNS damage
*Damage location-->type of aphasia (many) 
Term

Localizing/classifying Disorders of Language:

1. Fluency

2. Comprehension

3. Repetition 

 

Fluent aphasias vs. non-fluent aphasias 

Definition
Fluency=ability to perform fluid speech (defined as ability to string together 3 or more words per utterance)
3 parameters:
1. phrase length
2. effort
3. prosody: preservation of normal speech intonation 
Comprehension=auditory understanding of language
Repetition=ability to repeat phrases/instructions/etc.
 
Fluent aphasias=posterior BD (Wernicke's)
Non-fluent aphasias=anterior BD (Broca's)
 
 
Term
Fluent aphasias:
1. Transcortical sensory
2. Wernicke's
3. Conduction
4. Nominal/anomic
 
What structures/regions lesioned?  Comprehension intact?  Repetition? 
Definition
1. Transcortical sensory: lesions in angular gyrus or posterior, inferior temporal lobes
Sx: fluent (paraphasia), comprehension, repetition
 
2. Wernicke's: para-Sylvian lesions (posterior superior temporal lobe)
Sx: fluent (para), comprehension, repetition
 
3. Conduction: lesion=arcuate fasciculus (connects Broca's<-->Wernicke's)
Sx: fuent (para), comprehension, repetition
 
4. Anomic: lesion=temporo-occipital jxn area 
Sx: inability to assign names/meaning to objects, images, etc.
Fluency, comp & rep all intact
Term
Non-fluent aphasias:
1. Broca's
2. Transcortical motor
3. Transcortical mixed/isolation of speech
4. Global 
Definition
1. Broca's: lesion=dorsolateral frontal (opercular part of inferior frontal gyrus)
Sx: fluency, comprehension, repetition
 
2. Transcortical motor: lesion=SMA or M-I (just posterior to where Broca's aphasia lesions would be)
Sx: fluency, comprehension, repetition
 
3. Transcortical mixed/isolated: lesion=lesions in Broca's and Wernicke's areas or related secondary cortices
Sx: fluency, comprehension, repetition OK (weird clinical picture: hanging out with a robot)
 
4. Global: all structures affected related to language enough to disable all 3: fluency, comp. & rep.
Term
Clinical definitions: levels of consciousness (LOC)
 
1. awake
2. sleepy
3. stupor
4. coma 
Definition
1. ability to maintain wakefulness w/o stimulation
2. arousable w/ stimulation
3. arousable w/ stimulation, unable to maintain wakefulness w/o continuous stimulation
4. unarousable w/ ANY stimulation 
Term

 

Metabolic coma

vs.

Structural coma 

Definition
Diminished LOC=lesion to Retcular activating system (BS RF)
 
Metabolic: bilateral cerebral dysfunction
NOT bilateral-->NO COMA 
 
Structural:
(1) brainstem (RF=reticular formation)
(2) bilateral thalamus lesions 
Term

Diagnosis?

 

1. Complete R-sided hemiparesis  (face, body, works)

2. Eyes looking to R 

Definition
Dx: lesion in L frontal lobe
 
1. L motor cortex dysfunction
2. L FEF dysfunction-->unopposed R FEF pushed eyes to R 
Term
Primary vs. Cotrical sensation:
1. Difference
2. Examples of cortical sensation disorders.  Where do these disorders localize to in cortex? 
Definition
1. Primary=pain, vibration, temperature, etc.
Cortical=interpretation of sensory input/meaning
 
2. Parietal lobe lesion (contralateral)=
Agraphesthesia: inability to interpret letters/#s written on skin (palms usually)
Astereognosis: inability to assign proper names of shapes to objects (recognition defect)
 
Inferior parietal lobule (contralateral)=
Acalculia: acquired difficulty w/ simple mathematical tasks (Note: dyscalculia is a congenital similar disorder acquired when one learns math)
Finger agnosia: inability to recognize own thumb (agnosias: inability to recognize familiar objects)
Dys/agraphia: difficulty/inability to write (regardless of ability to read), mixing up words, symetrical letters, etc.
R-L confusion: "touch your R thumb to your L ear" 
Term

 

Gerstmann Syndrome 

Definition
Cause: lesion to inferior parietal lobule (L IPL if cerebral dominance is normal)
 
Sx:
1. Acalculia
2. Agraphia
3. L-R confusion
4. Finger agnosia 
Term

Neurofibromatosis Type 1:

1. Incidence

2. Inheritance, penetrance vs. expressivity (cause)

3. Diagnostic criteria (7)

4. Other sx 

 

Definition
1. 1:3000 (common)
2. AD, complete penetrance (if are hetero for NF1 mutant, you WILL have sx), variable expression (sx vary widely)
 
3. Dx criteria:
(1) ≥6 cafe-au-lait spots-->typically first sign
-80% will have 6 by 1 y/o
(2) intertrigenous freckles (axillary, inguinal)
-75%, usually appears in childhood
(3) ≥2 neurofibromas or 1 plexiform neurofibroma
-NFs: acquire, progressive, 100s-1000s, thoughout life
-PNFs: 25%, congenital, 10% malignancy, arise from Schwann cell precursors (malignant periph. n. sheath tumor; MPNST)
(4) Optic n. glioma
- 15%, by 4 y/o, astrocytoma, 50% asymptomatic (sx: proptosis, strabismus, vision-loss)
(5) ≥2 Lisch nodules (almost exclusive to NF1)
-bilateral, pigmented hamartoma, most NF1 px +10 y/o
(6) Bone abnormalities (scoliosis, tibial pseudoarthritis)
-NF1 accounts for 50% TPA, only 5% NF1 px actually have TPA)
(7) 1°  relative with NF1
- chromosome 17q, >300 muts, over 50% spontaneous
 
4. Other sx: LD (50%), HTN, vascular dysplasia, malignancy, macrocephaly, short stature, decr. life expectancy (~15 yr.) 
Term

 

RAS Pathway disorders (6)

Definition
 All are similar with characteristic facies (hypertelorism, flattened nose, ptosis, strabismus), cardiovascular, lymph and cutaneous symptoms.  All characterized (clinically) by sx, genetically by the distinct tumor suppressor mutated in the RAS pathway.
 
Distinguishing Sx: 
1. NF1 (Neurofibrin-->p53-->MPNST
Lisch nodules (iris), intertrigenous freckles, optic glioma 
2. Noonan Syndrome (NS) (SHP2/PTPN11)
Lentigines, facies, webbed neck, chest abn 
3. Cranio-Facial-Cut. Syndrome (CFCS) (BRAF)
More severe LD, skin path, GI problems, coarser face, NO neoplasias; JIMMY D.
4. Costello Syndrome (HRAS)
Papillomata, joint laxity, failure to thrive infancy, 15% malignancy risk 
5. LEOPARD Syndrome (SHP2)
(Acronym for sx) Note: deafness, lentigines
6. NF1-Like Syndrome (SPRED1)
No neural-derived tumors; lipomas & other tumor types
Term

 

Multiple Sclerosis (MS) 

Definition
Cause: chronic, demyelinating disease of CNS; both sensory & motor, AI?, gliosis/sclerosis (scarring)
 
Sx:
1. Paralysis, sensorimotor deficits (demyelination)
2. Periods of remission-relapse over years
 
Term

 

Guillain-Barre Syndrome 

Definition
Cause: acute, inflammatory demyelination in PNS; periph nn. of muscle & skin.  AI-myelin proteins (MBP, MAG, but not MOG (CNS only))
 
Sx: 
1. Sensorimotor disfunction (slowed conduction due to demyelination...similar sx to MS, but different (peripheral) pattern)
Term

 

 

Hyperkalemic Periodic Paralysis (HYPP)

or

Gamstorp Periodic Adynamy 

Definition
Cause: mutation in myocyte Na+ channels causing them to remain OPEN longer than normal (gene: SCN4A)
 
Sx:
1. Episodic muscle weakness and paralysis
2. Hyperkalemia (not always though)
3. Myotonia: muscle stiffness, spasms
 
Triggered by post-exercise rest, high-K+ foods, stress, etc.
Term
Myotonias:
 
1. Thomsen's (congenital)
2. Becker's 
Definition
Myotonia: muscle stiffness, spasms brought on by inactivity, cold (relieved by EXERCISE)
 
Cause: mutant Cl- channel (CLCN1) causing decreased Cl- conductance (in skeletal muscle, PCl is relatively high (unlike other tissues)-->PCl sets the resting Vm in skeletal myocytes
 
1. Thomsen's: AD inheritance, LESS severe
2. Becker's: AR inheritance, MORE severe 
Term

 

Myasthenia Gravis (MG)

Definition
Cause: Abs produced that bind to AChRs and block the stimulatory effect of ACh at post-synaptic membranes
Classes: I=mild, II...V=SEVERE 
Sx:
1. Muscle weakness (even though physical exam can be WNL) upon exertion, sudden onset
2. Often affects eye mm. first
3. Muscles of mastication, facial expression also are especially susceptible
 
Tx:
1. AChE inhibitors (neostigmine, etc.)
2. Immunosuppression
3. Thymectomy (extreme cases)
Term

 

Lambert-Eaton (Myasthenic) Syndrome (LEMS) 

Definition
Cause: (AI) Abs bind, block pre-synaptic Ca2+ channels required for the Ca influx responsible for efficient exocytosis of ACh from vesicles (CaM-mediated activation of synaptotagmin, which then causes rapid fusion event)
 
Associations: small cell (usually) lung cancers (Abs directed at tumor cells, accidentally attach NMJs); 50% LEMS px have an associated malignancy
 
Sx: (similar to MG sx) 
1. Unlike MG, ocular, facial & resp. mm. not affected as much; proximal limb mm. affected
2. ANS dysfunction sx (dry mouth, impotence)
3. Reduced reflexes
 
Tx:
1. Corticosteriods
2. aminopyrimidines (block IK(V), less K+ efflux, prolonged depolarization/AP)
3. Extreme: dialysis, IV immunoglobulins 
Term

 Compare:

1. Dorsal rhizotomy/lesion

2. Ventral rhizotomy/lesion

3. Complete spinal transection 

Definition
Rhizotomy: lesion/cutting of a spinal nerve ROOT
 
1. hypo/anesthesia (ipsilateral)
2. FLACCID (complete) paralysis, ANS nerves can be involved in ANTERIOR root lesions (ANS fibers pass out anteriorly from spinal cord), muscle atrophy
3. Initial "spinal shock"=no reflexes
After: SPASTIC paralysis (due to removal of desc. cortical motor regulation/inhibition=spontaneous LMN APs)
Term
Compare:
 
LMN vs. UMN lesions 
Definition
BOTH exhibit:
1. muscle weakness
 
LMN:
1. Muscle atrophy, fasciculations (no desc. ctrl.)
2. DECREASED reflexes & muscle tone
 
UMN:
1. (NO atrophy, fasciculations)
2. INCREASED reflexes & tone (once again: removal of desc. modulation=no inhibition)
Term

 

 

Thalamic Syndrome 

Definition
Cause: usually a vascular lesion or tumor to the VPL
 "A progression to dysesthesia"
Sx:
Initial: contralateral hemianalgesia
Next: excessive pain w/ noxious stimuli (loud noises, for example)
Later: pain provoked by ANY stimuli
Usually sensory input threshold is raised, but once reached, it is overwhelming & EMOTIONALLY linked
 
Dysesthesia: constant pain w/ or w/o stimulus
Term
Dorsal column lesions of spinal cord:
1. Examples
2. Sx 
Definition
Examples: (selective DC lesions are rare)
1. Tabes dorsalis
2. Neurosyphilis (advanced stages of infection)
3. Traums (stab wounds) to post. spinal cord 
 
Sx:
1. If below caudal medulla: IPSI touch/vib loss (w/ sparing of minor crude touch that decussated to contra ALS tracts)
2. IF above caudal medulla: CONTRA touch/vib loss 
 
Term
Nociceptive vs. neuropathic pain (and Tx):
1. NSAIDs
2. Opioids
3. Examples 
Definition
Nociceptive=tissue damage (chemical, inflammatory)
Sx: burning, prolonged quality (C-fiber mediated)
Tx: NSAIDS (block prostaglandin synthesis from arachidonic acid-->pain mediation)
Examples: rash, sprain, infected cuts
More severe: rheumatoid arthritis, tumors of soft tissue, fractures
 
Neuropathic=nerve tissue damage 
Sx: electrical, lancing quality
Tx: often do not respond to NSAIDs, opiods, so...
tricyclics (anti-depressant), anti-convulsants (raise neuron AP thresholds)
Examples: post-herpetic neuralgia, diabetic neuropathy, nerve entrapment (carpal tunnel)
Term
Anterior vs. Central Cord Syndrome:
1. Causes (examples)
2. Sx 
Definition
Anterior: unilateral=contra pain/temp loss, bi=total loss
Examples: ant. spinal aa. occlusion
 
Central: dermatome distribution of pain/temp. loss
Example: syringomyelia, glial cysts, trauma (fall-->cervical stretch)
Term

 

 

Asymbalia 

Definition
Cause: lesion to insular cortex
 
Sx:
Inappropriate responses to noxious stimuli (Ex: painful pinch causes laughter, placcidity (not caring), etc.) 
Term
Referred pain:
1. Cause
2. Review: spinal nerves carrying GVA from viscera
Diaphragm
Stomach
Duodenum
Kidneys/ureters
Crotch
Appendix
Gallbladder 
Definition
1. Visceral pain fibers converge on same 2o neurons as somatic afferents (more common), so brain interprets spinothalamic input as coming from a dermatome instead of the organ itself "hurting"
 
2. Some common referred pain patterns:
Diaphragm: C3-4
Heart/lungs: T1-4 (L mainly for HEART)
Stomach: T6-9 (LEFT)
Gallbladder: T7-8 (RIGHT)
Duodenum: T9-10
Appendix: T10 (RIGHT)
Crotch: T10-12
Kidney/ureter: L1-2
Term
(1) Trigeminal Neuralgia, (2) TMD
 
1. Causes
2. Sx 
3. Tx 
Definition
(1) Cause: TTT neuropathic pain (usually V2/V3)
Sx: lancing, severe pain, brief episodes triggered by shaving, chewing (allodynia)
Tx: carbamazopine (anticonvulsants: block INa(V) leading to hyperpolarization)
 
(2) Cause: chronic pain assoc. with chewing (nocicpetive pain, not neuropathic)
Sx: ear pain, headaches, masticatory mm. pain)
Tx: NSAIDs, opoids)
Term
Sensory deficits and CC examples:
1. Peripheral nerve lesions
2. Complete cord transection
3. Anterior vs. posterior cord syndromes
4. Central cord syndrome (small vs. large)
5. Brown-Sequard Syndrome
6. Lateral Medullay Syndrome 
Definition
1. Periph N.: "glove & stocking" distribution
Ex: trauma, diabetes, neuropathy
 
2. bilateral losses below (all GSA & GSE loss)
Ex: MS, expanding tumor, posterior trauma
 
3. ANT: ant. spinal aa. occlusion (pain/temp loss)
POST: (touch/vib loss), tabes dorsalis (syphilis)
 
4. Small: "cape-like" distrib. of pain/temp. loss
Large: expands to afect DCML, GSE tracts-->usually crotch discrim. touch is spared (this is good)
Ex: syringmyelia, C2 hyperextension (Reeves)
 
5. B-S Syndrome: CONTRA loss of pain/temp + IPSI touch/vib below level of lesion (pain/temp complete by 2-3 seg)
 
6. Lat Med Syn: loss of pain/temperature:
(1) ipsi face (2) contra body
Term
Color Vision Defects:
1. Red-Green Defects (Protanopes vs. Deutanopes)
2. Tritan defects
3. Blue Cone Monochromacy (BCM)
4. Rod Monochromacy/Achromatopsia 
Definition
X-chrom: L/M opsin array (C203R mutan=GCPRs)
3-chrom: S opsin
7-chrom: Rhodopsin (rod-opsin)
 
1. Protan=red-deficient, duetan=green-deficient
C203R mutant=G-cPR S-S bond residue (critical)
2. AD, incomplete penetrance, chrom-3 S opsin mutant in transmembrane domain-->blue deficits
3. L/M opsin array mutant:
Sx: poor acuity, myopia, nystagmus, ERG amp. low 
1-step mutant: loss of DNA enhancer=cannot txn opsin gene
2 step: fxnal receptor, mutant OPSIN
 
4. Absence of cone-based vision
Sx: photophobia, poor acuity (20/200), myopia, scotoma, visual field defects
Term

 

Ocular Albinism 

Definition
Cause: OA1 mutant (GCPR), x-linked recessive (only males affected fully)
 
Sx: decr./patchy pigmentation
1. iris transillumination (iris=translucent)
2. fundus hypopigmentation
3. nystagmus, loss of visual acuity
4. foveal hypoplasia 
5. skin macromelansosomes present (blotchy skin)
 
Term
Wet vs. Dry AMD
 
1. Causes
2. Sx

Definition
Dry: "drusen deposity"=protein+fat deposits in Bruch's membrane, 90% AMD
50% assoc. with CFH gene
sx: slow progression, can be asymptomatic or lead to photoR degen.
 
Wet: neuvascularization of choroid underlying retina, can progress rapidly, no gene association (yet)
Term
Ophthamologic pathology:
1. Congenitally Full Disc
2. Papilledema, pseudopapilledema
3. Optic N. pallor
4. Forster-Kennedy Syndrome
5. Optic Neuritis
6. Transient Monocular Visual Loss/Obscuration 
Definition
1. CFD: usually asymptomatic (small disc=ganglia axons crowd, bulge out)
 
2. Pseudo: not due to incr. intracranial pressure (other cause, like optic nerve head drusen (ONHD)
 
3. Pallor=axonal loss (optic nerve compression)
 
4. 1 eye=ipsi pallor
other eye=contra papilledema
Cause: IPSI pituitary tumor/meningioma
 
5. ON: 70% have no papilledema (normal fundoscope exam):
Clinical Triad: (1) low acuity, (2) color vision loss, (3) retro-ocular pain upon eye mvmt.
 
6. Hx: diabetes, coagulopathy-->thromboembolus leads to vision loss (ant. choroid, PComm, etc.)
Term
Localization of VF defects:
1. Behind/in front of Optic Chiasm?
2. Which side?
3. Congruence? 
Definition
1. Heteronymous: before optic chiasm
2. VF=contralateral processing
3. PCA supplies occipital lobe,  MCA does nearby, collateral flow=macular sparing
 
PCA occlusion=homonymous visual deficits (+ macular sparing often), weird shapes=MCA/stuff anterior to chiasm 
Term
Cochlear dysfunctions:
 
1. Conductive vs. Sensorineural hearing loss
2. Usher Syndrome 
Definition
1. Conductive: middle ear conduction issue
Dx: temporal bone conduction
Sensorineural: problem w/ transduction of signal (neural)
Hereditary: polygenic, high-f sound loss most common
 
Usher: 
1. severe, EARLY hearing loss
2. Assoc. Retinitis pigmentosa (due to shared gene for cytoskeleton constructing)
3. Balance issues can manifest also 
Term
Vestibular tests:
 
1. Optokinetic vs. vestibular nystagmus
2. Caloric testing (cold vs. hot water in ear) 
Definition
1. Optokinetic=fast phase opposite direction of object movement (object moves R=L-beating nystagmus)
Vestibular=fast in SAME direction as head rotation
 
2. COWS: cold=opposite, warm=same (Ex: inject cold water into R ear-->L-beating nystagmus...equiv. to rotating head to L or object in VF moving R)
 
Sumary:
OPPOSITE: cold, optokinetic
SAME: warm, vestibular/VOR 
Term
Effect of booze vs. deuterium on nystagmus:
 
1. Effect of each on endolymph
2. Position-dependent nystagmus: which direction (given one ear down/one up)? 
Definition
1. EtOH-->decr. endolymph density
Deuterim (heavy water) increases density
 EtOH:
R ear down: (1) R-nystagmus (into pillow) cupula is less dense than endolymph (absorbs first), (2) L-beating (endolymph absorbs'latent effect)
 
deuterium=opposite 
Term
DDX for vestibulopathies: hyper/hypoactive
1. BPPV
2. Vertebro-basilar Insufficiency
3. Peri-Lymphatic Fistula
4. Meniere's Disease
5. Vestibular Neuritis
6. Cerebello-pontine angle mass 
7. Rx-Induced Ototoxicity 
Definition
 Hyper=nystagmus toward, hypo=away
HYPERS: 
1. BPPV: seconds, dental work
3. Peri: minutes, valsalva/airplanes/swimming
4. Meniere: chronic, acute recurrences, tinnitus, ear pressure, Tumarkin's crises
 
 
HYPO: 
2. Vert-Bas: recurrent, brief, complete hearing loss
5. Neuritis: recent/on-going infection
6. C-pontine: hyperventilation, decr. hearing 
 
BOTH (no nystagmus):
7. Ototox: imbalance, unable to focus while moving, no vertigo or hearing loss 

Term
MOTOR disease localization, EXAMPLES:
 
1. UMN
2. LMN/peripheral NS
3. Myopathic 
Definition
ALL: weakness
1. UMN: incr. reflexes & tone (no atrophy, fascic.)
Stroke, etc.
 
2. LMN: decr. reflexes/tone, atrophy, fasciculations, interrupted/decr. MU recruitment pattern
Spinal: ALS, Spinal Muscular atrophy, Polio
root: disc herniation, rhizotomy
nerve: Pancoast's tumor, diabetes, Carpal tunnel
NMJ: MG, Lambert-Eaton, botulism
 
3. No fasciculations, MU recruitment pattern=normal/increased, MUP=small polyphasic
Myositis: (histo) clusters of myo-satellite cell nuclei (proliferating compensation)
 
Term
Cerebellar disorders:
 
1. Common sx
Definition
IPSIlateral manifestation of sx...
Sx:
Titubation (posture problems)
Scanning Dysarthria (deliberate pronunciation)
Hyperactive VOR
Dysdiadochokinesia (sup/pronation difficulty)
Appendicular ataxia
Gait imbalance/dysequilibrium
Decreased spinal reflexes, muscle tone
 
Term
BG disorders, 2 pathways, etc.
 
1. Common sx
2. Examples
3. The 2 pathways & assoc. dyskinesia type 
Definition
2 paths:
Direct: facilitates movement (lesion=hypokinesia)
Parts: Striatum (GABA, SubP; D1/SNc), GPi/SNr, Thalamus 
Example: Parkinson's (Tx: DA-replacement)
 
Indirect: inhibits (lesion-->HYPERkinesia) 
Parts: (above)+ STN (Glu/+), GPe, (SNc/D2 (-))
Examples: Hemiballismus, Huntington's
 
Term

 

Weber Syndrome 

Definition
Cause: cerebral peduncle lesion (common: PCA)
 
Sx:
1. CONTRA lower face-->body paralysis (CST)
2. IPSI occulomotor nerve palsy (CN III emerges in interpeduncular fossa) 
Term

Medullary Syndromes comparison:

 

1. Medial

2. Lateral (of Wallenberg)

Definition
Medial:
Sx:
1. CONTRA hemiparesis (body)
2. IPSI tongue paralysis & atrophy (CN XII)
3. CONTRA loss of vib/discrim. touch (Med Lemn)
 
Lateral:
Sx:
1. CONTRA loss of pain/temp. (STT/ALS)
2. IPSI dysphagia/dysarthria (CN X, XI/Ambiguus)
3. IPSI loss of pain/temp. (face) (spinal Nuc. of V)
4. Vertigo, nausea, nystagmus (Vestibular nuclei) 
5. Horner's syndrome (loss of SNS desc. fibers to eyes=ptosis, anhidrosis, miosis)
 
Term

 

Brown-Sequard Syndrome 

Definition
Cause: spinal cord hemisection
Sx:
1. CONTRA loss of pain/temp. (STT/ALS)
2. IPSI fine touch loss (DCML)
3. LMN lesion=atrophy, fasciculations (spastic paralysis), decr. reflexes & tone)
4. IPSI Babinski dysreflexia/+(CST)
Term
Posturing syndromes: sx, cause, contrast, etc.
 
1. Decorticate
2. Cederebrate 
Definition
Decorticate:
Cause: lesion cutting cortex off from Red Nuc. (lesion is rostral to Red Nuc.)
Intact: RuST, Cerebellum (from Dentate/lateral)
NOT: CST
Sx: LOWER limbs=extended, UPPER=flexed (mummy-style)
 
Decerebrate: WORSE
Cause: transection @ level btw. colliculi pairs (S/Inf)
Caudal to the Red Nuc.
Sx:
LOWER & UPPER limbs: extended/adducted
Why: removal of cortex excitation to LReST (which is inhibitory)=unopposed excitatory MReST
 
 
Term

 

Benedikt's Syndrome 

Definition
Cause: unilateral lesion to Red Nucleus (MB)
Sx:
1. IPSI occulomotor palsy (eye dev. lateral; unopposed CN VI)
2. IPSI miosis (pupil dilated; unopposed SNS), ptosis
3.  Usually occurs in conjunction w/ CST lesion (see Weber Syndrome, Medial medullary syndrome)
Term

 

Pseudobulbar Palsy 

Definition
Cause: bilateral corticobulbar tract lesion (passes thru genu of IC, synapses in RF/motor nuclei of BS)
 
Sx: similar to Lateral Medullary Syndrome w/o the ALS system assoc. deficits
1. Dysphagia/dysarthria (X, XI-Nuc. Ambiguus)
2. Tongue paralysis
3. Loss of emotional control (unique) 
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