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Flashcards in Diseases Block 4 Deck (42):

Injury to FEF

Sx: Loss of voluntary saccades to contralateral side and deviation of eyes towards lesion. Loss of ability to move gaze away from stimulus.

Sx: can resolve over time (days/weeks) due to plasticity


Premotor Cortex lesion

Lesion produces inability:
-respond properly to stimuli (i.e. can't use toothbrush but know how)
-Plan appropriate movements based on circumstances (can't reach under glass for treat)
-Lean new sensory-motor associations
-Steer arm accurately


Supplementary Motor Area Lesion

Lesion results in:
-reduction in volitional movements
-loss of suppression of motor programs triggered by visual stimuli (alien hand syndrome and utilization behavior)
-Neglect of affected limb


Alien Hand Syndrome

Contralateral "semi-purposeful" movements that are outside patient's volitional control

Seen in lesion to SMA


Utilization behavior

Use of objects in an inappropriate setting (excessive response to external stimuli

Seen in lesion to SMA


Multiple Sclerosis

Autoimmune inflammation and demyelination of CNS. Patients can present with optic neuritis (sudden loss of vision), MLF syndrome (internuclear opthalmoplegia), hemiparesis, hemisensory symptoms, or bladder/bowel incontinence. Relapsing and remitting course. Most often affects women in their 20s and 30s; more common in whites.

Genetic and environmental. Linked to HLA-DR2 and some IL-2 and IL-7 receptor polymorphisms

Findings: increased protein (IgG) in CSF. Oligoclonal bands are diagnostic. MRI is gold standard. Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with preservation of axons.

Charcot's triad: SIN Scanning speech, Intention tremor, Incontinence, Internuclear opthalmoplegia, Nystagmus.

Treatments: Beta- Interferon or immunosuppressant therapy. Symptomatic treatment for neurogenic bladder, spasticity, pain.


Acute Disseminated Encephalomyelitis (ADEM)

Diffuse, monophasic demyelination following a viral infection; usually in children

Rapid onset headache, lethargy, coma

Fatal in 10%; rest recover completely

May be an acute autoimmune rxn vs myelin


Acute Necrotizing Hemorrhagic Encephalomyelitis

Fulminant CNS demyelination in young adults/children
that is preceded by URI.

Fatal in many patients; significant deficits in survivors

May be a hyperacute variant of ADEM


Central Pontine Myelinolysis

Symmetric loss of myelin in basis pontis and part of pontine tegmentum. Results in rapidly evolving quadriplegia.

Monophasic disease.

Caused by rapid correction of hyponatremia


Treahcer Collins Syndrome

Deficiency of neural crest cells migrating into the first arch.

Sx: Mandibulofacial dysostosis, Abnormal development of first arch structures, Hypoplasia of jaw; middle and external ear deformities, palate and eyelid defects.

"Fishmouth appearance"

Conductive hearing loss.

Autosomal dominant or teratogenic cause.


Pierre Robin Syndrome

Deficiency of neural crest cells migrating into first arch.

Sx: Abnormal development of first arch structures. U shaped cleft palate.

Micrognathia (small jaw) and large, posteriorly placed tongue leads to airway obstruction.

Genetic or environmental etiology.

Rx: Tracheostomy to maintain airway and bilateral mandibular lengthening.


Goldenhar Syndrome

1st and 2nd arch syndrome. Possibly caused vascular insult etiology.

Sx: hypoplastic maxilla, mandible, and temporal bone. Ear may be absent, vertebral abnormalitis, dermoid tumors of the eye.


Digeorge Syndrome

No thymus or parathyroids ( = diminished immunity, hypocalcemia)

Failure of neural crest migration into arches leading to 3rd and 4th pouch failure and hypoplasia of 1st arch (cleft palate, low set ears, poor feeding, delayed speech)

Heart defects, poor circulation, poor muscle tone.

CATCH 22: Microdeletion of Chr. 22q11.2
Cardiac abnormality (especially tetralogy of Fallot)
Abnormal facies
Thymic aplasia
Cleft palate


Lateral cervical (branchial) cyst or fistula

When cervical sinus formed by 2nd, 3rd, and 4th clefts persists.


Thyroglossal Duct Cyst

Midline cystic mass
Preceding URI
Mobile with tongue protrusion and swallow.

-treat infx first
-avoid incision and drainage
-Excision of entire tract, cyst
-Remember anatomic relationships with nerves


Branchial Cleft Cyst or Lateral Cervical Cyst

Lateral Cystic neck mass
Preceding URI

-treat infx first
-avoid incision and drainage
-Excision of entire tract, cyst
-Remember anatomic relationships with nerves


Lingual thyroid

Undescended thyroid persists at back of tongue.



Tongue tied.

Presentation: neonatal, dysarthria, dental issues

When in doubt, cut it out



Transient Ischemic Attack and TSI???

Transient neurologic dysfunction without evidence for cerebral infarct.

TSI: increased risk of stroke relative to TIA,


Intracerebral hemorrhage

A brain bleed that occurs below the pia mater

Rx: Stop or reverse anticoagulants

Risk Factors: Age, male, HTN, EtOH, Tobacco, DM

Dx: Non contrast CT (white)


Subarrachnoid Hemorrhage

Bleed between pia and arachnoid. Caused by trauma or burst aneurysm.

Sx: "Worst headache of my life" Nausea and Vomiting, red or yellow LP.

Dx: Non-contrast CT (white and more diffuse than intracerebral hemorrhage)

Risk Factors: Smoking, HTN, EtOH, Nonwhite, Family history, Aneurysm

Rx: Nimodipine (Calcium channel blocker), coils or surgical clipping of aneurysms.


Small Vessel (Lacunar) Stroke

HTN induced endothelial damage which leads to clot formation.


MCA Stroke

Upper body, face, broca's on dominant side, neglect of nondominant side, dysphagia, homonomous hemianopsia (look at stroke)


ACA Stroke

Lower body, frontal lobe, personal problems


Lenticulostriate Stroke

Deep structures, hemiparesis, hemiplagea, weird


Verterbral Artery Stroke

Vomitting and Nystagmus.

Cerebellar dysfunction

Lateral medullary infarct (Wallenberg Syndrome)


Basilar Artery Stroke

Altered consciousness, CN involvement, and Respiratory Problems


PCA Stroke

Vision loss (often macular sparing)


Parkinson's Disease

Degenerative disorder of CNS associated with Lewy Bodies (alpha-synuclein intracellular inclusion) and depigmentation of the substantia nigra pars compacta (loss of dopaminergic neurons)

TRAP: Tremor at rest (pill-rolling) Rigidity (cogwheel), Akinesia, and Postural instability (shuffling gait)

Rx: Levodopa-Carbidopa is the standard. Many others. Goal is to increase CNS DA levels.



Sudden wild flailing of one arm +/- leg that is characteristic of contralateral subthalamic nucleus lesion (i.e. lacunar stroke). Loss of inhibition of thalmus through globus pallidus.


Huntington's Disease

Autosomal dominant trinucleotide repead disorder characterized by chorea, aggression, depression, and dementia. (sometimes mistaken for substance abuse)

Neuronal death via NMDA-R binding and glutuamte toxicity. Atrophy of striatal nuclei (main inhibitors of movement) can be seen on imaging.

Expansion of CAG repeats (Caudate loses Ach and Gaba)

Symptomatic care. Usually death withing 10-20 years of onset.


Diabetes Insepidus

Loss of vasopressin secondary to: 1.) head trauma, 2.) autoimmune attack of magnocellular neurons, or 3.) Idiopathic

Symptoms: Polyuria and Polydypsia

Rx: desammo? Vasopressin (retain water w/o increased BP)



Innapropriate lactation adn cessation of menstration due to too much prolactin.

Etiology: Pituitary microadenoma that releases prolactin leading to decreased FSH and LH. Patient will present with problems getting pregnant.

Rx: 1.) surgical resection 2.) give dopamine receptor agonist to block prolactin.


Prader-Willi sydrome

Deletion on chromosome 15

incidence: 1 in 25,000

Sx: fetal hypotonia, mental retardation, hypogonadotropic hypogonadism, hyperphagic obesity (due to increased grehlin release)


Urbache-Wiethe disease

Very rare genetic disease that affects medial temporal lobes

Impaired recognition of emotion and memory loss especially informational w/ emotional content



Triad of Symptoms: Re-experiencing phenomona (flashbacks), avoidance of situations parallel the initial trauma, and hypervigilance/arousal (increased anxiety)

Etiology: Decreased activity in medial prefontal cortex which inhibits amygdala activity. Secondary to fear conditioning.



Etiology: Two hypothesis: 1.)too much dopamine receptor activity 2.) too little glutamate NMDA activity

Rx: haloperidol (typical), clozapine (atypical)



Caused by decreased NE and 5-HT receptor activity

Symptoms: lethargy, anhedonia, and loss of sleep

Incidence: 15% (girls almost 2x)

Rx: MAOis, Tricyclics (Imipramine), or SSRIs (Fluoxetine)


Korsakoff's Syndrome

Damage to mamillary body or mamillo-thalamic tract secondary to Thiamin (B1) defeiciency. (Alcoholics or Malnutrition)

Symptoms: (Can of Beer) Disorder of immediate memory, Disorientation in space and time, and Confabulation


Kluver-Bucy Syndrome

Caused by bilateral medial temporal lobe damage secondary to encephalitis or trauma.

Symptoms: Oral tendencies, Neutral affect on emotions (amygdala), hypersexuality (hypothalamus), visual agnosia


Alzheimer's Dementia

Degenerative loss of cholinergic input to hippocampus starting in Nucleus Basalis. Loss of neurons in multiple areas. Presence of neurofibrillary tangles (NFTs) and Beta-amyloid plaques.

Symptoms: loss of memory, mood disorder (anxiety and depression), loss of motor function, complete loss of cognitive function


Chronic Traumatic Encephalopathy (CTE)

Progressive neurodegenerative disease caused by repetitive head trauma (mild traumatic brain injury, TBI or concussions).

Etiology: Generalized brain atrophy including prefrontal cortex, temporal lobes, and parietal lobes. NFTs present.

Triad of symptoms: 1.) cognition: anterograde amnesia and executive dysfunction, 2.) Mood: depression and apathy, and 3.) Behavior: loss of impulse control and aggressiveness