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Step 1 > Nervous System - Diseases > Flashcards

Nervous System - Diseases Flashcards

1
Q

Multiple Sclerosis

A

Autoimmune demyelinating disease of the CNS, most often affecting women in their 20s and 30s with a relapse-remitting course

Presents with optic neuritis (sudden loss of vision, Markus-Gunn pupil), hemiparesis/hemisensory defects, bladder/bowel incontinence, ‘scanning speech’, intention tremor

Treatment: Beta-interferon, immunosuppression, Natalizumab

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2
Q

Major Depressive Disorder

A

Characterized by at least one major depressive episode lasting 6+ months, characterized by patient-reported depressed mood/anhedonia + SIGECAPS (Sleep disturbance, loss of interest, guilt, energy loss, poor concentration, changes in appetite/weight, psychomotor retardation/agitation, suicidal ideation)

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3
Q

Bipolar Disorder I

A

Characterized by the presence of at least one manic episode lasting 1 week or requiring hospitalization, characterized by DIGFAST: Distractability, Irritability/Irresponsibility, Grandiosity, Flight of Ideas, Agitation/increased activity, Decreased need for Sleep, Talkativity/Pressured Speech

Acute mania is treated with atypical psychotics
Lithium prevents and reduces severity of manic episodes

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4
Q

Bipolar Disorder II

A

Characterized by the presence of at least one hypomanic episode and one depressive episode

Hypomania presents similarly to mania (DIGFAST) but less severe and with symptoms lasting at least 4 days and not requiring hospitalization

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5
Q

Schizoid Personality Disorder

A

Characterized by voluntary social withdrawal and limited emotional expression; content in social isolation (vs. avoidant)

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6
Q

Schizotypal Personality Disorder

A

Characterized by eccentric appearance, odd or magical thinking, and interpersonal awkwardness

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7
Q

Schizophrenia

A

Characterized by psychosis (hallucinations, delusions, disturbed thoughts/behavior) lasting at least 6 months; may also present with disorganized speech/behavior or negative symptoms (flat affect, social withdrawal)

Treated with anti-psychotics and atypical anti-psychotics

Schizophreniform Disorder - same diagnostic criteria but symptoms lat 1-6 months

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8
Q

Schizoaffective Disorder

A

Characterized by psychotic features of schizophrenia + major mood disorder (Bipolar, Major Depression)

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9
Q

Alzheimer Disease

A

90% Sporadic, 10% Familial; early onset familial cases associated with mutations in APP (Chr 21), Presenelin-1 (Chr 14), and Presenelin-2 (Chr 1); late onset associated with ApoE4 (Chr 19) whereas ApoE2 (Chr 19) is protective

Findings: Widespread cortical atrophy, B-amyloid plaques (extracellular), neurofibrillary tangles (intracellular tau protein accumulation), decreased ACh

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10
Q

Lewy Body Dementia

A

Findings: Lewy Bodies (a-synuclein inclusions)

Presents as dementia, visual hallucinations, and Parkinsonism

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11
Q

Parkinson Disease

A

Degenerative disorder of the CNS caused by accumulation of Lewy Bodies (a-synuclein) in the substantia nigra; characterized by decreased dopamine and increased ACh

Presents with resting tremor, cogwheel rigidity, bradykinesia, postural instability, and shuffling gait

Treatment: L-DOPA/Carbidopa, Amantidine, Selegiline, Entacapone/Tolcapone, Benzotropine

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12
Q

Communicating Hydrocephalus

A

Decreased CSF reabsorption by arachnoid granulations, i.e. caused by arachnoid scarring post-meningitis

Causes increased intracranial pressure, papilledema, and herniation

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13
Q

Normal Pressure Hydrocephalus

A

Communicating hydrocephalus causing enlargement of the ventricles which distort the corona radiata

Presents with triad of urinary incontinence, ataxia, and cognitive decline

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14
Q

Non-communicating Hydrocephalus

A

Caused by structural blockage of CSF circulation within the ventricular system

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15
Q

Erb-Duchenne (Waiter’s Tip) Palsy

A

Caused by damage to the C5 and C6 nerve roots during delivery; damages abductors, flexors, and supinators of the upper arm

Arm hangs by side (abducted) with forearm pronated and wrist flexed

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16
Q

Central Pontine Myelinolysis

A

Osmotic demyelination of the corticobulbar and corticospinal tracts caused by over-correction of hyponatremia with hypertonic saline

Presents with dysarthria, dysphagia, and rapid paralysis

MRI shows abnormally increased signal in pons

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17
Q

Lateral Medullary Syndrome (Wallenberg Syndrome)

A

Caused by vascular disruption of the PICA, which supplies the lateral medulla

Presents with:

Vomiting and vertigo (vestibular nuclei)
Loss of pain and temperature from contralateral body (lateral spinothalamic tract)
Loss of pain and temperature from ipsilateral face (spinal trigeminal nucleus)
Dysphagia and hoarseness (nucleus ambiguus)
Ipsilateral Horner Syndrome (descending sympathetics)

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18
Q

Horner Syndrome

A

Caused by disruption of the descending sympathetic fibers above the level of T1; disrupts innervation of facial sweat glands, pupillary dilator, and smooth muscle of the eyelid

Presents with: Unilateral ptosis, anhidrosis, miosis

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19
Q

Lithium Toxicity

A

Presents with memory impairment, confusion, nausea/vomiting, dysarthria lethargy, coarse hand tremor; progresses to ataxia, hyperreflexia, seizures, and coma

Also associated with hypothyroidism and nephrogenic diabetes insipidus

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20
Q

Viral Meningitis

A

Most often caused by enteroviruses (in kids) or HSV (in adults)

CSF findings: Elevated WBCs (mostly lymphocytes), slightly elevated or normal protein, normal glucose, normal opening pressure

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21
Q

Viral Encephalitis

A

HSV-1 is the most common cause

Presents with AMS, personality changes

CSF Findings: Bloody CSF, elevated WBC (mostly lymphocytes), normal or slightly elevated protein, normal glucose

Imaging shows temporal lobe involvement

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22
Q

HIV-associated dementia

A

Most common direct CNS complication of HIV, occurring late in the course of disease

Early symptoms - depression, apathetic withdrawal; progresses to global cerebral atrophy, motor deficits

Findings: Elevated protein and IgG in CSF; MRI shows non-enhancing, poorly demarcated areas of increased signal on T2 in the deep white matter

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23
Q

Locked-In Syndrome

A

Caused by occlusion or hemorrhage of the Basilar a. which supplies the pons, medulla, and lower midbrain including the corticospinal and corticobulbar tracts

Presents as quadriplegia and loss of all facial, mouth, and tongue movements

Oculomotor and Trochlear nerves are spared with preserved blinking and up-gaze

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24
Q

Cluster A Personality Disorder - Characteristics and Conditions (3)

A

Odd or eccentric thinking/behavior with inability to form meaningful social relationships

Paranoid
Schizoid
Schizotypal

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25
Q

Paranoid Personality Disorder

A

Cluster A

Characterized by pervasive distrust and suspicion

26
Q

Schizoid Personality Disorder

A

Cluster A

Characterized by voluntary social withdrawal - individual is content with social isolation (vs. avoidant); limited emotional expression

27
Q

Schizotypal Personality Disorder

A

Cluster A

Characterized by eccentric appearance, odd/magical thinking, and interpersonal awkwardness

28
Q

Cluster B Personality Disorders - Characteristics & Conditions (4)

A

Dramatic, overly emotional, and erratic; genetic association with mood disorders and substance abuse

Antisocial
Borderline
Histrionic
Narcissitic

29
Q

Antisocial Personality Disorder

A

Cluster B

Characterized by disregard for and violation of others’ rights, criminality, and impulsivity; males > females

Diagnosis requires age > 18 with hx of conduct disorder < 15

Diagnosed as conduct disorder in patients < 18

30
Q

Borderline Personality Disorder

A

Cluster B

Characterized by unstable moods and interpersonal relationships, impulsivity, self-mutilation; females > males

Major coping mechanism is “splitting” - a tendency to view others as ALL good or bad

31
Q

Histrionic Personality Disorder

A

Cluster B

Characterized by excessive emotionality and excitability, attention seeking, sexual provacation, and excessive concern with appearance

32
Q

Narcissistic Personality Disorder

A

Cluster B

Characterized by grandiosity, sense of entitlement, lack of empathy; require excessive praise and often respond to criticism with rage

33
Q

Cluster C Personality Disorders - Characteristics & Conditions (3)

A

Anxious and fearful

Genetic association with anxiety disorders

34
Q

Avoidant Personality Disorder

A

Cluster C

Hypersensitive to rejection, socially inhibited with feelings of inadequacy

Desires relationships with others (vs. Schizoid)

35
Q

Dependent Personality Disorder

A

Cluster C

Submissive, clinging, with low self confidence and excessive need to be taken care of

36
Q

Obsessive Compulsive Personality Disorder

A

Cluster C

Excessive preoccupation with perfection, order, and control

Ego-syntonic (vs. OCD) - behavior and thoughts are consistent with one’s own beliefs and attitudes

37
Q

Epidural hematoma

A

Most commonly caused by rupture of the middle meningeal artery secondary to temporal trauma

Rapid expansion of hematoma under arterial pressure causes trans-tentorial herniation (CNIII Palsy); presents with lucid interval

CT shows hyper-dense, “lens-shaped” accumulation of blood which crosses the falx and tentorium but does not cross suture lines

38
Q

Subdural hematoma

A

Caused by rupture of the bridging veins leading to gradual development of hematoma

Increased risk in elderly, alcoholics, blunt trauma, shaken babies

CT shows hyperdense, “crescent-shaped” hematoma which crosses suture lines but does NOT cross falx or tentorium; may cause mid-line shift (sub-falcine herniation)

39
Q

Subarachnoid hemorrhage

A

Caused by rupture of aneurysm; increased risk in connective tissue diseases (Marfan’s, Ehler’s Danlos, AVM)

Presents with “worst headache of my life”

CT shows hyperdense accumulation of blood in the sulci and/or ventricles; + blood in spinal tap

40
Q

Intraparenchymal (hypertensive) hemorrhage

A

Most commonly occurs as a result of systemic hypertension, but can also be seen in amyloid angiopathy, vasculitis, neoplasm

Most commonly affects the basal ganglia and/or internal capsule

41
Q

Subacute sclerosing panencephalitis (SSPE)

A

Sequelae of measles (Rubeola) virus, occurring 7-10 years after infection due to progressive demyelination of the CNS

Presents in 4 stages:

  1. Personality changes
  2. Myoclonus
  3. Worsening dementia
  4. Decorticate posturing, death
42
Q

Pineoblastoma

A

Arise from the pineal gland, located between the thalamic bodies and responsible for melatonin secretion and regulation of sleep/wake cycles

Presents as excessive sleepiness and/or vivid dreams; most commonly in patients < 20 years old

43
Q

Radial Neuropathy

A

Damage to the radial nerve

May occur as a result of mid-shaft fracture of the humerus (where radial n. sits within the spiral groove) or compression of the axilla (crutches, “Saturday Night Palsy”)

Radial n. innervates the brachioradialis, extensors of the wrist and fingers, supinator, and triceps (“BEST”)

Presents with loss of extension at elbow, wrist, and fingers (‘wrist drop’), diminished grip strength, and loss of sensation over the posterior arm/forearm and dorsal hand

44
Q

Craniopharyngioma

A

Most common supra-tentoral neoplasm in children; accounts for 80-90% of pituitary area lesions in kids

Most children present with endocrine dysfunction: GH deficiency, gonaotropin deficiency, ACTH deficiency, TSH deficiency; less commonly hyperprolactinemia or central DI due to pituitary stalk dysfunction

Dx: Large, well-circumscribed, suprasellar mass +/- calcifications

45
Q

Tricyclic Antidepressant Overdose - Clinical Syndrome

A

“3 Cs” - Coma, convulsions, cardiotoxicity

Presentation varies but may include hypotension, anticholinergic effects, respiratory depression, hyperpyrexia, cardiac conduction defects and life-threatening arrhythmias, seizures

Treated with bicarbonate; alkalinization of plasma increases bidning of TCA to plasma proteins, reducing the amount of TCA bound to myocardial calcium channels and thereby stabilizing the myocardium. Improved perfusion resolves the metabolic acidosis

46
Q

Cluster Headaches

A

Repetitive, unilateral, brief (15 minutes - 3 hour) headaches characterized by excruciating periorbital pain with lacrimation and rhinorrhea; may induce Horner syndrome

Treatment: Inhaled oxygen, Sumatriptan

47
Q

Tension headaches

A

Bilateral headaches lasting 30 minutes - 6 hours; pain is constant without photophobia, phonophobia, or aura

Treatment: Anangesics, NSAIDs, acetaminophen; Amitriptyline for chronic pain

48
Q

Migraine Headaches

A

Unilateral, pulsating headaches lasting 4-72 hours; accompanied by nausea, photophobia, phonophobia, or aura

Treated with abortive therapies (NSAIDs, Triptans) and prophylactic (Propanolol, Topiramate, CCBs, Amitriptyline)

49
Q

Primary Brain Tumors of Adults - 5

A 
Glioblastoma Multiforme
Meningioma
Hemingioblastoma
Schwannoma
Oligodendroglioma
Pituitary adenoma
50
Q

Glioblastoma Multiforme

A

i.e. Grade IV astrocytoma; most common primary brain tumor of adults with poor prognosis (median survival = 1 year)

Usually occurs in the cerebral hemispheres; can cross the corpus callosum (“butterfly glioma”)

CT/MRI shows ring-enhancing lesion
Histology shows “pseudopalisading” pleomorphic tumor cells surrounding central hemorrhage/necrosis

51
Q

Meningioma

A

Common, usually benign tumor arising from the arachnoid, usually in the cerebral convexities

Often asymptomatic +/- seizures, focal neuro deficits

Histology shows “spindle-like” cells +/- Psammoma bodies

Treatment: Resection +/- radiation

52
Q

Schwannoma

A

Tumor of Schwann cell origin, usually seen at the cerebellar-pontine angle with involvement of CN VIII (bilateral acoustic schwannomas seen in NF-2)

53
Q

Hemangioblastoma

A

Malignant proliferations of closely packed, thin-walled capillaries; most often cerebellar

May produce EPO causing secondary erythrocytosis

Associated with VHL when seen with retinal hemangiomas

54
Q

Oligodendroglioma

A

Slow growing tumor of oligodendrocytes; usually found in the frontal lobes

Histology shows tumor cells with “fried egg appearance” (round nuclei surrounded by clear cytoplasm)

55
Q

Lacunar Infarct

A

Infarction of the lenticulostriate arteries, penetrating branches off of the MCA which supply the internal capsule and basal ganglia

Often seen in the setting of long-standing HTN

56
Q

Kluver-Bucy Syndrome

A

Caused by bilateral damage to the amygdala, often secondary to ischemic stroke

Presents with hypersexuality, hyperorality, and disinhibited behavior

57
Q

Gerstmann’s Syndrome

A

Caused by lesion of the angular gyrus in the visual association cortex

Presents with inability to distinguish left from right, inability to recognize fingers, inability to understand arithmetic (acalculia), inability to write (agraphia)

58
Q

Broca’s aphasia

A

Caused by lesion of Broca’s area in the inferior gyrus of the frontal lobe

Characterized by non-fluent speech with in-tact comprehension; patients can understand and form thoughts but cannot generate speech, leading to frustration

59
Q

Wernicke’s aphasia

A

Caused by lesion of Wernicke’s area in the superior gyrus of the temporal lobe

Characterized by fluent speech with impaired comprehension (gibberish)

60
Q

Conduction aphasia

A

Caused by lesion of the arcuate fasciculus, connecting Broca’s and Wernicke’s areas

Characterized by fluent speech and intact comprehension but impaired repetition

61
Q

Global aphasia

A

Caused by lesion of arcuate fasciculus + Broca’s area + Wernicke’s area

Characterized by non-fluent speech and impaired comprehension

62
Q

Tyramine reaction

A

Adverse drug interaction seen with use of non-selective MAOIs; decreased metabolism of NE, Epi, DA, 5-HT and tyramine (found in wine, cheese) may cause hypertensive crisis

Also seen with concomitant use of sympathomimetics (SSRIs, TCADs) - “serotonin syndrome”

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