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Medical Pathology > Neurological Diseases > Flashcards

Neurological Diseases Flashcards

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USMLE® Step 1 flashcards
1
Q

Multiple Sclerosis

A

80%: most common demyelinating disease

a cell-mediated (t cell) autoimmune disease of brain and spinal cord leading to central demyelination from scarring of myelin sheaths directly (Type IV autoimmune reaction)

Average onset is 29 years old

RF: low vitamin D, smoking, genetic predisposition, northern latitude, WHITE > everyone else

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2
Q

What are the types of MS?

A

Clinically Isolated Syndrome

Relapsing-Remitting: 85%

Primary Progressive: straight line (10%)

Secondary Progressive: rarest form

Progressive Relapsing: 5%

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3
Q

What are the ocular symptoms of MS

A

optic neuritis + CN palsy and Nystagmus

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4
Q

how do you dx MS?

A

2+ attacks with lesions on MRI

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5
Q

what is NMO

A

neuromyelitis optica - Devic’s Disease

autoimmune disorder where optic nerve and spinal cord is affected by an immune attack on AQ4 (Type II rxn)

IgG Antibody in 70% of NMO patients

more women than men (8:1) and onset around 40

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6
Q

how do you dx NMO

A

MRI with 3+ vertebral segments, IgG Antibody, no brain lesions on MRI

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7
Q

what is Guillain barre syndrome

A

acute and idiopathic polyneuritis

peripheral NS demyelination and progressive ASCENDING numbness/paralysis

caused by GI or respiratory infection (V/B)

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8
Q

what is the ocular variant of GB syndrome

A

miller fisher syndrome

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9
Q

What is myasthenia gravis?

A

autoimmune disorder of NMJ where antibody blocks Ach and inactivates voluntary muscle

onset around 20

Thymoma Patients: 50% develop MG

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10
Q

what are the ocular manifestations of MG

A

ptosis + fluctuating diplopia + alternating CNP

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11
Q

how do you dx MG

A

Tensilon (+) in 90%, Ice pack test (on droopy eyelid), EMG, Anti-Ach Receptor antibody

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12
Q

What is bell’s palsy

A

inflammation of CN VII (facial nerve) and most common cause of facial paralysis

usually idiopathic + rapid onset

Unilateral facial weakness, facial droop, diminished eye blink, hyperacusis, decreased lacrimation

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13
Q

what are the ocular manifestations of traumatic brain injuries?

A

VFD / Neglect, CNP, Accommodative Dysfunction, CI, Photophobia

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14
Q

what are the 3 locations a brain tumor can come from?

A

lung > breast > colorectal

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15
Q

what are the symptoms of a brain tumor?

A

HA (increased ICP), papilledema, vomit, dysphagia, personality change + VFD/neglect, loss of vision, CNP, Retinal Detachment

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16
Q

What is cerebral palsy

A

group of syndromes – permanent, nonprogressive neuronal damage of motor control

most common motor disability in children

Spastic: most common form: stiff + tight muscles
Ataxic: shaky movements
Dyskinetic: involuntary movements

17
Q

what are the ocular manifestations in CP (40% of patients)

A

strabismus, RE, nystagmus, amblyopia, optic atrophy and VFD

18
Q

what is muscular dystrophy

A

progressive genetic disorder where striated muscle is degenerated

19
Q

what is duchenne dystrophy

A

x-linked disease that is most common form where absence of dystrophin protein in striated muscle and muscle fibers is replaced by fat + CT

death by 30s due to dilated cardiomyopathy

20
Q

what is amyotrophic lateral sclerosis (ALS)

A

progressive neurodegenerative disorder of motor neurons without dementia

onset at 55 year old and idiopathic (90%)

RF: smoking, lead, military services

weakness of lower and upper extremities + tongue atrophy (speech impairment)

death by respiratory failure

DOES NOT AFFECT EOMS OR PERSONALITY

21
Q

what is Huntington’s disease

A

progressive degeneration of basal ganglia leading to amyloid-protein related accumulation

autosomal dominant with enlarged lateral ventricles as early indicator

dx with MRI

fatal within 10-20 years of pneumonia

22
Q

what is leukodystrophy and the most common type?

A

progressive disruption of myelin sheaths due to defective myelin production in CNS

manifests early in life and rapidly fatal

Adrenoleukodystrophy: very long FA chain buildup and disrupts myelin sheath
x linked disease and impairment of adrenal function (90%)

23
Q

What is tay-sach’s disease

A

autosomal recessive disease and manifests by 6 mos

cherry red spot on macula with white surround

24
Q

what is neimann-pick disease

A

autosomal recessive disease + manifests by 1 year old

cherry red spot on macula

25
Q

Fabry’s disease?

A

x linked disease by 3-10 years old

whorl keratopathy or vortex no effect on vision

26
Q

what is an EEG

A

electroencephalography

- graphic recording of electrical activity of superficial layers of cerebral cortex

27
Q

where do we do a lumbar puncture?

A

insertion of spinal needle between L3-L4 into lumbar subarachnoid space to instill air, dye, meds

28
Q

what is the Blood Brain Barrier and its function?

what is it made up of?

A

reduce capillary permeability and protects brain from hazardous substances

made up of endothelial tight junctions, thickened basement membrane, and astrocytes

Passive Transport: O2, CO2, lipid solubles
Active Transport: glucose, AA, small ions

29
Q

what are the clinical manifestations of pathophysiology of CNS

A

Altered Mental status
Syncope
Localizing Signs
Seizures

30
Q

What are the causes of AMS

A

drugs/alchohol, stroke, infection, syncope, seizures

not a diagnostic - just a finding

31
Q

What is CSF and where is it produced and where does it go? What is its purpose?

A

surrounds the brain and spinal cord
produced by choroid plexus and fills up subarachnoid space

buoyancy + protection + nutrient delivery/metabolic waste removal

32
Q

What is ICP and what are the normal ranges?

How is it regulated?

What can increase it?

A 
Intracranial pressure (pressure inside skull) 
7 - 15 mmHg or 95 - 200 mmH20

Regulated by CSF: production, circulation, drainage and reabsorption

Caused by brain edema/tumor, CSF obstruction, intracranial hemorrhage, idiopathic intracranial HTN (IIH)

33
Q

What is IIH and what is it known for? what is the most common cause of this? how do you dx?

symptoms?

Tx?

A

IIH is idiopathic intracranial HTN (pseudotumor cerebri)

increased ICP idiopathically and decreases veinous drainage

caused by papilledema (OU swelling of optic nerve)

HA + VFD + Overweight (usually)

Tx: decrease weight, oral CAI, Sx, decrease CSF

34
Q

what is hydrocephalus and what are the types?

Symptoms?

Tx?

A

abnormal accumulation of CSF (congenital more common)

Normal Pressure, Non-Communicating, Communicating

Asymptomatic to Severe (sunset eyes, bossing forehead, scalp veins, increase head size)

Tx: ventricular shunt to drain areas and decrease ICP

35
Q

What is meningitis and how is It caused?

A

inflammation of meninges and caused by viral and bacterial infections

36
Q

what are the viruses associated with meningitis

A

enterovirus (GI)

least damaging compared to bacterial meningitis

37
Q

what is the bacteria associated with meningitis

symptoms?
ocular manifestations?

A

more severe than viral
S. Pneumoniae

hearing loss + brain damage

Stiff neck, sudden fever, HA, AMS

Pupil changes, CNP, Fundus Changes (papilledema and retinal hemorrhage)

38
Q

What is encephalitis and what can it lead to?

caused by what?

symptoms?

A

inflammation of the entire brain usually with concomitant meningitis

inflammation leads to cerebral edema and neural dysfunction and brain damage

infants/elderly are more susceptible

usually idiopathic (60%) but can be viral (enterovirus)

seizures, stupor, coma + permanent brain damage + coma

39
Q

what are the types of seizures?

A

Type 1: Partial (focal areas are discharged)

  • Simple: limited + patient is aware
  • Complex: Automatisms

Type 2: Generalized

  • Absence: staring spell, brief, recurrent, patient is unaware
  • Tonic Clonic (Grand Mal): dramatic loss of consciousness and convulsions of all extremities, incontinence, amnesia

Medical Pathology (13 decks)

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