Trauma and Demyelinating D/o Flashcards

1
Q

EPIDURAL HEMATOMA: What is seen on CT? What is the chronology of Sx? What is the fatal complication?

A

LENS SHAPED HEMATOMA
LUCID period -> Rapid recurrent unconsciousness = RAPID PROGRESSION
UNCAL HERNIATION = fatal complication

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2
Q

EPIDURAL HEMATOMA: What is the most common location of trauma causing the epidural hematoma? Which artery is most commonly ruptured?

A

TEMPORAL BONE FRACTURE

Middle meningeal artery rupture

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3
Q

SUBDURAL HEMATOMA: What is most common cause of a subdural hematoma? Second most common cause? What is ruptured? How does this clinical presentation differ from EPIDURAL hematoma? What is the fatal complication?

A

Most common cause = TRAUMA to the head
Second most common cause = BRAIN ATROPHY -> resulting in lengthening of the cortical bridging veins

CORTICAL BRIDGING VEIN = Most commonly ruptured
SLOWER progression of Sx than epidural hematoma - Slower leaking blood

Fatal complication = HERNIATION

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4
Q

What are the 3 types of brain herniations (due to mass effect OR increased ICP)

A
  1. CEREBELLAR TONSILLAR HERNIATION
  2. SUBFALCINE HERNIATION
  3. UNCAL HERNIATION
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5
Q

CEREBELLAR TONSILLAR HERNIATION clinically presents as __?

A

Herniation into foramen magnum -> BRAINSTEM COMPRESSION -> CARDIOPULMONARY ARREST

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6
Q

What is a SUBFALCINE HERNIATION?

A

Herniation of CINGULATE GYRUS under the FALX CEREBRI (separating the left and right hemispheres)

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7
Q

What vascular supply does a SUBFALCINE HERNIATION compress?

A

ANTERIOR CEREBRAL ARTERY COMPRESSION -> ACA Infarct

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8
Q

What is an UNCAL HERNIATION?

A

Herniation of the TEMPORAL LOBE UNCUS under the TENTORIUM CEREBELLI (area between the cerebellum and brainstem)

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9
Q

What 3 structures does an UNCAL HERNIATION compress and what are the associated clinical signs?

A
  1. Compression of CN III - Eyes will move DOWN and OUT, DILATED PUPIL (mydriasis)
  2. Compression of POSTERIOR CEREBRAL ARTERY - Infarction of OCCIPITAL LOBE = CONTRALATERAL HOMONYMOUS HEMIANOPSIA
  3. Pull on and Rupture of PARAMEDIAN ARTERY (running along brainstem) = DURET HEMORRHAGES of brainstem
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10
Q

What is a LEUKODYSTROPHY?

A

Inherited mutations in enzymes necessary for PRODUCTION or MAINTENANCE of myelin

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11
Q

What is the most common LEUKODYSTROPHY? What enzyme is deficient? What accumulates as a result?

A

METACHROMATIC LEUKODYSTROPHY - AR mutation of arylsulfatase -> Sulfatides of myelin can NOT get degraded -> MYELIN accumulates in LYSOSOMES of OLIGODENDROCYTES

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12
Q

Other than a LEUKODYSTROPHY, how can METACHROMATIC LEUKODYSTROPHY be categorized? [HINT: Think organelle]

A

LYSOSOMAL STORAGE DISEASE

Accumulation of myelin that could NOT be degraded in OLIGODENDROCYTE LYSOSOMES

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13
Q

Name the 3 LEUKODYSTROPHIES.

A
  1. METACHROMATIC LEUKODYSTROPHY
  2. KRABBE DISEASE
  3. ADRENLEUKODYSTROPHY
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14
Q

What enzyme is deficient in KRABBE DISEASE? What accumulates as a result? Where does it accumulate?

A

BETA GALACTOCERBROSIDASE - Accumulation of GALACTOCEREBROSIDE in MACROPHAGES

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15
Q

What is the inheritance pattern of METACHROMATIC LEUKODYSTROPHY and KRABBE DISEASE?

A

AUTOSOMAL RECESSIVE

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16
Q

What is impaired in ADRENOLEUKODYSTROPHY? What accumulates as a result? Where does it accumulate?

A

Addition of COENZYME A to long chain fatty acids is impaired -> Accumulation of FATTY ACIDS in ADRENAL GLANDS + BRAIN WHITE MATTER

17
Q

What is the inheritance pattern of ADRENOLEUKODYSTROPHY?

18
Q

What is the etiology of MULTIPLE SCLEROSIS? What is its strongest association?

A

AUTOIMMUNE destruction of MYELIN + OLIGODENDROCYTES

Strongest association with HLA-DR2

19
Q

Like other autoimmune conditions, what is the genetic susceptibility + environmental trigger of MS?

A

GENETIC SUSCEPTIBILITY = HLA-DR2

ENVIRONMENTAL TRIGGER = Location - away from the equator

20
Q

**UW: What is a common INITIAL MANIFESTATION of MS? What is affected?

A

OPTIC NEURITIS- Monocular visual loss with pain on eye movement

**UW DECREASED VISION + PAIN AROUND EYE
Due to involvement of OPTIC NERVE

21
Q

What are other possible structures affected with MS and their associated clinical effect?

A
  1. BRAINSTEM: Vertigo + Scanned Speech (Mimics alcohol intoxication)
  2. LEFT MLF: Inability to do a RIGHTWARD gaze with right nystagmus (From functioning eye) - Right eye CAN look right (CN 6),
  3. PERIVENTRICULAR CEREBRAL WHITE MATTER: Hemiparesis/ Unilateral loss of sensation
  4. SPINAL CORD: LE loss of sensation/weakness
    5: AUTONOMIC NERVOUS SYSTEM: Loss of bowel/bladder/sexual function
22
Q

How is the diagnosis of MULTIPLE SCLEROSIS made (2 Diagnostic Tools)? What are the + results?

A
  1. MRI: Multiple OVOID, white matter lesions
  2. LP: INCREASED LYMPHOCYTES + OLIGOCLONAL IgG bands [high-resolution electrophoresis] + MYELIN BASIC PROTEIN (from myelin destruction and protein leakage)
23
Q

NEUROLOGIC SYMPTOMS THAT PRESENT DISSEMINATED IN TIME AND SPACE = ?

A

MULTIPLE SCLEROSIS

24
Q

What is seen on biopsy of MULTIPLE SCLEROSIS?

A

Loss of myelination = GRAY-APPEARING PLAQUES in the white matter

25
What is the Tx of acute MS attacks?
HIGH DOSE STEROIDS
26
What is the long-term Tx of MS?
IFN-beta: Slows progression of disease
27
Pt is a an infant who gets a MEASLES VIRUS infection in infancy. Presents with neurologic signs years later during childhood. What is the most likely diagnosis?
SUBACUTE SCLEROSING PANENCEPHALITIS
28
What is the hallmark defining feature of SUBACUTE SCLEROSING PANENCEPHALITIS?
Subacute, Sclerosing (S,S) = S for SLOWLY progressing, persistent infection of the brain (BOTH WHITE/GRAY MATTER) by MEASLES VIRUS -> Presents with neuro Sx later during childhood -> FATAL
29
What is the diagnostic test confirming SUBACUTE SCLEROSING PANCENPHALITIS?
VIRAL INCLUSIONS of MEASLES VIRUS within GRAY MATTER NEURONS + WHITE MATTER OLIGODENDROCYTES
30
What is PROGRESSIVE MULTIFOCAL PANENCEPHATLITIS? What viral infection is it associated with? What is the trigger of this disease?
JC (POLYOMAVIRUS) VIRAL INFECTION of OLIGODENDROCYTES (WHITE MATTER - MULTIPLE FOCI) Normally LATENT infection REACTIVATION in an immunosuppressed pt (e.g. AIDS or LEUKEMIA pt receiving immunosuppression)
31
What is the classic presentation of PROGRESSIVE MULTIFOCAL PANENCEPHALITIS?
PROGRESSIVE = RAPIDLY PROGRESSIVE neurologic sx (vision loss, weakness, dementia) resulting in DEATH
32
What is CENTRAL PONTINE MYELINOLYSIS?
FOCAL Demyelination of CENTRAL PONS (anterior brainstem)
33
What is the most common cause of CENTRAL PONTINE MYELINOLYSIS? Who is the typical pt?
RAPID IV CORRECTION OF HYPONATREMIA | In severely malnourished pt (LIVER DISEASE, ALCOHOLIC)
34
What is the classic presentation/ syndrome presented with CENTRAL PONTINE MYELINOSIS?
LOCKED IN SYNDROME = Acute bilateral paralysis (HEAD ->TOE) except for the EYES VERTICAL EYE MOVEMENTS + EYELID ELEVATION SPARED (cn 3,4,6 arise from the tegmentum of the midbrain = rostral to the pons)
35
What are the 5 demyelinating disorders?
1. LEUKODYSTROPHY - Metachromatic (myelin in lysosomes), Krabbe (galactocerebroside in macrophages), Adrenoleukodystrophy (fatty acids in adrenal glands + white matter) 2. MULTIPLE SCLEROSIS - autoimmune [myelin + oligodendrocytes] 3. SUBACUTE SCLEROSING PANENCEPHALITIS: Measles virus affecting BOTH White/Gray matter 4. PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY: Re-activation of Latent JC virus affecting White matter multifocally 5. CENTRAL PONTINE MYELINOSIS: Rapid IV correction of HYPONATREMIA, LOCKED IN SYNDROME