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STEP 5: Neuro PATHOMA > Dvlm + SC Lesions > Flashcards

Dvlm + SC Lesions Flashcards

1
Q

Neural tube defects arise from an incomplete closure of the NEURAL TUBE. What vitamin deficiency is this associated with?

A

LOW FOLATE LEVELS - Prior to conception

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2
Q

How do you protect a pregnant woman against NEURAL TUBE DEFECTS?

A

Ensure adequate vitamin folate (VitB9) supplementation

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3
Q

How are neural tube defects detected during prenatal care? What is the one exception?

A 
ELEVATED AFP in maternal blood + amniotic fluid 
ELEVATED AchEsterase (Fetal Ach-esterase in CSF flows through defect into amniotic fluid) 
Exception = SPINA BIFIDA OCCULTA
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4
Q

What is the neural tube defect that results as a disruption of the CRANIAL END of the neural tube?

A

ANENCEPHALY = absence of skull + brain

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5
Q

FROG-LIKE APPEARANCE of the fetus - prominent eyes. What neural tube defect is this?

A

ANENCEPHALY (Absence of brain and skull)

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6
Q

How does ANENCEPHALY result in MATERNAL POLYHYDRAMNIOS?

A

Absence of CNS-controlled swallowing centers -> Baby can NOT swallow some of the amniotic fluid

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7
Q

What is the neural tube defect associated with failure of the POSTERIOR VERTEBRAL ARCH to close (i.e. disruption of the CAUDAL end of the tube)?

A

SPINA BIFIDA

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8
Q

Name the 3 types of neural tube defects that resulted from a disruption of the CAUDAL end of the neural tube (i.e. spina bifida).

A

SPINA BIFIDA OCCULTA
MENINGOCELE
MENINGOMYELOCELE

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9
Q

Which neural tube defect presents simply with a SKIN DIMPLE or PATCH OF HAIR overlying the vertebral defect? What will AFP levels show?

A

SPINA BIFIDA OCCULTA - Failure of bony spinal canal to close BUT NO HERNIATION (usually at lower vertebral levels)
NORMAL AFP

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10
Q

Which neural tube defect arises from a CYSTIC PROTRUSION of the MENINGES through the vertebral defect?

A

MENINGOCELE

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11
Q

Which neural tube defect arises from a CYSTIC PROTRUSION of the MENINGES/SPINAL CORD through the vertebral defect?

A

MENINGOMYELOCELE

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12
Q

What is the most common cause of HYDROCEPHALUS in newborns? Where is the blockage?

A

CEREBRAL AQUEDUCT STENOSIS -> Blocks CSF drainage from 3rd into 4th ventricle -> Accumulation of CSF in ventricular space = HYDROCEPHALUS

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13
Q

**UW: How does CONGENITAL HDYROCEPHALUS present in an infant?

A

ENALRGING HEAD CIRCUMFERENCE - Due to cranial suture lines not being fused
BULGING FONTANELLE + MACROCEPHALY + POOR FEEDING + DVLM DELAY + SPASTICITY/HYPER-REFLEXIA

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14
Q

What is the difference between COMMUNICATING and NON-COMMUNICATING HYDROCEPHALUS?

A

COMMUNICATING HYDROCEPHALUS: Normal CSF communication between ventricle system, ABNORMAL CSF resorption (particularly in subarachnoid granulations).
DEFECTIVE CSF resorption can not keep up with normal CSF production

NON-COMMUNICATING (OBSTRUCTIVE) HYDROCEPHALUS: Normal CSF resorption, ABNORMAL communication between ventricles due to space-occupying lesion or tumor in cerebellum/midbrain creating obstruction at Foramen of monroe, cerebral aqueduct, or foramen of luschka/magendie

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16
Q

POSTERIOR FOSSA MALFORMATION: What is the CONGENITAL FAILURE of the CEREBELLAR VERMIS to develop (AGENESIS)?

A

DANDY WALKER MALFORMATION

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17
Q

POSTERIOR FOSSA MALFORMATION: What are two of the most common associations of DANDY WALKER SYNDROME?

A
  1. NON-COMMUNICATING HYDROCEPHALUS (enlarged 4th ventricle)

2. SPINA BIFIDA

18
Q

POSTERIOR FOSSA MALFORMATION: What are the 2 high-yield items seen on an MRI of a DANDY-WALKER MALFORMATION?

A
  1. ABSENCE OF CEREBELLUM

2. MASSIVELY DILATED 4th ventricle (enlarged posterior fossa)

19
Q

POSTERIOR FOSSA MALFORMATION: What is the congenital extension of CEREBELLAR TONSILS through the foramen magnum? Can it present with hydrocephalus?

A

CHIARI II MALFORMATION

YES, it can present with hydrocephalus as it compresses on cerebral aqueduct (AQUEDUCTAL STENOSIS)

20
Q

POSTERIOR FOSSA MALFORMATION: What are the 2 most common associations with CHIARI II MALFORMATION?

A
  1. Associated with LUMBOSACRAL MENINGOMYELOCELE (Paralysis/sensory loss at and below site of lesion)
  2. Associated with SYRINGOMYELIA
21
Q

POSTERIOR FOSSA MALFORMATION: How does CHIARI I MALFORMATION differ from CHIARI II MALFORMATION? What is CHIARI I malformation most likely associated with? What is CHIARI II malformation most likely associated with?

A

CHIARI I: =>3-5 cerebellar tonsilar ectopia (downward displacement)
Most associated with SYRINGOMYELIA (CENTRAL CORD SYNDROME)

CHIARI II: SIGNIFICANT herniation of cerebellar tonsils + vermis + (possibly lower brainstem) + AQUEDUCTAL STENOSIS (HYDROCEPHALUS)
Most associated with LUMBOSACRAL MENINGOMYOCELE

22
Q

What does SYRINGOMYELIA first affect? If it expands, what two areas can it affect next?

A
  1. ANTERIOR COMMISSURE - Bilateral STT fibers carrying pain and temperature sensation = BILATERAL “CAPE-LIKE” loss of pain/temperature sensation in UE (C8-T1)
  2. Expansion 1: LMN of anterior horn: LMN signs (Muscle weakness, atrophy, Decreased reflexes)
  3. Expansion 2: Lateral horn carrying Hypothalamospinal tract (sympathetic nerves) resulting in HORNER SYNDROME
23
Q

What is the clinical triad of HORNER SYNDROME?

A

MIOSIS: Loss of SNS innervation to pupillary dilator muscle = pupillary constriction
PTOSIS: Loss of SNS innervation to superior tarsus muscle = lipid drooping
ANHIDROSIS: Loss of SNS innervation to sweating of face and neck = decreased sweating

24
Q

Pt presents with 2-day history of fever, sore throat, abdominal pain, N/V. Two days later, pt presents with FLACCID PARALYSIS and MUSCLE ATROPHY, weakness with hypotonia, fasciculations, impaired reflexes, negative babinski sign (downgoing toes). What is the underlying etiology?

A

POLIOMYELITIS
Pt had poliovirus infection -> First infected oropharynx -> Small Bowel -> Eventually spreads to CNS from blood -> Damaged ANTERIOR MOTOR HORN contain LMN.

25
Q

Pt has both UMN and LMN signs. What does pt have? What is the early sign of this disease? How do you distinguish this from SYRINGOMYELIA?

A

ALS
Early sign = HAND WEAKNESS/ATROPHY
Distinguishing form syringomyelia: NO LOSS OF PAIN/TEMPERATURE SENSATION as in syringomyelia (which also presents with LMN signs if it expands into the anterior horn)

26
Q

What is the inherited pathology that results in FLOPPY BABY and inevitable death within a few years after birth? What can present similarly? What is the inheritance pattern?

A

WERDNIG-HOFFMAN DISEASE
AR inherited degeneration of ANTERIOR MOTOR HORN
Presents similarly to POLIOMYELITIS

27
Q

What is the most common cause of ALS? What is the rare, but very high yield inherited mutation of familial ALS?

A

MOST CASES are SPORADIC
Mutation of Zn-Cu SUPEROXIDE DISMUTASE

(Normally removes O2 free radicals by converting O2- to H2O2)
No conversion into H2O2 -> Greater O2- radical-mediated neuronal damage

28
Q

What is a degenerative disorder of BOTH the cerebellum + Spinal cord tracts? How does it present?

A

FRIEDREICH ATAXIA
Degen (Cerebellum) = ATAXIA
Degen (SC tracts) = Loss of vibratory sense + proprioception (DCML) + muscle weakness in LE/loss of DTR (LCST)

29
Q

What is the inheritance pattern of FRIEDREICH ATAXIA? What is the underlying genetic mechanism of inheritance? What is the normal function of the mutated protein?

A

AR inheritance pattern of TNR (GAA) - of the FRATAXIN GENE
FRATAXIN = Essential for mitochondrial Fe regulation
LOSS FRATAXIN = IRON BUILDUP = Free radical damage (Fentin reaction)

30
Q

What are the derivatives of NEURAL CREST CELLS? of the NEURAL TUBE WALL? of the NEURAL TUBE LUMEN?

A

NCC = PNS, SCHWANN CELLS
NT WALL = CNS
NT LUMEN = VENTRICLES/ SPINAL CORD (including cauda equina)

31
Q

**UW: Why do infants with CONGENITAL HYDROCEPHALUS present with muscle hypertonicity and hyper-reflexia?

A

HYDROCEPHALUS -> STRETCHING OF PERI-VENTRICULAR PYRAMIDAL TRACTS -> UMN injury -> Hyperspasticity, hypertonicity

32
Q

What is the cardiac association with FRIEDREICH ATAXIA?

A

HCM

33
Q

**UW: What is the inheritance pattern of SPINA BIFIDA?

A

MULTIFACTORIAL INHERITANCE - Genetic + dietary folate deficiency

STEP 5: Neuro PATHOMA (6 decks)

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