Neuro - Pathology (Demyelinating/Dysmyelinating disorders & Adrenoleukodystrophy) Flashcards Preview

FA - Neurology > Neuro - Pathology (Demyelinating/Dysmyelinating disorders & Adrenoleukodystrophy) > Flashcards

Flashcards in Neuro - Pathology (Demyelinating/Dysmyelinating disorders & Adrenoleukodystrophy) Deck (33)
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1
Q

What processes are occurring in multiple sclerosis?

A

Autoimmune inflammation and demyelination of CNS (brain and spinal cord).

2
Q

What are the 5 main components of presentation that can be seen in multiple sclerosis patients?

A

Patients can present with (1) optic neuritis (sudden loss of vision resulting in Marcus Gunn pupils), (2) Internuclear ophthalmoplegia, (3) Hemiparesis, (4) Hemisensory symptoms, or (5) Bladder/bowl incontinence.

3
Q

Describe (very briefly) the course of multiple sclerosis.

A

Relapsing and remiting course

4
Q

In what patient population is multiple sclerosis most common? In which racial/ethnic group is multiple sclerosis more common?

A

Most often affects women in their 20s and 30s; More common in whites

5
Q

What CSF finding characterizes multiple sclerosis?

A

Increased protein (IgG) in CSF. Oligoclonal bands are diagnostic.

6
Q

What imaging modality is the gold standard for multiple sclerosis? What are 2 major imaging findings that may be seen?

A

MRI is gold standard; (1) Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with destruction of axons. (2) Multiple white matter lesions separated in space and time

7
Q

What are 3 main methods of treatment for multiple sclerosis? What are 3 additional symptomatic treatments?

A

(1) Beta-interferon (2) Immunosuppression (3) Natalizumab; Symptomatic treatment for (1) neurogenic bladder (catheterization, muscarinic antagonists), (2) spasticity (baclofen, GABA receptor agonist), (3) pain (opioids)

8
Q

What is the most common variant of Guillain-Barre syndrome?

A

Acute inflammatory demyelinating polyradiculopathy.

9
Q

What is Acute inflammatory demyelinating polyradiculopathy?

A

Autoimmune condition that destroys Schwann cells –> inflammation and demyelination of peripheral nerves and motor fibers

10
Q

What are 3 categories of deficits to associate with acute inflammatory demyelinating polyradiculopathy?

A

(1) Results in symmetric ascending muscle weakness/paralysis beginning in lower extremities. (2) Facial paralysis in 50% of cases. (3) Autonomic function may be severely affected (e.g., cardiac irregularities, hypertension, or hypotension).

11
Q

Describe the survival rate and recovery time of patients with Acute inflammatory demyelinating polyradiculopathy.

A

Almost all patients survive; the majority recover completely after weeks to months

12
Q

What is the CSF finding associated with Acute inflammatory demyelinating polyradiculopathy? What physical sign is also found in Acute inflammatory demyelinating polyradiculopathy, and what causes it?

A

Findings: Increased CSF protein with normal cell count (albuminocytologic dissociation); Increased protein –> papilledema

13
Q

Name 2 infections with which acute inflammatory demyelinating polyradiculopathy is associated. Explain this association.

A

Associated with infections (Campylobacter jejuni and CMV) –> autoimmune attack of peripheral myelin due to molecular mimicry, inoculations, and stress, but no definitive link to pathogen.

14
Q

What intervention is critical until recovery of Acute inflammatory demyelinating polyradiculopathy patients? What are 2 additional treatments?

A

Respiratory support is critical until recovery; Additional treatment: Plasmapharesis, IV immune globulins.

15
Q

What is the name of the classic triad of MS? What symptoms are included in this triad?

A

Charcot classic triad of MS is a SIN: Scanning speech, Intention tremor (also Incontinence and Internuclear ophthalmoplegia), Nystagmus.

16
Q

What is Progressive multifocal leukoencephalopathy (PML)?

A

Demyelination of CNS due to destruction of oligodendrocytes.

17
Q

With what pathogen is Progressive multifocal leukoencephalopathy associated?

A

Associated with JC virus.

18
Q

In what patient population is PML seen, at what percentage of this population, and why?

A

Seen in 2-4% of AIDS patient (reactivation of latent viral infection)

19
Q

Describe the progression and prognosis of PML.

A

Rapidly progressive, usually fatal.

20
Q

What drug is associated with increased risk of PML?

A

Increased risk associated with natalizumab.

21
Q

What is another name for Acute disseminated encephalomyelitis? What characterizes this condition?

A

Acute disseminated (postinfectious) encephalomyelitis; Multifocal perivenular inflammation and demyelination after infection (commonly measles or VZV) or certain vaccinations (e.g., rabies, smallpox)

22
Q

Name 2 infections and 2 vaccines associated with Acute disseminated (postinfectious) encephalomyelitis.

A

Infections: (1) Measles (2) VZV; Vaccines: (1) Rabies (2) Smallpox

23
Q

What kind of disease is Metachromatic leukodystrophy, and what mode of inheritance does it have? What is the most common cause of it?

A

Autosomal recessive lysosomal storage disease, most commonly due to arylsulfatase A deficiency

24
Q

What is the underlying pathophysiology of Metachromatic leukodystrophy?

A

Buildup of sulfatides –> impaired production of myelin sheath

25
Q

What are physical exam findings associated with metachromatic leukodystrophy?

A

Findings: Central and peripheral demyelination with ataxia, dementia

26
Q

What is another name for Charcot-Marie-Tooth disease?

A

Also known as hereditary motor and sensory neuropathy (HMSN).

27
Q

Define Charcot-Marie-Tooth disease.

A

Group of progressive hereditary nerve disorders related to the defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath.

28
Q

What is the mode of inheritance of Charcot-Marie Tooth disease? What are 2 physical findings associated with it?

A

Typically autosomal dominant inheritance pattern and associated with scoliosis and foot deformities (high or flat arches)

29
Q

What kind of disease is Krabbe disease? What is its pathogenesis?

A

Autosomal recessive lysosomal storage disease due to deficiency of galactocerebrosidase. Buildup of galactocerebroside and psychosine destroys myelin sheath.

30
Q

What are 4 findings associated with Krabbe disease?

A

Findings: (1) Peripheral neuropathy (2) Developmental delay (3) Optic atrophy (4) Globoid cells

31
Q

What type of disorder is Adrenoleukodystrophy, and what patient population does is typically affect?

A

X-linked genetic disorder typically affecting males

32
Q

What is the pathogenesis of Adrenoleukodystrophy?

A

Disrupts metabolism of very-long-chain fatty acids –> excessive buildup in nervous system, adrenal gland, and testes

33
Q

What are 2 complications to associate with the course of Adrenoleukodystrophy?

A

Progressive disease that can lead to long-term coma/death and adrenal gland crisis

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