Nerve/Muscle Disorder Pathology Flashcards Preview

MOHD4: Exam 5 > Nerve/Muscle Disorder Pathology > Flashcards

Flashcards in Nerve/Muscle Disorder Pathology Deck (40):
1

What four things contribute to ALS

Abnormal protein aggregates
Excitotoxicity
ROS and MT dysfunction
Inflammation

2

Clinical presentation of neuromuscular disease

weakness, sensory loss, pain

3

Neurogenic disease

peripheral nerve cell bodies, peripheral nerves, neuromuscular junction

4

Autoimmune neuromuscular junction disease

Myasthenia gravis

5

Motor unit includes...

Cell body, axon, every muscle fiber innervated by axon

6

Myopathic diseases

Muscular dystrophy
Inflammatory myositis
Metabolic/toxic storage diseases
Congenital myopathy

7

Wallerian degeneration (i.e. anterograde)

trauma + vascular disease cause axon distal to injury to degenerate

8

Distal axonopathy

systemic metabolic disease, toxin or genetic disease causes distal axon to die

9

Peripheral nerve biopsy can be helpful for...

mononeuropathy multiplex

10

mononeuropathy multiplex biopsy findings

Vasculitis with fibrinoid necrosis, hemorrhage

11

Genetic neuromuscular junction disorder

congenital myasthenic syndromes

12

X-linked recessive muscle disorders

Duchenne-Becker
Emery-Dreifuss

13

Autosomal Dominant

Limb-girdle
Myotonic dystrophy

14

Autosomal Recessive

Limb-girdle
Most congenital

15

Muscular Dystrophy proteins compose

ECM proteins
Sarcolemma-associated
Sarcomere-associated
Glycosyltransferases for a-dystroglycan
Nuclear-envelope associated

16

Metabolic myopathic disorders

Rhabdomyolysis
Glycogen storage disease
Pompe disease
Mitochondrial myopathy

17

Microvascular disorder with perifascicular atrophy

Dermatomyositis

18

fibrosis and fatty replacement, rimmed vacuoles

inclusion body myositis

19

Motor neuron disease (ALS and SMA) is a...

Neurogenic disease

20

DMD/BMD are _______ diseases

Myopathic

21

In a peripheral neuropathy, the pathology is in the

Axon

22

Myopathic neuromuscular diseases

NMJ
Muscle

23

Distal symmetrical polyneuropathy appears as

stocking-glove distribution

24

Myasthenia gravis is a ____genic neuromuscular disease that affects the _______________

Neurogenic

NMJ

25

Neurogenic neuromuscular diseases

Peripheral nerve cell bodies
Peripheral nerves
NMJ

26

Hereditary peripheral myelin disorders

Charcot-Marie-Tooth diseases (many genes)

27

Two neuropathies that can be identified with biopsy

Vasculitis, Amyloidosis

28

Electron microscopy shows great variance in myelination around axons, layers of Schwann cell processes

Charcot-Marie-Tooth

29

Hereditary myelin disorder in the CNS myelin protein gene

Leukodystrophies

30

Loss of junctional folds is seen in

Congenital myasthenic syndromes

31

Internally placed nuclei are characteristic of ______ disease

myopathic

32

Path findings in NEUROGENIC disease

Atrophy: angulated fibers, fiber type groups, pyknotic nuclear clusters
Fiber type regrouping

33

Nemaline rods are shown as

Little dots/aggregates

34

Path findings in MYOPATHIC disease

atrophy/hypertrophy (rounded fibers, scattered)
internally placed nuclei
inflammation
fiber necrosis
regeneration
endomysial fibrosis

35

In motor neuron disease, the cell body

dies

36

Fiber type regrouping is characteristic of ______ disease

Neurogenic

37

Type 1 muscle fibers

One mighty slow fat red ox
sustained, weight bearing
slow myosin heavy chain
abundant lipids, scant gluc
NADH dark

38

Inflammation occurs in _______ disease

Myopathic

39

Angulated fibers are characteristic of _______ disease

Neurogenic

40

Ragged red fibers are associated with

Mitochondrial myopathies