PNS

Question 0

Morphology:

Albuminocytologic dissociation in CSF

Answer 0

GBS

Question 1

Immune-mediated demyelinating neuropathy asso with Campylobacter jejuni
- Ascending motor paralysis

Answer 1

Guillain-Barre Syndrome

Question 2

Increased CSF protein in the absence of increased WBCs

Answer 2

Albuminocytologic Dissociation

Question 3

Aka Charcot-Marie-Tooth Disease

Answer 3

Hereditary Motor and Sensory Neuropathy

Question 4

Distal symmetric neuropathy
Axonal neuropathy
Segmental demyelination

Answer 4

Diabetic neuropathy

Question 5

aka Infantile Motor Neuron Disease

Answer 5

Spinal Muscular Atrophy

Question 6

Destruction of anterior horn cells
Mutations affecting SMN1
Panfascicular atrophy

Answer 6

Spinal muscular Atrophy

Question 7

Floppy Baby Syndrome

Answer 7

Spinal Muscular Atrophy

Question 8

Muscular fibers undergo degeneration and are replaced by fibrofatty tissue and collagen
Mutations in gene coding for Dystrophin

Answer 8

Muscular Dystrophy

Question 9

Types of Muscular Dystrophy

Answer 9

Duchenne Muscular Dystrophy

Becker Muscular Dystrophy

Question 10

Fibrofatty replacement of muscles
Proliferation of endomysial connective tissue
Degeneration of muscle fibers

Answer 10

Muscular Dystrophy

Question 11

Clinical Features of Muscular Dystrophy

Answer 11

Gower Sign

Calf Pseudohypertrophy

Question 12

Sustained involuntary contraction of muscles

Asso with CTG trinucleotide repeats

Answer 12

Myotonic Dystrophy

Question 13

Ring fiber

Sarcoplasmic mass

Answer 13

Myotonic Dystrophy

Question 14

Sarcolemmal band of cytoplasm

Answer 14

Ring Fiber

Question 15

Irregular mass of sarcoplasm

Answer 15

Sarcoplasmic mass

Question 16

MELAS Syndrome

Answer 16

Mitochondral Encephalomyopathy, Lactic Acidosis, Stroke-like Episodes

Question 17

Proximal muscle weakness
External ophthalmoplegia
Neurologic Symptoms

Answer 17

Mitochondrial Myopathies (MELAS)

Question 18

Aggregates of abnormal mitochondria
RAGGED FIBERS
PARKING LOT INCLUSIONS

Answer 18

Mitochondrial myopathies

Question 19

irrrgular fiber with subsarcolemm collections of mitochondria

Answer 19

RAGGED FIBERS

Question 20

alterations in structure of cristae

Answer 20

PARKING LOT INCLUSIONS

Question 21

Inflammatory disorder of the SKIN and SKELETAL MUSCLE

Answer 21

Dermatomyositis

Question 22

Lilac or HELIOTROPE rash on eyelids with periorbital edema
Dusky patched over the knuckles, elbows and knees (Gottron lesions)
PROXIMAL muscle weakness

Answer 22

Dermatomyositis

Question 23

Inflammatory disorders that present as Proximal Weakness

Answer 23

Dermatomyositis

Polymyositis

Question 24

Lacks cutaneous involvement Occurs mainly in adults Proximal muscle weakness

Answer 24

Polymyositis

Question 25

Asymmetric DISTAL muscle weakness | Adults age > 50

Answer 25

Inclusion Body Myositis

Question 26

PERIFASCICULAR ATROPHY

Answer 26

Dermatomyositis

Question 27

PANFASCICULAR ATROPHY

Answer 27

Spinal Muscular Atrophy -> Pan | Polymyositis -> Pan

Question 28

rimmed vacuoles (inclusion bodies)

Answer 28

Inclusion Body Myositis

Question 29

Immune-mediated loss of Ach Receptors due to Antibodies Asso with thymic hyperplasia and thymoma Morpho: type 2 fiber atrophy Immune complex deposition along postsynaptic membrane

Answer 29

Myasthenia Gravis