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Neuro Muscular Psych Block 3 > Peripheral Nerve and Skeletal Muscle Path > Flashcards

Peripheral Nerve and Skeletal Muscle Path Flashcards

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1
Q

Segmental Neuropathy

Etiologies (2) Unaffected Structures (2) Characteristic Morphology (1)

A

Schwann cell dysfunction
Direct damage to myelin sheath

Axon and Myocytes untouched

Onion bulb concentric layers of Schwann cytoplasm

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2
Q

Axonal Neuropathies

Morphology (2) Complication with Description (2)

A 
Wallerian degeneration
Myelin ovoids (degrading myelin sheath)

Traumatic neuroma
Painful pseudotumor
Disordered orientation of regrowing axons

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3
Q 
Nerve Regeneration and Muscle Reinnervation Process
Early Compensation (2) Muscle Typing
A

Unaffected axons reinnervate denervated myocytes
Form new motor unit, adhering to type grouping

Motor neuron determines type of muscle fiber

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4
Q

Muscle Fiber Atrophy
Etiologies (4) Morphology (2)
Reactions (2) with Descriptions (2/1)
Regeneration Characteristics (3)

A

Denervation (group atrophy)
Neurodegenerative diseases
Inactivity
Glucocorticoid therapy

Angulated Fibers
Target Fibers

Segmental necrosis: myophagocytosis followed by collagen and fat deposition

Hypertrophy: muscle fiber splitting

Regeneration:
Red cytoplasm
Central nuclei
Stains basophilic

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5
Q

Mononeuritis Multiplex

Etiologies (2) Description

A

Vasculitis (polyarteritis nodosa)
HIV

Nerves damaged in haphazard fashion

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6
Q

Peripheral Nerve Damage
Common Symptom
Anatomic Variants with Descriptions
Mono (1) Poly (3) Polyradiculo (2)

A

Pins and needles pain

Mononeuropathy affects single nerve

Polyneuropathy: multiple nerves
Symmetric Ascending symptomology
Glove and stocking distribution

Polyradiculoneuropathy: multiple nerves and nerve roots
Symmetric proximal and distal symptomology

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7
Q

Bell’s Palsy

Etiology, Associations (2) Presentation

A

CN VII mononeuropathy

Upper respiratory infection
Diabetes mellitus

One sided face droop

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8
Q 
Neurogenic Bladder
Disease Associations (4)
A

Multiple sclerosis
Parkinson’s Disease
Spina Bifida
Diabetes

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9
Q

Guillain-Barre Syndrome

Etiologies (2) Pathogenesis (2) Morphology (4) Presentation (2) CSF Finding (1) Treatment (2)

A

Influenza-like illness
Campylobacter jejuni

Anti-myelin Abs
Macrophages attack Schwann cells at nodes of ranvier

Perivenular and endoneurial inflammatory infiltrate
Segmental demyelination of nerves and nerve roots

Ascending paralysis starting in distal LE
Loss of DTR’s

Elevated Protein in CSF

Plasmapharesis
IV Ig

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10
Q 
Chronic Inflammatory Demyelinating Polyradiculoneuropathy
Clinical Features (3) Morphology
A

Symmetrical mixed sensorimotor neuropathy
Lasts > 2 months
Responsive to corticosteroids

Onion bulb Schwann cells

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11
Q

Leprosy

Pathogenesis (3) Morphology (2) Clinical Features (3) Staining (1)

A

Schwann cells invaded by Mycobacterium leprae
Segmental demyelination/remyelination
Loss of myelinated and unmyelinated axons

Endoneurial fibrosis
Thickened perineural sheaths

Symmetrical polyneuropathy of cool extremities
Loss of pain sensation
Large traumatic ulcers

(+) AFB staining

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12
Q

Diphtheria

Pathogenesis (3) Clinical Features (3)

A

Exotoxin mediated
Selective demyelination of axons going to anterior/posterior root and sensorimotor nerves

Early paresthesias and weakness
Bulbar and respiratory dysfunction
Tonsillar exudate

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13
Q

Diabetic Peripheral Neuropathy

Pathogenesis (3) Morphology (3) Clinical Features (4)

A

Segmental demyelination
Loss of small myelinated fibers and unmyelinated fibers
Decreased axons

Thickened, hyalinized, PAS (+) endoneurial arterioles

Ascending distal symmetric sensorimotor polyneuropathy
Paresthesias
Dysthesias
Autonomic Dysfunction (postural hypotension)

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14
Q

Uremic Neuropathy

Etiology and Clinical Features (2)

A

Renal failure

Distal symmetric neuropathy
Healed by dialysis

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15
Q

Thyroid Associated Neuropathy

Hyper (1) Hypo (2)

A

Hyperthyroidism causes Guillain-Barre-like syndrome

Hypothyroidism causes compression neuropathies
Carpal tunnel

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16
Q

Malignant Neuropathies
Paraneoplastic (2)
B cell Neoplasms: Pathogenesis, Presentation (5)

A

Sensorimotor paraneoplastic syndrome
Small cell lung carcinoma

B Cell Neoplasms:
Deposition of paraprotien between noncompacted myelin lamellae
POEMS: polyneuropathy, organomegaly, endocrinopathy, Monoclonal gammopathies, skin changes

17
Q

Compression Neuropathies
Syndromes (3) with Entrapped Nerve
Other Commonly Entrapped Nerves (2)

A

Carpal Tunnel: Median N

Saturday Night Palsy: Radial N

Morton Neuroma (metatarsalgia): Interdigital N

Ulnar N
Peroneal N

18
Q

Charcot-Marie Tooth

Genetics (3) Presentation (2)

A

Autosomal Dominant
Chr 22 mutation
PMP22 defect

Slowly progressive demyelination starting in second decade

19
Q

Myasthenia Gravis

Etiology, Clinical Features (5) Treatments (3)

A

Ach receptor autoantibodies

Diplopia
Ptosis
Worsened by activity, repeated stimulation
Thymic Hyperplasia (B cells on histo)
Thymoma

Acetylcholinesterase inhibitors
Immunosuppression or plasmapharesis
Thymectomy

20
Q

Lambert Eaton Syndrome

Etiology, Pathogenesis (2) Presentation (2)

A

Paraneoplastic syndrome of small cell lung carcinoma

Autoantibody to Ca2+ channel
Blocks acetylcholine release

Proximal muscle weakness
Improved by activity, repeated stimulation

21
Q

Muscle Fiber Type Characteristics
Action, Activity Type, Specialization
Type I, Type II

A

Type I:
Sustained force
Aerobic exercise
High resistance to fatigue

Type II:
Fast movement
Anaerobic exercise
High power production

22
Q

Dermatomyositis

AutoAbs (3) Morphology (3) Clinical Features (3) Histology, Complications (4)

A

anti-Mi2
anti-Jo1
anti-P155/P140

Upper eyelid rash* (lilac/heliotrope/violet)
Telangiectasias causing rash and periorbital edema
Grotton lesions: scaly, on knuckle/elbows/knees

Proximal muscle weakness (trouble walking up stairs)
Dysphagia
Interstitial lung disease

Perifascicular atrophy*

Visceral cancer
GI ulcers (juvenile)
Calcinosis (juvenile)
Lipodystrophy (juvenile)

23
Q

Polymyositis

Pathogenesis, Morphology(2) Clinical Features (3)

A

CD8+ T cell mediated inflammation (mononuclear infiltrate)

Endomysial inflammation*
Random fiber atrophy*

Adult onset
Myalgias
Proximal limb weakness

24
Q 
Inclusion Body Myositis
Clinical Features (3) Morphology
A

Slow progressive muscle weakness
Quadriceps and Distal Upper Extremities most affected
Dysphagia

Rimmed vacuole inclusions

25
Q

Toxic Myopathies

Examples (5) with Descriptions

A

Statins
Chloroquine and Hydroxychloroquine
Corticosteroids

Thyrotoxic: proximal muscle weakness
Alcohol: Rhabdomyolysis, renal failure

26
Q

X Linked Muscular Dystrophies
Common Genetic Defect and Common Morphology
Duchenne Features (3)
Becker Features (3)

A

Xp21 gene mutation
Pseudohypertrophy: muscle replaced with Adipose, CT

Duchenne: Dystrophin absent
Early symptoms and wheelchair bound by 10 years
Females are carriers

Becker: Dystrophin decreased
Normal lifespan
Cardiac disease

27
Q

Myotonic Dystrophy

Genetics (3) Clinical Description (2) Symptoms (5) Morphology

A

Autosomal dominant
CTG repeat
DMPK defect

Sustained involuntary contraction of muscles
Myotonia elicited by percussion of thenar eminence

Ptosis
Hatchet face
Cataracts
Endocrinopathy
Cardiomyopathy

Ring fibers

28
Q

Emery Dreifuss Syndrome

Genetics (2) Clinical Triad

A

X linked: EMD1
Autosomal Dominant: EMD2

Slowly progressive weakness
Cardiomyopathy
Early contractures

29
Q 
Lipid/Glycogen Metabolism Defects
Common Symptomology (2) Examples with Defects (3)
A

Slowly progressive muscle damage
Symptoms show with exercise and fasting

Carnitine Palmitoyltransferase II deficiency

McArdle Disease: myophosphorylase deficiency

Pompe Disease: Acid Maltase deficiency

30
Q

Mitochondrial Myopathies

Common Clinical Features (3) Examples (4) Common Morphology (2)

A

Increased Creatine Kinsae
Rhabdomyolysis
Extraocular Muscle Defects

Leber Optic Neuropathy
Leigh syndrome
Barth syndrome
Kearns-Sayer syndrome

Ragged red fibers
Paracrystalline parking lot inclusions

31
Q

Spinal Muscular Atrophy

Genetics (3) Pathogenesis, Morphology, Wernig Hoffman Syndrome (5)

A

Autosomal recessive
Chromosome 5 mutation
SMN1 defect

Loss of motor neurons in the Anterior Horn

Panfascicular atrophy

Floppy baby*
Truncal and extremity weakness
Dysphagia
Dsypnea
Death by age 3
32
Q

Malignant Hyperthermia

Mutation, Pathogenesis, Symptoms (3) Antidote

A

RYR1 mutation

Anesthesia triggers increased Calcium release causing hypermetabolic state

Hyperpyrexia
Tachycardia
Muscle spasms

Dantrolene

33
Q

Tuberous Sclerosis

Genetics (3) Clinical Features (8)

A

Autosomal dominant
TSC1: Hamartin defect (Chr 9q34)
TSC2: Tuberin defect (Chr 16p13)

Hamartomas
Renal Angiomyolipomas
Pulomary Lymphangioleiomyomatosis
Cardiac Rhabdomyomas
Angiofibromas
Subungual fibromas
Shagreen patches
Ash-leaf patches
34
Q

Von Hippel Lindau Disease

Genetics (3) Clinical Features (5)

A

Autosomal Dominant
Chromosome 3 mutation
VHL defect

Polycythemia
CNS/Retinal Hemangioblastomas
Cysts
Renal cell carcinoma
Pheochromocytoma
35
Q

Neurofibromatosis
Clinical Features Type 1 (4) Type 2 (3)
Mutations Type 1 and Type 2

A 
Type 1: Chr 17 mutation (neurofibromin)
Neurofibromas (plexiform can become MPNST)
Optic N gliomas
Lisch nodules in iris
Cafe au Lait spots

Type 2: Chr 22 mutation (merlin)
Bilateral CN VIII Acoustic Neuromas (schwannomas)
Meningiomas
Ependymomas

Neuro Muscular Psych Block 3 (7 decks)

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