Diseases

Question 1

Chronic degenerative neurological disease that causes demyelination of the spinal cord. Disease involves hyperreflexia, spasticity, weakness of lower limbs & gait abnormalitiy
Occurs between 30-40 years and more common in females

Answer 1

Tropical Spastic Paraparesis (TSP)

Question 2

Etiology of Tropical Spastic Paraparesis

Answer 2

Caused by HTVL-1 Associated Myelopathy (HAM) Virus

Transmitted through placenta, blood transfusion, breastfeeding, contaminated needles and sex

Question 3

Treatment of Tropical Spastic Paraparesis

Answer 3

Incurable but cortciosteroids have improved symptoms

Question 4

Viral Infection primarily attacking ventral spinal grey matter.
Acute Flaccid Paralysis & Profound Asymmetrical Muscular Weakness
Destroyed motor neurons & denervated muscles

Answer 4

Poliomyelitis (Soma)

Question 5

Etiology of Poliomyelitis

Answer 5

Small RNA viruses of enterovirus genus of Picornaviridae family
Transmission via fecal-oral route & aerosol droplets

Question 6

Toxic anaerobic bacterial protease that reduces ACh release by acting on presynaptic exocytotic proteins

Answer 6

Botulinum Toxin

Question 7

Toxin in venom of black widow spider that causes massive release of ACh by affecting presynaptic exocytotic proteins

Answer 7

Alpha-Latrotoxin

Question 8

Toxin in venom of snake initially provokes ACh release followed by ACh depletion by acting on proteins in nerve terminals involved in exocytosis

Answer 8

Beta-Bungarotoxin

Question 9

Blocks nAChRs (blow darts)

Answer 9

Curare (delta-tubocurarine)

Question 10

Autoimmune disorder, antibodies attack presynaptic voltage gated calcium channels, waxing response to EMG

Answer 10

Lambert Eaton Syndrome

Question 11

Lambert Eaton Syndrome Characteristics

Answer 11

Muscle weakness & facilitated neuromuscular block
Often in patients with pulmonary small cell cancer
Reduced ACh release

Question 12

Types of Congenital Myasthenias

Answer 12

1. Deficiency of ACh-esterase in synaptic cleft at end plate
2. Prolonged opening of channels
3. Abnormal binding of ACh to nAChR
4. ACh-gated channels have brief opening times

Question 13

Chronic autoimmune disorder where autoantibodies to nAChRs develop and reduce transmission at NMJ
Weakness, Fatigability, Temporary Restoration after rest, waning response to EMG

Answer 13

Myasthenia Gravis

Question 14

Autosomal Dominant
Influencing gene encoding chloride channels in muscle membrane
Increased Excitability

Answer 14

Myotonia Congenita (lesion of the chloride channel)

Question 15

X-linked Recessive Disorder
Absence of Muscle Protein, Dystrophin
Die from Cardiovascular & Respiratory Insufficiency

Answer 15

Duchenne Muscular Dystrophy

Question 16

Positive Manifestations of PNS Disease

Answer 16

Fasiculations
Parathesia
Brief Periods of Pain (Trigeminal Neuralgia)

Question 17

Negative Manifestations of PNS Disease

Answer 17

Paralysis/ Paresis
Areflexia/ Hyporeflexia
Anhidrosis
Numbness

Question 18

Acute inflammatory polyradiculoneuropathy with resultant rapid ascending motor and sensory loss 2-3 weeks after respiratory or GI illness
Causes rapid demyelination of peripheral neurons

Answer 18

Guillian-Barre Syndrome

Question 19

Painless skin patch accompanied by loss of sensation but not itchiness
Wasting & muscle weakness

Answer 19

Leprosy (Hansen Disease)

Question 20

Motor neuropathy in adults but encephalopathy in children younger than 6
Bilateral focal weakness and wasting of extensor muscles of fingers, wrist and arms

Answer 20

Lead Poisioning

Question 21

The lens looses its elasticity during aging, thereby reducing the ability to focus on near objects

Answer 21

Presbyopia

Question 22

Diseases affecting soma

Answer 22

Lou Gehrig’s disease (ALS)

Poliomyelitis

Question 23

Diseases affecting nerve ending

Answer 23

Botulism
Alpha-Latrotoxin
Beta-Bungarotoxin
Curare

Question 24

Diseases affecting schwaan cells

Answer 24

Guillain-Barre

Diphtheria

Question 25

Diseases affecting end plate

Answer 25

Myasthenia Gravis | nAChR defects

Question 26

Diseases affecting synaptic cleft

Answer 26

Myasthenias

Question 27

Diseases affecting muscle fiber

Answer 27

Myotonias | Muscular dystrophy

Question 28

irregular spontaneous contractions of the muscle fibers in motor units giving visible twitches at surface of body

Answer 28

Fasciculations

Question 29

are spontaneous contractions of single muscle fibers (not visible)

Answer 29

Fibrillations (need to be tested with EMG)

Question 30

syphilitic myelopathy | significant demyelination of neural tracts in the dorsal root and dorsal column

Answer 30

Tabes dorsalis

Question 31

Tabes dorsalis Symptoms

Answer 31

ipsilateral paresthesia and/or reduced touch in dermatomal pattern gait disturbances (diminished proprioception) diminished patellar reflex involvement of DRG cells leads to short, shooting pain

Question 32

Fluid filled cavity within the central canal of the spinal cord progressive expansion interrupts fibers that cross through the anterior white commisure

Answer 32

Syringomyelia

Question 33

lesion of one half of the spinal cord (hemisection)

Answer 33

Brown Sequard Syndrome

Question 34

symptoms of anterior spinal artery syndrome

Answer 34

Paraparesis Bilateral Extensor Plantar Response Bilateral loss of pain & temp below lesion Touch, vibration & proprioreception intact Retention of urine Sexual functions impaired

Question 35

symptoms of syringomyelia

Answer 35

unilateral or bilateral paralysis of upper extremities | loss of pain & temp in affected spinal cord- cap like distribution of sensory loss with cervical involvement