Neuro 3 (Final Exam GBS, Post polio,
CMT, ALS) Verified 2024
90% of ALS is _
5-10 % of ALS is - ANSWER-Sporadic
Familial inherited (X-Linked dominant)
ALS
Also Known as?
Described as?
Average life span - ANSWER-"Lou Gehrigs Disease"
-Fatal, progressive, neuromuscular disease; motor neurons between brain and SC stop
functioning from an unknown etiology
-Following diagnosis =2-5 years; Average diagnosis age= 53 to 65 years old
ALS
How to diagnose
4 types of onset
What is used as a measurement of trajectory of disease - ANSWER-No specific tests,
based on ruling out other diagnoses
- Bulbar, Limb, Respiratory, and Cognitive
-FVC
ALS
Medical Management
Why do majority of deaths occur
Greater incidence in - ANSWER-NO cure, only symptom management
-Respiratory failure, less common are due to malnutrition, swallowing problems, PEs,
cardiac arrhythmias, and pneumonia
-Men until 65- 60% in veterans
ALS drugs
Riluzole
Nuedexta
Radicava - ANSWER-R- increase survival by 2 to 3 months
N- May help bulbar symptoms
R- New, may decrease slope of delcine
ALS FVC and what it means - ANSWER-50%= NIV
40%=Advanced directive discussion
30%= Hospice discussion
Charcot Marie Tooth
, S/S - ANSWER-Symmetrical insidious weakness and atrophy of intrinsic foot and
peroneal muscles
-Equinovarus foot deformities, pes cavus defomities, and high steppage gait
-Sensory changes not often reported, show up in careful examination
Charcot Marie Tooth
How to diagnose
Medical Management
Outcome measures - ANSWER--Genetic testing, blood studies- CK is normal, EMG and
Nerve conduction studies- decreased velocity, differential diagnosis of other auto-
immune disorders
-NO cure or effective management: OT and PT are preferred treatment
-CMT neuropathy (CMTNS) and Overall Neuropathy Limitations Scale (ONLS)
CMT 1 - ANSWER-Hypertrophic demyelination neuropathy (MOST COMMON)
CMT 2 - ANSWER-Axonal neuropathy with normal or near normal nerve conduction
velocities (LESS COMMON)
CMT 3 - ANSWER-Dejerine-Sottas Disease (hypertrophic demyelination with onset in
infancy)
Explain Charcot Marie Tooth
What occurs
-How it is inherited - ANSWER-Hereditary motor and sensory neuropathy
-Progressive loss of myelinated fibers and sclerosis in the posterior column of the spinal
cord and peripheral nerves
-Common inherited neurological disease, Autosomal Dominant CMT1, Autosomal
recessive, X-linked dominant/recessive
Explain GBS
What is affected
How does it present
Prognosis - ANSWER-Rapidly evolving, symmetrical ascending weakness
-Spinal roots and peripheral nerves are infiltrated with macrophages and T lymphocytes
(myelin sheath affected or bypassed and axon itself attacked)
-Neuropathy and flaccid paralysis
-Good, worse if patient on mechanical vent
First priority for those with ALS
Why? - ANSWER-Establish a Yes/No and maybe system
-It can save the individuals life
GBS
% that require Mechanical Ventilation
% that have CN involvement
CMT, ALS) Verified 2024
90% of ALS is _
5-10 % of ALS is - ANSWER-Sporadic
Familial inherited (X-Linked dominant)
ALS
Also Known as?
Described as?
Average life span - ANSWER-"Lou Gehrigs Disease"
-Fatal, progressive, neuromuscular disease; motor neurons between brain and SC stop
functioning from an unknown etiology
-Following diagnosis =2-5 years; Average diagnosis age= 53 to 65 years old
ALS
How to diagnose
4 types of onset
What is used as a measurement of trajectory of disease - ANSWER-No specific tests,
based on ruling out other diagnoses
- Bulbar, Limb, Respiratory, and Cognitive
-FVC
ALS
Medical Management
Why do majority of deaths occur
Greater incidence in - ANSWER-NO cure, only symptom management
-Respiratory failure, less common are due to malnutrition, swallowing problems, PEs,
cardiac arrhythmias, and pneumonia
-Men until 65- 60% in veterans
ALS drugs
Riluzole
Nuedexta
Radicava - ANSWER-R- increase survival by 2 to 3 months
N- May help bulbar symptoms
R- New, may decrease slope of delcine
ALS FVC and what it means - ANSWER-50%= NIV
40%=Advanced directive discussion
30%= Hospice discussion
Charcot Marie Tooth
, S/S - ANSWER-Symmetrical insidious weakness and atrophy of intrinsic foot and
peroneal muscles
-Equinovarus foot deformities, pes cavus defomities, and high steppage gait
-Sensory changes not often reported, show up in careful examination
Charcot Marie Tooth
How to diagnose
Medical Management
Outcome measures - ANSWER--Genetic testing, blood studies- CK is normal, EMG and
Nerve conduction studies- decreased velocity, differential diagnosis of other auto-
immune disorders
-NO cure or effective management: OT and PT are preferred treatment
-CMT neuropathy (CMTNS) and Overall Neuropathy Limitations Scale (ONLS)
CMT 1 - ANSWER-Hypertrophic demyelination neuropathy (MOST COMMON)
CMT 2 - ANSWER-Axonal neuropathy with normal or near normal nerve conduction
velocities (LESS COMMON)
CMT 3 - ANSWER-Dejerine-Sottas Disease (hypertrophic demyelination with onset in
infancy)
Explain Charcot Marie Tooth
What occurs
-How it is inherited - ANSWER-Hereditary motor and sensory neuropathy
-Progressive loss of myelinated fibers and sclerosis in the posterior column of the spinal
cord and peripheral nerves
-Common inherited neurological disease, Autosomal Dominant CMT1, Autosomal
recessive, X-linked dominant/recessive
Explain GBS
What is affected
How does it present
Prognosis - ANSWER-Rapidly evolving, symmetrical ascending weakness
-Spinal roots and peripheral nerves are infiltrated with macrophages and T lymphocytes
(myelin sheath affected or bypassed and axon itself attacked)
-Neuropathy and flaccid paralysis
-Good, worse if patient on mechanical vent
First priority for those with ALS
Why? - ANSWER-Establish a Yes/No and maybe system
-It can save the individuals life
GBS
% that require Mechanical Ventilation
% that have CN involvement
