Neurologic System NCCPA outline Latest

Neurologic System (NCCPA outline) (Latest / Questions and Answers 100% Correct

• What is the genetic basis of Huntington disease?

Answer: Expansion of the

*c*ytosine- *a*denine- *g*uanine trinucleotide repeats in the HTT gene

think: you *HUNT* and animals go in a *CAG*e

• PE will show pain in V2 and V3 distributions, not V1

Answer: Trigeminal Neuralgia (Tic douloureux)

• CT scan of someone with Huntington will reveal

Answer: cerebral and caudate nucleus atrophy

• PET scan in someone with Huntington disease

Answer: decreased

glucose metabolism in caudate nucleus and putamen

• - Patient will be a 30 - 50-years-old
• With a history of a family member with similar symptoms
• Complaining of gradual chorea and dementia
• Diagnosis is made by genetic testing
• Most commonly caused by autosomal dominant *CAG trinucleotide repeats*-

Answer: Huntington Disease

• - Patient will be complaining of rigidity, bradykinesia, postural instability, micrographia
• PE will show a resting pill-rolling tremor, mask-like facies, cog wheeling of extremities,

shuffling gait

• Most commonly caused by dopamine depletion in basal ganglia
• Treatment is levodopa-carbidopa

Answer: Parkinson's Disease

• what is a cytoplasmic inclusion?

Answer: Lewy body

• glycogen
• A/W Parkinsons and Lewy Body Dementia

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• Parkinson disease pathophys

Answer: idiopathic *dopamine depletion* [from loss of pigment cells in substantial nigra] leads to *failure to inhibit acetylcholine* in the *basal ganglia* [what causes the movement d.o]

• a/w Lewy bodies (cytoplasmic inclusions)
• *loss of pigment cells seen in the SUBSTANTIA NIGRA*

• loss of pigment cells in substantial nigra

Answer: Parkinson

• Which comorbid psychiatric condition is usually a medication side effect in patient with

Parkinson's disease?

Answer: Psychosis, related to the

"pro-dopamine" or dopamine-agonism convention used in treating Parkinson's dis- ease.

• Extrapyramidal Symptoms
• what causes it?
• what are EPS?
• how do you treat them?

Answer: 1. Typical > atypical antipsychotics

--- Acute dystonia: muscle spasms, stiffness, oculogyric crisis, rx: *benztropine, dyphenhydramine* --- Akathisia: restlessness, rx: *benztropine*, Bs --- Bradykinesia: Parkinsonism, rx: *benztropine*

--- Tardive dyskinesia: orofacial involuntary movements

• - necrosis of both upper and lower motor neruons
• muscle weakness, loss of ability to initiate and control motor movements
• mixed upper and lower motor neuron signs

- UMN: spasticity, stiffness, hyperreflexia

• LMN: progressive bilateral fasiculations, muscle atrophy, hyporeflexia, muscle weakness.
• Bulbar symptoms: dysphagia, dysarthria, speech problems, respiratory dysfunction
• *Sensation, urinary and voluntary eye movements are SPARED*

Answer: Amyotrophic Lateral Sclerosis

note: Riluzole reduces progression for up to 6 months

• CNS disorder a/w muscle tone and postural abnormalities dude to brain injury during

perinatal or prenatal period

• Spasticity = hallmark 2 / 3

• varying degrees of motor deficits
• may have learning disabilities
• may develop seizures

Answer: Cerebral Palsy

Physical exam:

• hyperreeflexia
• limb-length discrepencies
• congenital defects

• How do you manage Cerebral Palsy?

Answer:

• multidisciplinary approach

2. improve spasticity: baclofen, diazepam, anti-epileptic

• CSF of someone with Guillian barre

Answer: high protein with a normal WBC count aka - albuminocytological dissociation

• - Patient with a history of recent minor respiratory or GI illness
• Complaining of symmetric, progressive ascending muscle weakness
• PE will show lack of deep tendon reflexes
• Lumbar puncture results will demonstrate increased CSF protein but a normal cell count
• Most commonly caused by Campylobacter jejuni
• Treatment is supportive, plasmapheresis, or IVIG

Answer: Guillain-Barré Syndrome

• - Patient will be complaining of proximal muscle weakness, *ptosis*, and

*diplopia* that is worse at the end of the day (relieved with rest)

• PE will show *ice test improves sx*
• Diagnosis is made by *edrophonium (tensilon) test*, EMG
• Most commonly caused by *autoimmune destruction of acetylcholine recep- tors*
• Treatment is acetylcholinesterase inhibitors, such as *pyridostigmine*

- Comments: associated with *thymoma*

Answer: Myasthenia Gravis

• Which of the following is true about myasthenia gravis?

A Limb weakness and dysarthria are the most common presenting symptoms B Muscle weakness tends to worsen after long periods of rest C Optic neuritis is commonly associated with the condition D Symptoms are decreased by cooling Answer: D - symptoms are decreased by cooling Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weak- ness and fatigue seen particularly with repetitive use of voluntary muscles. It is caused by antibodies that attack and reduce the number of nicotinic

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