Neurology #3 Flashcards
Although a Bell Palsy may be idiopathic, it may also be related to
A herpes simplex virus reactivation
Symptoms of a Bell Palsy
-ipsilateral hyperacusis (ear pain)
-unilateral facial weakness or paralysis involving the forehead
-taste disturbance
(weakness and paralysis ONLY affects the face)
Although no treatment is required for a Bell Palsy, what medication, if started in the first 72 hours of symptom onset, reduces the time to fully recovery and increases likelihood of complete recuperation?
Prednisone
What is the pathophysiology Guillain Barre Syndrome?
Autoantibody attacks the myelin sheath of peripheral nerves after an infection
Etiologies of Guillain Barre Syndrome
- Campylobacter Jejuni (MC)
- GI or respiratory infections
- EBV, HIV
- Immunizations
Symptoms of Guillain Barre Syndrome
- Symmetric ascending weakness and sensory changes
- Weakness of respiratory muscles (breathing difficulties)
- Decreased DTRs
Diagnostics for Guillain Barre Syndrome
- Electrophysiologic studies: nerve conduction and needle electromyography (most specific)
- CSF analysis: high protein with normal WBC count
Treatment for Guillain Barre Syndrome
- Plasmapheresis or IVIG are first line
- Mechanical ventilation if needed
Myasthenia gravis is an autoimmune peripheral nerve disorder due to autoantibodies against
the acetylcholine receptor at neuromuscular junction on muscles, leading to weakness
Myasthenia Gravis is MC in young women and older men. It has a strong association with what genetic things?
- Abnormal thymus gland (thymoma)
- HLA-B8
- HLA-DR3
Symptoms of Myasthenia Gravis
- Ocular weakness: diplopia and ptosis
- Generalized weakness worse with repeated muscle use
- Respiratory weakness = myasthenia crisis
In an outpatient setting, what is the diagnostic of choice for myasthenia gravis?
- Acetylcholine receptor antibodies (initial)
- MuSK antibodies if ACR negative
- Electrophysiology testing: most accurate
- CXR, CT, MRI to determine if thymus gland abnormal in all patients
Diagnostics for Myasthenia Gravis in an emergent setting
- Edrophonium (Tensilon) Test: brief improvement after administration
- Ice pack test: to improve ocular symptoms
If the patient is having a myasthenic crisis, what is the treatment?
-Plasmapheresis or IVIG
For long-term treatment of myasthenia gravis, use…
-Acetylcholinesterase inhibitors: Pyridostigmine or Neostigmine
What are some medications that can exacerbate myasthenia gravis?
- Fluoroquinolones (-floxacin)
- Aminoglycosides (gentamicin, tobramycin, etc.)
- Beta Blockers
Multiple sclerosis is an autoimmune, inflammatory demyelinating disease of the CNS that is associated with
axon degeneration of the white matter
MC type of multiple sclerosis
-Relapsing-remitting: episodic exacerbations
Symptoms of Multiple Sclerosis
- Sensory disturbances followed by weakness and visual disturbances
- Diplopia, optic neuritis
- Trigeminal neuralgia
Physical exam findings of a patient with MS
-Upper motor neuron signs: spasticity, upward Babinski, hyperreflexia, rigidity
Regarding MS, what is Lhermitte’s Sign?
Neck flexion causes lightning-shock type pain radiating from the spine down the leg
What is Uhtoff’s Phenomenon in MS?
-Worsening of symptoms with heat (exercise, fever, hot tub, weather, etc.)
What is a Marcus-Gunn Pupil in MS?
-during swinging flashlight test, from unaffected eye into the affected eye, the pupils dilate
Best initial and most accurate diagnostic for Multiple Sclerosis? What does it show?
MRI with gadolinium
-Hyperintense white matter plaques
However, what does an LP show in MS? This is only performed if the MRI is negative
-Increased IgG and oligoclonal bands
First line treatment for MS
IV glucocorticoids (high dose) -Plasmapheresis if no response to IV glucocorticoids
What are the first line medications given to prevent relapse and progression of MS
-Beta-interferon or Glatiramer
Most benign type of all astrocytomas and are most common in children
-Pilocytic Astrocytoma
MC primary CNS tumor in adults
Glioblastoma Multiforme
Symptoms of an astrocytoma
- Headaches that wake patients up at night
- Increased intracranial pressure: headache, nausea, vomiting, stupor, ataxia
Diagnostics for astrocytoma
- CT or MRI with contrast
- Brain biopsy: most diagnostic
Treatment for astrocytoma
-Surgical excision (chemo and radiation as well for Grade IV)
What is the study of choice for a glioblastoma multiforme (the most common and most aggressive primary malignant CNS tumor in adults)?
MRI brain with contrast
What does the brain MRI with contrast show?
Variable ring of enhancement with central necrosis and irregular margins
Treatment for glioblastoma multiforme?
Surgical excision when possible, radiotherapy and chemotherapy
Meningiomas are benign, slow growing tumors that most commonly arise from
The dura
Although meningiomas are often asymptomatic, what are some symptoms that can occur due to the compression and displacement of the brain?
seizures or focal neurological signs
Fixed dilated pupil (CN III) common
Diagnostics for meningioma
- MRI with contrast of brain: ring enhancing lesion often attached to dura
- Histology: spindle-cells in a whorled pattern. Psammoma bodies (round calcifications)
Treatment for meningiomas
- Asymptomatic: observation
- Symptomatic: surgical excision when possible
An ependymoma is a tumor of the ependymal cells in the ventricles and parts of the spinal cord. Who is it most common in and where is it most common?
Most common in children
-In the 4th ventricle, spinal cord, and medulla
What is delirium?
Acute, abrupt transient confused state due to an identifiable cause (due to medications, infections, electrolyte abnormalities, CNS injury, uremia, illicit drugs)
Symptoms of Alzheimer Dementia (MC type of dementia)
- Short-term memory loss (first symptom)
- Progression to long-term memory loss and cognitive deficits
- Behavioral and personality changes
- Loss of motor skills, gradual in nature
Although Alzheimer Dementia is a clinical diagnosis, there are two diagnostics that can be done to diagnose. What are they and what do you see?
MRI (preferred): cortex atrophy, white matter lesions
Histology: amyloid-beta protein deposition (senile plaques) in he brain. Neurofibrillary tangles of tau protein.
Treatment for Alzheimer Dementia
- Acetylcholinesterase Inhibitors: Donepezil, Tacrine, Rivastigmine, Galantamine.
- NMDA Antagonist: Memantine: may be used as monotherapy or adjunctive
Brain disease due to chronic ischemia and multiple infarctions (lacunar infarcts)
Vascular dementia
What is the most important risk factor for vascular dementia?
Hypertension
-Others: DM, history of CVA, A-fib
Symptoms of vascular dementia
-Stepwise progression of symptoms: random infarct, then decline –> stable, then another infarct, then decline, etc.
Main prevention of vascular dementia?
Strict blood pressure control!
localized brain degeneration of the frontotemporal lobes
Frontotemporal Dementia (Pick’s Disease)
Symptoms of Pick’s Disease
marked changes in social behavior, personality, and language (aphasia) with eventual exercise and memory dysfunction
-Behavioral changes: socially inappropriate behaviors, apathy, hyperorality (binge eating, putting large amounts of food in their mouth, changes in taste preferences)
On histology for Pick’s Disease, what is seen?
Pick bodies: round or oral aggregates of Tau protein seen on silver-staining of cortex