Case files Flashcards
Potential PET finding on someone with essential tremor
increased activity in thalamus
Therapies for essential tremor
propranolol
primidone -structural analog of phenobarbital
Westphal variant of HD
develops in childhood with more severe symptoms with more similarities to parkinson’s
How to symptomatically manage chorea found in HD?
high pot neuroleptics like haloperidol or tetrabenazine (depletes dopamine supply presynaptically)
Dystonia is a movement disorder characterized by sustained or intermittent muscle contractions that are worsened by voluntary action and assoc with overflow muscle activation. What are the primary causes?
over 15 different types
early onset
most common is GAG deletion in exon 5 of DYT-1 of chromo 9
large phenotypic variability
What symptoms might point you to a secondary cause of dystonia
- dystonia at rest
- atypical site like leg in adult or head in child
- early onset of speech difficulty
- other neuro abnormalities
- abnormal imaging
- abnormal labs
In PD, the severity of bradykinesia is related to?
decreasing levels of DA
Multiple system atrophy is a type of Atypical parkinsonism. How does it differ?
tremor is less prominent
orthostatic hypotension
impotence
abnormalities on MRI
characterize symp found in progressive supranuclear palsy
- supranuclear downgaze palsy (inability to look downward voluntarily
- pseudobulbar emotionality (involuntary crying or uncontrollable episodes of emotions)
How to treat the tremors assoc with PD?
anticholinergics like trihexyphendyl or diphenhydramine
What does amantadine do in PD treatment?
Amantadine is a NMDA receptor blocker that helps with resting tremor and dystonia. It also alleviates levodopa-induced dyskinesis
Surgical option for PD
DBS to inhibit subthalamic nucleus
If someone with PD fails to respond to levodopa/carbidopa, suspect?
2/2 causes; not idiopathic
How come in someone who is young with mild PD that starting with the most efficacious treatment levodopa/carbidopa is not a great idea?
levodopa/carbidopa in the long-term can cause dyskinesis like chorea form movements and dystonic movements. Therefore, in mild cases, start with dopamine agonists (pramipexole, ropinirole, bromocriptine), MAO B inhibitors like selegiline.
Over 25+ types of spinocerebellar ataxia. The most common is type III (Machado-joseph disease). Describe symptoms.
- pure cerebellar ataxia, familial parkinsonism, spastic paraplegia, restless leg syndrome
- most specific sign: impaired TEMP discrimination in all limbs and even trunk and face
- no cure or treatment
- will see degeneration of cerebellum and thoracic cord
- genetic testing is diagnostic
Treatment for tardive dyskinesia and causes of TD
causes: antipsychotics, metoclopramide
treatment: d/c meds that are causing TD, can try benzos, baclofen and Vit E
If someone fails to respond to painful stim of all 4 extremities and is quadriplegic, where’s the lesion of cord injury
upper cervicalboth ascending sensory and corticospinal tracts are affected
afferent and efferent limbs of corneal reflex
afferent: trigem V1
efferent: VII -to blink
afferent and efferent limbs of pupillary light
afferent: II
efferent: III
Brown sequard can be caused by injury to spinal cord. What are symptoms
hemisection of spinal cord
- lesion is two levels above symptoms
- ipsilateral weaknes and loss of fine touch & vibration
- contralateral loss of pain & temp
Anterior cord syndrome can be caused by traumatic or vascular insult to anterior 2/3 of spinal cord leading to?
b/l loss of spinothalamic tract and corticospinal tract
CEntral cord syndrome is most commonly due to chronic process (e.g. syringomyelia). leading to
capelike distribution of loss of pain and temp
What is spinal cord injury without radiographic abnormality most common in?
SCIWORA is found in children
How come head trauma can cause hyponatremia?
SIADH leading to hyponatremia leading to AMS and delirium
How to classify grade 1, 2, 3 concussions
grade 1-no LOC -all symptoms resolve within 15 min
grade 2-no LOC -symp last > 15 min
grade 3 -LOC for any period of time
What is Todd’s paralysis?
brief period of transient paralysis following a seizure
how to prevent cerebral vasopasm that can occur after subarachnoid hemorrage?
triple H therapy -induce hypertensive hypervolumic hemodiluation
nimodipine
ADEM is a T cell mediated targeted myelin after viral disease. What’s the pathology?
Perivenular inflamm myelinopathy with engorgement of veins in white matter of brain
List the 4 options you can use to abort migraines
1) sumatriptan (5-HT1D agonist) -contraindicated in someone with CAD/HTN or with aura involving vision loss
2) Ergotamines -only if triptans don’t work
3) Dihydroergotamine -given with anti-nausea meds
4) Midrin -combo of acetaminophen, dichloraphenazone (muscle relaxant)
3 options for ppx for migraines
1) AED like valproic acid (side effects: tremor, alopecia), topiramate (side effects: sleepiness, increase IOP –> blindness)
2) B blockers -propranolol (side effects: depression)
3) CCB
4) antidepressants -duloxetine, amitryptiline, nortryptiline
Interestingly, which dementia is assoc with impaired olfaction?
Alzheimers
what is typical SPECT finding of someone with Lewy Body Dementia (visual hallucinations + parkinsonism features)
occipital lobe hypometabolism
MR spectroscopy results of someone with MS
lower N-acetyl aspartate which is a marker for neuronal and axonal structure
How to confirm if someone with Adie holmes pupil (dilated) with light-near dissociation (cannot constrict with light but does when near) has it?
apply 0.1% pilocarpine to the pupil to see if it constricts. If it does, then it confirms Adie-Holmes. b/c if you put pilocarpine in a normal eye, nothing should really happen.
All pts with increased intracranial pressure with papilledema (blurring of optic disc) require? DDX?
- CT without contrast emergently
- DDX: meningitis, hydrocephalus, space occupaying lesions, dural sinus thrombosis and pseudotumor cerebri
Placement for LP is at which spinal level
L4-L5
Which imaging test should be done to assess diplopia?
MRI
What is a cholesteatoma
a benign tumor of epithelial debris that is produced when the squamous layer of the eardrum is trapped and cannot exfoliate properly, usu occuring in pts with preexisting ear problems. If untreated can lead to brain abscesses.
VZV reactivation that leads to Ramsay Hunt Syndrome. where does this virus reside before reactivation?
virus in sensory ganglia (geniculate ganglion).
The most common cause of acute facial weakness in an adult is?
Bell palsy -linked to herpes simplex virus (can treat with antiviral + steroids)
Someone with myasthenia gravis can have antibodies against the achR or to muSK. They present with ptosis, fatigue as they continue to use their muscles. What is the ice test that can be used bedside to evaluate these pts with ptosis?
Place ice over ptotic eyelid for 2 minutes. If ptosis improves after removing the ice, a dx of underlying NMJ transmission d/o can be made b/c cooling improves NMJ tx whereas heat worsens it. A reason why pts with MG have worsening symptoms during the summer months.
Rotary and horizontal nystagmus (fast component is beating away from affected side) are usu due to problems in
inner ear
Vertebrobasilar insufficiency can cause vertigo that lasts for seconds to minutes with repeated episodes or evolving symp. What are some associated smyptoms? Treatment?
symptoms can include changes in speech or swallowing, cerebellar symptoms, h/o consistent with atherosclerosis; neuro exam may be normal but could also find CN findings, cerebellar signs or carotid bruit.
Treat with antiplatelet therapy and management of risk factors
Viral vestibular neuronitis can last for 24+ hours and may take few weeks to resolve. some N/V. horizontal nystagmus early on in course. How to treat?
supportive care
anti-emetics
Acute suppurative labyrinthitis with vertigo attacks lasting 2-3 days taking over a few weeks to resolve. More symptoms than viral. Will be severely ill with N/V, hearing loss, tinnitus, otorrhea. Can present with concomitant otitis media or cholesteatoma. If with underlying cholesteatoma, worry about possible facial paralysis. How to treat
IV abx
possibly surgery
Acute serous labyrhinthitis like acute suppurative can have vertigo attacks lasting 2-3 days, also taking few weeks to resolve. Can be assoc with hearing loss, tinnitus, N/V. Usu with h/o of past otologic surgery. How to treat?
supportive care
use steroids if hearing loss is possible
What is chronic inflammatory demyelinating polyneuropathy (CIDP)/? these lesions pathologically exhibit?
acquired peripheral neuropathy w/ extremely variable clinical presentation and course. Pts usu present w/ generalized pattern of numbness & weakness in UE and LE. Spontaneous pain develops gradually.
Lesions have patchy regions of demyelination and edema with infiltrates of macrophages and T cells
How to diagnose Chronic inflammatory polyneuropathy?
EMG/NCV -will see blocked conduction velocities
CSF-will see cytoalbumoinologic dissoc with GBS
Nerve biospy
In GBS, one should always do what b/c this complication can lead to death?
check FVC to see if pt needs to be intubatedbefore starting treating with IVIG or plasmapheresis. Also need ICU care bc of dysautonomia can lead to arryhtmias and low BP
Describe characteristics of the Miller-Fisher variant of GBS
- triad of areflexia, ataxia, ophtalmoplegia
- positive anti-GQ1b (ganglioside) abs
Tick paralysis can present like GBS but is more rapid. Removal of tick can cure it. What are symptoms
rapidly ascending paralysis
areflexia
ataxia
respiratory insuffiency
Polymyositis and dermatomyositis present with proximal muscle weakness, with increased CK and ESR.. What are other symptoms that can be assoc with these myopathies.
- intersititial pneumonitis (poly > derma)
- myocardial involvement: CHF, EKG abnormalities, left anterior fascicular block, RBBB
- increased risk of malignancy (derma > poly) reflective of age and gender + non-hodgkins
Inclusion body polymyositis unlike derma and polymyositis has no antibodies that can be detected. It is more indolent course with involvement of proximal and sometimes distal muscles. What are the inclusion bodies?
muscle cells have abnormal inclusions like eosinophilic cytoplasmic inclusions, vacuoles rimmed with basophilic granules, foci that stain + for Congo red (so amyloid deposits).
on EM, you will see cytoplasmic helical filaments aka tonofilaments with beta-amyloid protein.
what is the genetic cause of ALS
- mutation in Cu/Zn superoxide dismutase gene (SOD1) on chromosome 21
- gain of function mutation
Peroneal neuropathy (L4-L5) is caused by compression where? It is the most common compressive neuropathy in the lower extremity.
fibular head
How does ankle foot orthosis help with treating foot drop
It provides toe dorsiflexion during swing phase, medial and lateral stability at ankel during stance
what’s the best modality to assess damage and recovery of peripheral nerve?
EMG
EMG vs NCS
EMG measures electrical activity of muscles at rest and during contraction
NCS measure how well and fast the nerves can send electrical signals
when do juvenile myoclonic seizures usu occur
after awakening
most common cause of seizures in neonates to 3 years old is injury. From 3-20, probably genetic. List the 4 most common hereditary epilepsies
1) febrile convulsions -usu resolves on own by 5, usu assoc illness with high fevers
2) benign rolandic epilepsy
3) childhood absence epilepsy
4) juvenile myoclonic epilepsy -occurs shortly after awakening
Criteria to list febrile seizures as simple
1- pt age btw 3 mo and 5 yrs 2- generalized without focal elements 3- seizure lasting less than 15 min 4- assoc with a fever that is not caused by CNS infection 5- occurs only once in a 24 hr period
Which virus seems to be disproportionately assoc with febrile seizures?
herpesvirus 6
Febrile seizures may not warrant daily phenobarbital or valproic acid b/c of side effects. What’s as effective and better tolerated to prevent these seizures?
oral or rectal diazepam
HOw to differentiate btw simple from complex febrile seizures?
- simple: less than 15 min, no more than 1 in 24 hours, generalized
- complex: than 15 min, focal
T/F: Having migraine headaches doubles the chance that a pt will have epilepsy, and having epilepsy doubles a patient’s chance of having migraines
True
Best abortive therapy in pediatric migraines
ibuprofen (NSAIDS!)
first line treatment for tourette
-fluphenazine
-pimozide
both neuroleptics
Benign rolandic epilepy is a common childhood epileptic disorder. Describe it.
onset btw 3-13 y.o that stops before 20
- sensorimotor manifestations on one side of the face and mouth
- u/l oral paresthesias as well as facial clonic and/or tonic activity
- speech arrest
- hypersalivation
- most occur at night
EEG results characteristic of benign rolandic epilepsy that occursi n children that often start out in the face and at night…
centrotemporal sharp waves
Benign epilepsy of childhood with occipital paroxysms. Describe them and what EEG can show
visual symptoms start first and the psychomotor, sensoirmotor or migraine like phenomena follow
EEG reveals occipital spikes that go away with eye opening
What is Miller-Dieker syndrome aka Lissencephaly (smooth brain) type 1? (deletion on chromosome 17 –> prob with neruonal migration)
severe retardation recurrent seizures infantile spasms severe developmental delays microcephaly dysmorphic facial features generalized hypotonia past history will reveal gestation complicated by polyhydramnios, IUGR, decreased fetal movements
unilateral sensorineural hearing loss should further be evaluated by?
MRI with gadolinium contrast
