Term
|
Definition
| M: Deltoid N: Axillary R: C5 |
|
|
Term
|
Definition
| M: Biceps N: Muskulocutaneous R: C5/6 |
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Term
|
Definition
| M: Triceps N: Radial R: C7-8 |
|
|
Term
|
Definition
| M: Extensor Carpi Radialis N: Radial R: C5-6 |
|
|
Term
|
Definition
| M: Finger flexors N: Median/Ulnar R: C8/T1 |
|
|
Term
|
Definition
| M: Finger extensors N: Radial R: C7 |
|
|
Term
|
Definition
| M: Interosseous N: Ulnar R: C8/T1 |
|
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Term
|
Definition
| M: Illiopsoas N: Femoral R: L1-3 |
|
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Term
|
Definition
| M: Gluteus medias and minims N: Superior gluteal R: L5 |
|
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Term
|
Definition
| M: Hip adductors N: Obturator R: L3 |
|
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Term
|
Definition
| M: Gluteus maximus N: Sciatic R: S1 |
|
|
Term
|
Definition
| M: Hamstrings N: Sciatic R: L5-S2 |
|
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Term
|
Definition
| M: Quadriceps N: Femoral R: L2-4 |
|
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Term
|
Definition
| M: Tibialis anterior N: Deep perineal R: L4/5 |
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Term
|
Definition
| M: Gastrocnemius and Soleus N: Tibial R: S1/S2 |
|
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Term
|
Definition
| M: Peroneus (longus and brevis) N: Superficial Peroneal R: L5/S1 |
|
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Term
|
Definition
| M: Tibialis posterior N: Tibial R: L4/L5 |
|
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Term
|
Definition
| M: Entensor Hallicus Longus N: Deep Peroneal R: L5/S1 |
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Term
|
Definition
| M: Triceps N: Radial R: C7 |
|
|
Term
|
Definition
| M: Biceps, elbow flexion N: Musculocutaneous R: C5 |
|
|
Term
|
Definition
| M: Brachioradialis N: Radial R: C5/6 |
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Term
|
Definition
|
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Term
|
Definition
|
|
Term
| What reflexes can be checked in a comatose patient and what are the afferent/efferent limbs? |
|
Definition
- Pupillary (II/III)
- Corneal (V1 afferent, VII efferent)
- Oculocephalic (VIII afferent; III, IV, VI)
- Gag |
|
|
Term
| What are the 3 things you should do in a patient with increased ICP? |
|
Definition
1) Raise head: venous drainage
2) Hyperventilation, blow off CO2 and encourage vasoconstriction
3) Mannitol: osmotic diuresis |
|
|
Term
| Why might you have bilateral ptosis and weakness of contralateral SR? |
|
Definition
| Lesion at level of CN III nucleus |
|
|
Term
| What CN palsy presents with trouble reading and walking down stairs. These patients compensate with head tilt. |
|
Definition
| Trochlear CN IV palsy: Superior oblique (down and in is normal action) |
|
|
Term
| Why might a patient present with Esotropia? What might you be worried about? |
|
Definition
CN VI palsy: LR muscle
Non-localizing sign of increased ICP |
|
|
Term
Describe the neural pathway of the MLF control of eye movement.
What lesion is responsible for lack of adduction of R eye with abduction of left eye? |
|
Definition
VI nucleus projects to contralateral III nucleus via ipsilateral MLF.
Lack of abduction of L eye indicates problem with L VI nucleus and L MLF. So left-sided INO |
|
|
Term
| What are the important signs of increased ICP? |
|
Definition
Due to space-occupying lesion or IHH
Morning headache. No RAPD
Get MRI/CT, LP (opening pressure) |
|
|
Term
| What is the treatment for IHH? |
|
Definition
Give acetazolamide (inhibits choroid CA), nerve decompression, shunt
Weight loss long term |
|
|
Term
| Why are you worried if a patient develops pain with eye movement in one eye that is worse with exercise and heat? |
|
Definition
Sounds like ON demyelination
Treatment is IV steroids. Get MRI
If MRI shows > 3 lesions, MS within 5 years 50% |
|
|
Term
| What are the signs of Foster Kennedy syndrome? |
|
Definition
Foster Kennedy (frontal lobe tumor):
ipsilateral optic atrophy and contralateral papilledema |
|
|
Term
| What are the distinguishing features between Peripheral and Central nystagmus? |
|
Definition
1) Peripheral (Vestibular): vascular, trauma, toxicity, meniere, vestibular neuronitis
- Unidirectional, fast phase away from lesion
- Visual fixation inhibits nystagmus and vertigo
- Tinnitus and hearing loss common
- Severe vertigo present
- Short duration
2) Central: vascular, demyelination, neoplastic
- Bidirectional or unidirectional
- No change with visual fixation
- Rarely see tinnitus or hearing loss
- Vertigo often mild and duration chronic. |
|
|
Term
| What is your differential for Vertigo? |
|
Definition
1) Vestibular Neuronitis
- Acute unilateral peripheral vestibulopathy
- Sudden onset vertigo, nausea and vomiting
- Lasts 24h and improves gradually over several days/weeks
2) Labryinthine concussion (trauma)
3) Lateral medullary or brainstem infarction
4) Menierre’s Disease: SNHL, vertigo and tinnitus (increase in endolymphatic volume)
5) Perilymphatic fistula:
- sudden “pop” With increase ME pressure and abrupt vertigo
6) Positional
- Peripheral: BPPV: brief episodes lasting seconds to minutes, usually when awakening or reclining in bed. Look for vertical and torsional nystagmus on Dix-Hallpike. Treat with Epley (direction of offending ear) |
|
|
Term
| How does a cerebellar hemorrhage present? |
|
Definition
- Acute onset vertigo, vomiting and inability to walk. Neck stiffness and occipital headache is also characteristic.
- Usually SCA or PICA
- vermal is truncal
- hemispheric is ipsilateral limb |
|
|
Term
| How does alcoholic cerebellar degeneration differ from cerebellar hemorrhage? |
|
Definition
| progressive and slower than infarct. Primarilly vermal. |
|
|
Term
| What malignancies are associated with paraneoplastic cerebellar degeneration? |
|
Definition
Gynecologic or small cell lung cancer.
- Truncal, gait and limb ataxia
- Disturbance in ocular motility and dysarthria |
|
|
Term
7 year old boy presents with gradually progressive ataxia arms > legs with dysarthria.
He also has loss of reflexes, with spasticity and extensor plantar responses and impaired vibration/position sense.
What is the diagnosis and what are the concerning associated issues? |
|
Definition
Freidrich's Ataxia: AR TNR disorder
Associated with necrosis and degeneration of cardiac muscle fibers (myocarditis) |
|
|
Term
| Where in the brain is micturition initiated? |
|
Definition
|
|
Term
| What are the 3 types of urinary incontinence and how are they treated? |
|
Definition
1) Urge incontinence: detrusor hyperreflexia (UMN issue)
- Common in stroke, frontal lobe dysfunction, spinal cord lesions, MS
- Anticholinergics: Oxybutynin, Tolterodine, Tropsium (increase capacity and decrease strength of contractions)
- Can also use TCAs, Desmopressin, Capsaicin
2) Stress incontinence:
- Multiparous women with weakened muscles of pelvic floor and/or urethral hypermobility.
- Floor exercises with Alpha agonists (Pseudoephedrine, Midodrone), Estrogen therapy or Duloxetine.
3) Overflow incontinence: Atonic bladder (LMN issue)
- Drugs, DM, lower spinal cord injury
- Cholinergic drugs like Bethanechol.
- Valsalva/Crede’s maneuver and self-catheterization is long-term solution. |
|
|
Term
| What are the abortive and prophylactic agents for the 3 major types of HA? |
|
Definition
1) Migraine (primary)
- Triptans are first line abortive (watch out in CAD)
- PPX involves beta blockers, TCAs and anticonvulsants.
2) Tension (primary)
- NSAIDs and stress reduction
3) Cluster (primary)
- Triptans and Oxygen as abortive agents, steroids may shorten duration.
- Verapamil PPX and alcohol avoidance. |
|
|
Term
| What type of HA is worse in upright position and improved with recumbency? What do you do about it? |
|
Definition
Low-pressure headache (secondary)
- Usually complication of LP
- Treatment with recumbency and fluid replacement, with caffeine |
|
|
Term
| What condition can mimic IHH? |
|
Definition
|
|
Term
| What is the major concern with anyone > 50 with new onset headache? |
|
Definition
Temporal Arteritis
- Get ESR/CRP. If borderline, consider biopsy
- Steroids for 6 months |
|
|
Term
| What is the etiology of trigeminal neuralgia and how is it worked up and treated? |
|
Definition
1) Compression of nerve at CP angle (aberrant vascular loop)
2) MRI to exclude other lesions
3) CBZ is treatment of choice. |
|
|
Term
| How is post-herpetic neuralgia managed? |
|
Definition
- Early treatment of shingles with acyclovir will reduce risk of PHN.
- Management is symptomatic with TCA and gabapentin being most efficacious. |
|
|
Term
| What are the key features of a NMJ disorder? |
|
Definition
a. Fluctuating character is key. Change with use or rest or time of day
b. Usually proximal
c. No sensory features
d. AChR antibodies (MG) or other blood markers |
|
|
Term
| Which arterial distribution infarct would produce a Broca's aphasia. What about wernickes? |
|
Definition
1) Broca: superior division of left MCA (inferior frontal lobe)
2) Wernicke: Inferior division of left MCA (usually embolic stroke)
- Posterior part of superior temporal gyrus
Global aphasia would be entire MCA or ICA |
|
|
Term
| How do transcortical aphasias differ from Broca and/or Wernicke? |
|
Definition
1) Transcortical motor aphasia (Broca equivalent): production disturbed only, with in tact repetition
- Lesion slightly superior to Broca’s area in anterior superior frontal lobe
2) Transcortical sensory aphasia (Wernicke equivalent): only comprehensive affected, in tact repetition
- Inferior portion of left temporal lobe
- PCA infarcts and small left temporal lobe hemorrhages |
|
|
Term
| What type of lesions would produce Apraxia? |
|
Definition
Cannot carry out learned motor tasks (hammer a nail) despite having all functionality necessary
Frontal: can recognize but not perform
Parietal: cannot recognize
lesion usually in dominant hemisphere |
|
|
Term
| What is Gerstmann's Syndrome? |
|
Definition
- Agraphia, acalculia, right-left confusion, finger agnosia, inability to recognize name
- Dominant inferior parietal lobule lesion (angular gyrus): symbolic respresentation. |
|
|
Term
| What lesion produces unilateral neglect? |
|
Definition
- Damage to non-dominant (right) hemisphere
- Test for with extinction to double simultaneous stimulation. Draw clock.
- Lesions to right frontal (motor) or parietal (sensory neglect) lobe (acute finding after stroke) |
|
|
Term
| Describe the defining characteristics of the 4 stages of sleep |
|
Definition
1) Stage 1 (N1): attenuation of posterior dominant background rhythm
2) N2: Sleep spindles and K complexes
3) N3: Deep/slow-wave >20% delta frequency
4) REM: atonia of skeletal muscles other than EO muscles |
|
|
Term
| How is restless leg syndrome treated? What are the SE |
|
Definition
Treatment: Dopamine Agonists: Pramipexole, Ropinirole:
SE: nausea, lightheadedness, sleep attacks |
|
|
Term
| How is Narcolepsy diagnosed and treated? |
|
Definition
1) Diagnose with multiple sleep latency test (PSG type). REM at sleep onset
2) Modafinil first line. Sodium oxybate and antidepressents for cataplexy. |
|
|
Term
| How is REM behavior disorder treated? |
|
Definition
Benzo at bedtime.
Remember risk of LBD |
|
|
Term
| What are the major intracranial branches of the anterior circulation? |
|
Definition
ICA gives of ophthalmic artery, penetrates dura, gives of
1) anterior choroidal
2) PCOM
3) Bifurcates into ACA and MCA |
|
|
Term
| What are the major intracranial branches of the posterior circulation? |
|
Definition
Supplies brainstem, thalami, cerebellum, posterior portions of cerebral hemispheres (Occipital and medial temporal)
Vertebral arteries give off penetrating arteries of medulla and PICA before joining to form
1) Basilar at pontomedullary junction
2) Basilar gives off AICA and SCAs before dividing into PCAs.
- ACOM connects anterior circulations on each side (ACA to ACA).
- PCOM connects anterior and posterior circulations on each side (PCA to MCA) |
|
|
Term
Where is the stroke?
- Contralateral: Legs > arms, primitive reflexes
- Urinary incontinence
- Gait disturbance
- Mutism |
|
Definition
|
|
Term
Where is the stroke? How is it managed?
Ipsilateral
1. Facial pain or Reduced pain/temp of face
2. Horner’s
3. Incoordination of arm
Contralateral
1. Pain/temp loss limbs/body
iv. Nystagmus and Gait ataxia
v. Dysphagia and hoarseness |
|
Definition
Lateral medullary stroke (Wallenberg)- Intracranial VA occlusion/dissection
Get MRA/Angio and treat with anti-coagulation. |
|
|
Term
Where is the stroke?
Ipsilateral
1. VII nerve palsy
Contralateral
1. Hemiplegia (sparing face)
2. Hemianesthesia (sparing face) |
|
Definition
|
|
Term
Where is the stroke?
Ipsilateral
1. Gaze and cerebellar signs
Contralateral
1. Hemiparesis and Hemianesthsia |
|
Definition
| Medial Pontine (paramedian pontine branch of basilar) |
|
|
Term
Where is the stroke?
Quadriparesis
Conjugate gaze paresis or VI palsy
IF medial tegmentum involved bilaterally, coma |
|
Definition
| Bilateral pontine base (basilar artery occlusion) |
|
|
Term
Where is the stroke?
i. Gait ataxia
ii. Dysarthria
iii. Ipsialteral arm dysmetria |
|
Definition
| Cerebellar Infarction (PICA, SCA) |
|
|
Term
Where is the stroke?
i. Right homonymous hemianopia
ii. Amnesia
iii. Alexia without agraphia if splenium of CC involved. |
|
Definition
|
|
Term
Where is the stroke?
i. Left homonymous hemianopia
ii. Left-sided visual neglect possible |
|
Definition
|
|
Term
Who to each of the following AEDs work? What are the SE?
1) Phenytoin:
2) Carbamazepine:
3) Valproic acid:
4) Phenobarbital:
5) Ethosuximide:
6) Lamotrigine:
7) Topiramate:
8) Levetiracetam: |
|
Definition
1) Na+ channel, treats partial seizures, SE (gingival hyperplasia, ataxia, coarsening facial features).
2) Na+ channel, partial seizures, SE: hyponatremia, agranulocytosis, diplopia
3) Na+ and GABA, partial or generalized, SE: GI, tremor, weight gain, hair loss, LFTs, thrombocytopenia
4) GABA receptor: partial, generalized, SE: sedation
5) T-type CCB, absence seizures, GI symptoms
6) Na+, partial, generalized, SJS
7) Na+, partial, generalized, SE: cognitive slowing, stones, weight loss
8) Unknown, partial, generalized, SE: insomnia, anxiety, irritability |
|
|
Term
| Describe your series of management steps for Status Epilepticus |
|
Definition
1) ABCs, fingerstick, IV access
2) Send blood studies, give thiamine/dextrose
3) Lorazepam 0.1 mg/kg IV
- If persists: Phenytoin 20 mg/kg (or fosphenytoin)
- If persists: Intubate and Phenobarbital 20 mg/kg IV
- If persists: induced coma and continuous EEG |
|
|
Term
| How does diffuse axonal injury appear on imaging? |
|
Definition
| Multiple areas of punctate hemorrhage in deep white matter and CC on CT. |
|
|
Term
Patient presents with decreased alertness, small, reactive pupils, and emerging decerebrate posturing.
What are you worried about? |
|
Definition
| Central (Transtentorial) Herniation |
|
|
Term
| Describe the classic findings of uncial herniation |
|
Definition
| Ispilateral CN III (dilated pupil) palsy followed by compression of contralateral cerebral peduncle against free edge of tentorium resulting in ipsilateral hemiplegia. |
|
|
Term
| Women has suffered from recurrent fetal loss and you suspect APA syndrome. How do you confirm and treat? |
|
Definition
1) Diagnosis required IgG APA on two occasions 6 weeks apart.
2) Treatment is long-term anticoagulation with INR to 3-4. |
|
|
Term
| What are the general medical treatments for CNS tumors? |
|
Definition
| glucocorticoids (reduce vasogenic edema) and anticonvulsants (seizures) |
|
|
Term
| What genetic syndromes are associated with CNS tumors? |
|
Definition
1) NF-1: AR chromosome 17 associated with glioma (optic nerve) and ependymoma
2) NF-2: AR chromosome 22 associated with meningioma and glioma (bilaterally vestibular schwanommas)
3) vHL: 3p25 associated with Hemangioblastoma.
4) Li-Fraumeni (p53): 17p13.1 associated with glioma and medulloblastoma |
|
|
Term
|
Definition
surgery w/ adjuvant temozolomide for 6 months. Often added nitrosoureas
Different from low-grade glioma, which is treated with observation |
|
|
Term
| What are the key features of MS? |
|
Definition
1) Autoimmune affects young, white woman with genetic link
2) Involves multiple white matter lesions separated in space and time.
3) Symptoms tend to be worse in heat (Uhthoff’s phenomenon) and neck flexion may produce tingling, electric sensation (Lhermite’s sign). |
|
|
Term
| In a patient with suspected MS, what is your diagnostic workup? |
|
Definition
1) MRI: new lesions are discrete T2-hyperintense areas in white matter of brain or SC. Old lesions are hypo-dense on T1.
2) CSF: Pleocytosis and elevated protein. Oligoclonal bands |
|
|
Term
| What are the acute and chronic management options for a patient with MS? |
|
Definition
1) Acute for relapse: Steroids IV with taper.
2)Chronic
- Beta IFN: reduce rate of relapses, burden of lesions and rate of accumulated disability
- Glatiramer acetate injection
- Natalizumab (alpha-1 integrin antibody) infusions. Can cause PML!
-Fingolimod (agnostic/antagonist of sphingosine-1P1-receptor). Watch out for Cardiac effects. |
|
|
Term
| How can spasticity and bladder dysfunction in MS be managed? |
|
Definition
1) Spasticity: Baclofen, Diazepam
2) Bladder dysfunction: Anticholinergic and self-catheterization |
|
|
Term
| How can Acute Disseminated Encephalomyelitis be differentiated from MS? How is it managed? |
|
Definition
1) ADE is Monophasic.
2) Lesions are more patchy and confluent compared to discrete lesions in MS
3) Shorten duration with CS course. Usually will improve to baseline. |
|
|
Term
| How does Neuromyelitis Optical differ from MS? How is it managed? |
|
Definition
1) Transverse myelitis and Optic neuritis, without demyelination of brain.
2) Confirmed with anti-aquaporin-4 antibodies
3) Steroids T/x as well as plasmapheresis in certain cases. |
|
|
Term
| Why might a patient with MS develop dementia, focal cortical dysfunction and ataxia? What is the first test you order? |
|
Definition
PML can develop in patients treated with Natilizumab (alpha integrin antibody). JC virus lads to demyelination of oligodendrocytes
Dementia, focal cortical dysfunction and cerebellar abnormalities.
Also seen in patients with AIDs, leukemia and lymphoma
2) MRI shows multiple foci of white matter abnormalities in posterior regions. CSF will be normal (differentiates from MS) |
|
|
Term
| What is PRES? How does it present and how is it diagnosed and managed? |
|
Definition
1) Develops in rapidly-developing HTN and eclampsia or 2/2 immunosuppression
Acute confusion state and cortical vision loss (preserved pupil reactivity)
2) MRI shows posterior white matter hyperintensitives on T2.
3) Treat by addressing underlying cause |
|
|
Term
| What are the typical CSF findings in adult bacterial meningitis? How is it treated? |
|
Definition
1) low glucose, high PMN count, high protein
2) Vanco, Ceftriaxone, Acyclovir often +/- steroids |
|
|
Term
| What are you most concerned about in a patient with neck and back pain, as well as LE weakness and sensory loss below a clear level? What do you order and how can you treat? |
|
Definition
1) Sounds like spine involvement, perhaps spinal epidural abscess
2) Get urgent MRI and look for ring-enhancing lesion. DO NOT get LP
3) IV antibiotics mainstay and sometimes drainage. If compression, surgical decompression indicated. |
|
|
Term
| Describe the mode of transmission, diagnostic strategy and treatment for Cryptococcal meningitis |
|
Definition
1) Airborne, then hematogenous
2) CSF shows lymphocytic, elevated protein and low glucose (like bacterial, except lymphocytic vs. PMN)
- Rapid latex agglutination assay for antigen is standard
3) Amphotericin treatment |
|
|
Term
| How is Neurocistercosis treated? |
|
Definition
| Albendazole and steroids +/- AED. |
|
|
Term
| How does a spinal chord hemisection present? |
|
Definition
1) Ispilateral
- Weakness below lesion
- Impaired position sense
2) Contralateral
- Loss of pain/temperature |
|
|
Term
| What medical condition is associated with Syringomyelia? |
|
Definition
| Arnold Chiari malformation |
|
|
Term
| What physical findings would you expect in a patient with an ASA infarction of the spinal chord? |
|
Definition
- ASA (caudally fed by Artery of Adamkieqicz off of aorta)
- Perfused anterior horns and CS tracts
1) Weakness below lesion with pain and sphincter dysfunction.
2) Sensory loss sparing dorsal columns
3) Joint position sense preserved at toes |
|
|
Term
| What are the first line treatments for spinal chord damage secondary to 1) Traumatic compression and 2) Tumor Compression |
|
Definition
1) High dose methylprednisolone IV for traumatic cord compression
2) High dose dexamethasone IV for compression due to tumors |
|
|
Term
| What is the only medication that has been shown to help ALS? How does it work and what does it do? |
|
Definition
| Riluzole (blocks glutamate) retards speed of deterioration in ALS |
|
|
Term
| What functions are carried by small vs. large fibers in PNS? |
|
Definition
1) Small fiber (pain, temperature, autonomic)
2) Large fiber (motor, vibration, proprioception, fasciculation, DTRs) |
|
|
Term
| What are the defining diagnostic study findings in GBS? |
|
Definition
1) Normal nerve conduction studies
2) anti-Gq1b ABs (90%)
3) CSF: albuminocytologic dissociation (elevated protein with few cells)
DDX includes transverse myelitis, acute M. gravis, myopathy |
|
|
Term
| Who is an acute GBS attack managed? |
|
Definition
1) Hospitalization with monitor FVC (<15 means ICU)
2) IVIG or plasmapheresis are equal but NOT steroids. |
|
|
Term
| Who can you distinguish GBS from Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)? |
|
Definition
Other than being more progressive, CIDP has abnormal EMG in addition to high protein on CSF.
Treatment is IVIG and Steroids (unlike GBS) for years. |
|
|
Term
| What are the defining diagnostic findings in multifocal motor neuropathy? How is it treated? |
|
Definition
1) Pure motor multiple mononeuropathy, slowly progressive with distal limb weakness usually beginning in arms
2) Normal CSF protein. Very high IGM anti-GM1 is characteristic.
3) Responds to IVIG, Rituximab and Cyclophosphamide. |
|
|
Term
| How does uremic peripheral neuropathy present? |
|
Definition
| Symmetrical distal (foot drop and leg weakness) |
|
|
Term
| What do you think about in an adolescent patient with distal muscular atrophy in legs and feet, hammer toes and pes cavus. |
|
Definition
| Charcot-Marie-Tooth disease (CMT): No treatment available |
|
|
Term
| What are the classic findings in Leprosy-related neuropathy? |
|
Definition
1) Mononeuropathy multicomplex in nose, ears or distal limbs (cooler areas)
2) Preserved DTRs with severe nerve hypertrophy |
|
|
Term
| Which 3 antibodies are associated with M. gravis? |
|
Definition
| nAChRs, Anti-MuSK and Lrp4 |
|
|
Term
| What organ systems are most commonly involved in M. gravis? |
|
Definition
1) Ocular involvement is common (ptosis and dipolopia) without pupils
2) Bulbar muscles commonly involved (dysarthria or dysphagia)
3) Weakness is symmetric and proximal. |
|
|
Term
| What are the characteristic findings on nerve monitoring for M. gravis? How does this differ from LES? |
|
Definition
1) Slow repetitive nerve stimulation (RNS) reveals decremental response.
2) Most sensitive test is single-fiber EMG (increased jitter).
3) In LES, incremental response is seen on fast repetitive nerve stimulation (RNS) |
|
|
Term
| How do you treat M. gravis acutely and chronically? |
|
Definition
1) AChE inhibitors for symptoms (not underlying)
2) IVIG, plasmapheresis work acutely.
3) Steroids and steroid-sparing agents (cyclosporine, azathioprine) mainstay |
|
|
Term
| How is LES diagnosed and how is it managed? |
|
Definition
Common in SCLC. Will see motor facilitation
1) Diagnosis Antibodies and incremental response on fast repetitive nerve stimulation (RNS)
2) Treatment: Treat malignancy, 3,4-diaminopyridine is most effective +/- pyridostigmine. |
|
|
Term
| What are the classic clinical features, diagnostic evaluation and treatment of Duchenne and Becker muscular dystrophy? |
|
Definition
1) Features
- Both onset in early childhood with XLR inheritance
- Gower’s sign (push down on this while trying to rise).
- DMD leads to wheelchair by age 11-12 and death by 20. BMD is less severe
2) Diagnosis:
- CK level and muscle biopsy with absent or reduced dystrophin staining.
3) Treatment: Steroids slow decline |
|
|
Term
| Which muscular dystrophy is most common in girls, how is it diagnosed? |
|
Definition
1) Think of Limb-Girdle Muscular Dystrophy, which is AR inheritance
2) Diagnosis: CK elevated, EMG is myopathic and biopsy demonstrates nonspecific dystrophic changes |
|
|
Term
| What is the most common inherited muscular dystrophy in adults? How is it diagnosed and treated? |
|
Definition
1) Myotonic muscular dystrophy
- Look for weakness and stiffness of distal muscles
- Eye changes, dysphagia, testicular atrophy, frontal balding all common
2) AD inheritance of CTG mutation in DMPK gene
3) Diagnosis: CK normal, EMG shows myotonia, DNA test.
4) Cardiac eval important to screen arrhythmia. Management otherwise supportive |
|
|
Term
| What do you worry about in a patient with periodic spastic muscle paralysis and a + family history? |
|
Definition
Channelopathies
- Periodic paralysis and AD inheritance associated with change in serum K+ |
|
|
Term
| What are the 3 major types of inflammatory myopathy and how are they differentiated? |
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Definition
1) Polymyositis: symmetric, proximal weakness. Associated with other autoimmune conditions
- Mediated by CD-8+ T cells
- On biopsy, endomesial inflammation with invasion of non-necrotic fibers with CD8+ T cells
- Treat with steroids
2) Dermatomyositis: symmetric, proximal weakness. Heliotrope rash and gottron’s papules. Anti-Jo antibodies
- Antibodies against intramuscular blood vessels
- Treat with steroids
3) IBM: asymmetric proximal and distal weakness
- Endomesial inflammation and basophilic-rimmed vacuoles on biopsy. |
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Term
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Definition
| Stop drug, give fluids, and give IV benzos, bromocriptine, amantadine or dantrolene. |
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Term
| Why would you get an MRI in a child with suspected CP? |
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Definition
| exclude structural causes (stroke, AVM, tumor) |
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Term
| What are the 2 forms of inherited MR that are associated with maternal and paternal gene silencing and how are they differentiated? |
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Definition
1) Prader-Willi: MR, olive eyes, emotional lability, obesity. Absence segments 11 and 13 from long arm of paternally derived chromosome 15.
2) Angelman’s: MR, Abnormal gait, seizures, “happy puppet.” deletion of 11 and 13 from long arm of maternally derived chromosome 15. |
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Term
| What are you particularly concerned about in a 12 month old who develops hypotonia and a subsequent regression in mental ability? |
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Definition
| Rett Syndrome: MeCP2 gene on X chromosome. Regression in mental ability in girls with stereotyped hand movement. |
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Term
| What disease is associated with male sex, mental retardation, enlarged balls, long face, enlarged ears and enlarged head? |
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Definition
| Fragile X: CGG repeat disorder on X chromosome. |
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Term
| Which 4 developmental regressive inherited conditions are associated with cherry red spot on macula? How are they differentiated? |
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Definition
All AR inheritance 1) Tay-Sachs: Hexosaminidase A, chromosome 15, AR
- Cherry red spot in Ashkenazi jews
2) Neimann-Pick: Sphingomyelinase, chromosome 11, AR
- Cherry red spot, hepatosplenomegaly, cytoplasmic vacuolization
3) Gaucher: Glucocerebrosidase, chromosome 1, AR
- Cherry red spot. Gaucher cells in bone marrow
4) Metachromatic leukodystrophy: Arylsulfaase A, chromosome 22 AR
- Cherry red spot
- Demyelinating
- Positive urine sulfatides. |
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Term
| How do you distinguish between NF type 1 and type 2? |
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Definition
1) NF1: AD, chromosome 17, ON glioma, Café-au-lait spots, Lisch nodules in iris
2) NF2: AD, chromosome 22, Bilateral acoustic neuromas, |
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Term
| Which organ systems are involved in Tuberous Sclerosis? |
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Definition
Brain, kidney, cortex, skin (Neurocutaneous)
AD inheritance with cortical tubers, subependymal nodules, MR, Ash-leaf spots, kidney and cardiac involvement. |
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Term
| What is the worrisome associated disease with the AR condition that presents with truncal ataxia and progressive dementia? |
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Definition
Ataxia Telangiectasia
Immunosuppression and susceptibility to lymphoma is characteristic. |
|
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Term
| What are the defining features between vHL disease, ataxia telangiectasia and sturge weber syndrome? |
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Definition
All Neurocutaneous syndromes
1) vHL: AD inheritence on chromosome 3
- Cerebellar hemangioblastomas producing ataxia, Café-au-lait spots, renal lesions and pheochromocytoma
2) Ataxia telangiectasia: AR on chromosome 11
- Truncal ataxia, progressive dementia, telangiectasias, immunosuppression and susceptibility to lymphoma.
3) Sturge Weber: Sporadic
- venous angioma of pia mater, port-wine stain in V1 distribution, Seizures and MR. |
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Term
| Which antibodies are associated with peripheral neuropathy in SCLC? |
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Definition
|
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Term
| What chromosome is HD associated with? |
|
Definition
| CAD repeat on chromosome 4 |
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Term
| What kind of malignancies is Paraneoplastic cerebellar degeneration (PCD) associated with? |
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Definition
|
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Term
| Why might you see bilateral leg weakness in a sub-falcine herniation? |
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Definition
|
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Term
| Why might you use IV steroids in patients with optic neuritis? |
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Definition
| IV steroids in patients with ON can delay onset of MS. |
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Term
| How does Neurosarcoid generally present? |
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Definition
| Bilateral LMN weakness of Facial and Optic nerves |
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Term
| What is the classic Miller Fisher syndrome presentation? What antibody is associated? |
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Definition
| Ataxia, opthalmoplegia, areflexia, antiGQ1-b antibodies (also seen in GBS) |
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Term
| What neurological symptoms is classically associated with Vitamin E deficiency? |
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Definition
|
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Term
| What aneurysm classically causes compresses CN III palsy? |
|
Definition
| PCOM aneurysm causes CN III palsy |
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Term
| Which type of ADE must be applied slowly to avoid hypotension and arrhythmia |
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Definition
|
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Term
| What is the classic EEG finding in Lenox-Gastaut syndrome? |
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Definition
| 1-2 Hz generalized spike-wave discharges on EEG and associated with mental dysfunction |
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Term
| If you see a pure motor stroke in a patient with vascular disease, what does it make you think of? |
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Definition
| Lacunar stroke (microatheroma formation and lipohyalinosis |
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Term
| What are the most likely sources of metastatic brain disease? |
|
Definition
| Lung > Breast > Melanoma > Colon |
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Term
| How are the clinical presentations of ICH and SAH different? |
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Definition
| Intraparenchymal brain hemorrhages gradually worsen over min-to-hours while SAH is maximal at onset and ischemic exhibits stuttering course. |
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Term
| What condition is classically associated with momentary vision loss with head position changes + morning H/A |
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Definition
|
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Term
| True or False: Patient with positive romberg indicates issue with proprioception |
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Definition
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Term
| What is the classic presentation of Tabes Dorsalis? |
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Definition
| DRG damage and secondary degeneration of dorsal columns leads to sensory ataxia, lancinating pains, neurogenic urinary incontinence, Argyll Robertson pupils |
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Term
| What is the primary pathophysiolgical mechanism underlying NPH? |
|
Definition
| Diminished CSF absorption at AV or obstructive hydrocephalus |
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Term
| What do you think about in a patient with bilateral trigeminal neuralgia? |
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Definition
| If Trigeminal neuralgia is bilateral, think MS. |
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Term
| If you have a pure sensory stroke, where might it localize (brain and vascular)? |
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Definition
| Thalamic stroke of PLT due to occlusion of PCA |
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Term
| What are the classic physical findings in Neonatal botulism? |
|
Definition
Remember honey and dust
Descending flaccid paralysis with constipation, drooling and autonomic signs commons |
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Term
Young boy presents with fat cheeks, thin extremities and a protuberant abdomen.
Labs reveal hypoglycemia, lactic acidosis, hyperuricemi and hyperlipidemia.
What is your diagnosis? |
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Definition
| G6P deficiency (Von Gierke’s disease) |
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Term
| What do you think about in a child with evidence of self mutilation + dystonia? What do you do next? |
|
Definition
Lesch Nyan syndrome: XLR deficiency in HPRT (purine metabolism)
Increased uric acid leads to hypotonia and vomiting, usually by 6 months. |
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Term
| What do you think about in an infant with FTT, bilateral cataracts, HSM, jaundice and hypoglycemia |
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Definition
| Galactose-1-phosphate uridyl transference deficiency in Galactosemia. |
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Term
| What is the biggest risk factor for Cerebral Palsy? |
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Definition
|
|
Term
| What are the biggest RF for IVH in neonate? |
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Definition
| Prematurity and low birth weight |
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Term
| Which organisms cause brain abscess formation as complication of sinusitis? |
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Definition
| Staph aureus and step viridans |
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Term
| How can you differentiate between Cauda Equina and Conus medullaris is dysfunction? |
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Definition
1) Conus medullaris will be sudden onset, symmetric and hyperreflexic (UMN).
2) Cauda equine will be progressive, asymmetric and hyporeflrexic (LMN) |
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