Muscle and Nerve
My least favorites
Wide assortment
Shake it off
100
Seen on EMG after radiation plexopathy
Myokimic discharges
100
Wallenberg syndrome results from... and clinical symptoms...
occlusion of PICA
Ipsilateral Horner syndrome, ipsilateral hemiface sensory loss, contralateral sensory loss in the arm/leg, dysarthria and dysphagia, ipsilateral ataxia
100
During neurulation, failure of fusion of the posterior neuropore may lead to
spina bifida
3 subtypes
- spina bifida occulta, the vertebrae are not fully formed, but the spinal cord remains intact. often without symptoms or with minor issues, such as a small tuft of hair or a dimple on the back.
- meningocele: the meninges protrude through the opening, but the spinal cord itself remains intact and in place.
- myelomeningocele: both the spinal cord and meninges protrude through the opening in the spinal column
100
Family hx of stroke, migraines, seizures, mood disorders
What is the disease? What is the gene?
CADASIL
NOTCH 3 MUTATION on Cr 19
100
first line treatment for cervical dystonia
botulinum toxin
200
X linked, CAG trinucleotide repeat, muscle weakness, atrophy of facial, bulbar, limb muscles, fasciculations
Kennedy's disease
proximal>distal, hyporreflexia, males, gynecomastia, infertility, testicular atrophy.
200
finger agnosia, acalculia, agraphia, left-right disorientation
Gerstmann syndrome
angular gyrus affected (inferior parietal dominant hemisphere)
200
Nucleu within the RAS that primarily involves serotoninergic neurotransmission
Raphe nuclei
dopamine--> SN, ventral tegmentun
Ach--> basal nucleus of Meynert
GABA--> nucleus accumbens
norepi--> locus ceruleus
200
masses with "fried egg appearance" and non enhancing solitary with regions of calcification
oligodendroglioma
200
GAA expansion in FXN gene on Cr 9
Friedrich ataxia
gait ataxia, cerebellar dysarthria, loss of proprioception and vibration, hypertrophic cardiomyopathy, reduced or absent DTR, DM,.
MRI: atrophy of spinal cord and medulla
300
Stiffness that improves with activity, CLCN1 mutation
Myotonia congenita
300
Diffuse, poorly demarcated, large >1-2 cm lesions in cerebral white matter in children after infection or vaccine
ADEM
300
Name 3 NREM parasomnias
Sleep walking, confusional arousal, sleep terror, sexsomnia, sleep related eating disorder
REM: RBD, nightmares, sleep paralysis
300
Serotonin 5HT1F receptor agonist for acute migraine
lasmiditan (Reyvow)
useful in patients with cardiac history
300
Safinamide and selegiline for PD belongs to which medication class
MAOB- inhibitors
adjunctive treatment with levodopa for motor fluctuations and dyskinesia
400
hyperexcitability+ dysautonomia+encephalopathy+neuropathic pain+ insomnia
Morvan syndrome
also associated with VGKC antibodies (thymoma) and CASPR 2
Issacs syndrome does not have CNS manifestations
400
Disease characterized by neuropathy, angiokeratoma, cardiorenal disease, stroke, corneal deficits
Fabry disease
X linked, alpha galactosidase A activity deficiency, mutation GLA gene
400
polymicrogyria results from
cortical organization failure
400
Nasal GCRP receptor antagonist for acute migraine treatment
zavegepant (Zavzpret)
400
Treatment for Friedrich ataxia (activates Nrf2 pathway functioning as an antioxidant) and inhibits nuclear factor kappa B
omaveloxolone
500
symmetric sensory or sensorimotor dysfunction in lower extremities, absent DTRs, more likely to have monoclonal gammopathy, some may have also anti myelin associated glycoprotein
DADS (distal acquired demyelinating symmetric neuropathy)
DADs WITH monoclonal IgM tends to be resistant to standard therapies for CIDP
500
Prolonged P100 on VEP indicates...
demyelination of optic nerve
500
A 2-month-old male with a history of irritability, failure to thrive, stiffness with clenched fists, and recurrent fevers without an identified source of infection starts to have seizures and become increasingly stiff with scissoring of the legs and arching of the back.
GALC gene
globoid cell leukodystrophy (Krabbe disease)
500
walking difficulties as adult, childhood behavioral problems and adrenal insufficiency are clinical phenotypes of..
X linked adrenoleukodystrophy
-childhood cerebral
-adolescent cerebral
-adult cerebral
-adrenomyeloneuropathy
-adrenal insufficiency
500
EEG findings (high amplitude delta waves that occur bilaterally and synchronously at a fixed interval) in an immigrant patient to US with altered mental status, abnormal jerking movements
Subacute sclerosing panencephalitis: complication of measles virus