Neuro combined 1 Flashcards
2 features of essential tremor? 2 conditions to rule out?
Worsens with age Occurs with certain postures Does not occur at rest Rule out hyperthyroidism and Wilson’s disease (ceruloplasmin)
3 Hz spike and wave on EEG?
Childhood absence epilepsy 3-8 years Ethosuzimide or VPA
A baby is born to a mother with myasthenia gravis. She exhibits generalized hypotonia and inability to suck. How do you confirm the diagnosis? What is your management? When can you expect resolution?
Infants born to moms with MG are affected because mom’s auto-antibodies cross the placenta. -Confirmation of diagnosis: administer IM neostigmine and watch for improvement -Treatment: oral pyridostigmine or neostigmine 30 minutes before feeding until resoltuion occurs -Resolution: approximately 3 wks (up to 10 wks)
A baby presents to yuo with a prominent occiput, broad forehead, absent anterior fontanelle and a long and narrow head. Otherwise normal. What is your diagnosis?
Primary craniosynostosis of the sagittal suture leading to scaphocephaly = long and narrow skull = boat shaped head!
A patient presents to the ED with sudden onset of headache with fall to the floor. On exam, he has a left central facial weakness, left hemiparesis with conjugate ocular deviation to the right. What is the diagnosis?
Stroke! Abrupt onset of hemisyndrome with eyes looking AWAY from the paralyzed side indicates acute stroke.
A patient presents with signs of meningitis, pleiocytosis in CSF with high protein and low glucose and a CT scan with contrast enhancement of the basal cisterns. What is your diagnosis?
TB meningitis -Exudate in the basal cisterns is classic -can also see tuberculomas, ringed lucencies, infarction
A teenager presents to you with progressive distal extremity weakness and myotonia (difficulty with muscle relaxation). He is also found to have cardiac arrhythmia, cataracts, male pattern baldness and hollowing of muscles around temples, jaw and neck. His face is expressionless with a tented mouth. What is the diagnosis?
Myotonic dystrophy
An infant presents to you a few weeks after a fall from the changing table with a soft, pulsatile palpable scalp mass. What is your diagnosis?
Leptomeningeal cyst: rare complication of linear skill fracture with separation of the edges of the fracture and laceration of the dura. The arachnoid membranes can then be trapped between the edges of the fracture and results in herniation of fibrotic tissue/meninges/brain to form a soft tissue mass. -requires surgical excision of abnormal tissue and dural repair
Apert syndrome
Associated with premature fusion of multiple sutures, including the coronal, sagittal, squamosal, and lambdoid sutures. Characterized by syndactyly of the 2nd, 3rd, and 4th fingers,
At what time of the day does benign myoclonus of infancy usually occur? -age of onset? -prognosis? -how to differentiate between infantile spasms?
Benign nonepileptic infantile spasms = brief tonic contractions occur in clusters -USUALLY at mealtimes -first occur at 3-8 mo of age, then resolve spont at 2-3 yo -really cannot differentiate on history or physical exam between these and infantile spasms (REMEMBER YOUR LITTLE BABY FROM ED) and thus, need to refer to neuro asap and get EEG/MRI -if EEG and MRI are normal and baby is developmentally normal, then this is benign myoclonus of infancy.
By definition, how long can a Todd’s paresis last up until?
24 hours maximum
Crouzon Syndrome ?
premature craniosynostosis and is inherited as AD trait
Differentiate tics from stereotypies?
Tics: asymmetric, occur when bored or distracted (not excited), onset at 4-5 years old, worsen with time into different movements, semivoluntary Stereotypies: usually symmetric, occur when excited or happy, same movement over time
Drug of choice for absence epilepsy?
Ethosuximide Valproate Lamotrigine
During what phase of sleep does neonatal sleep myoclonus occur in? -prognosis?
Non-REM sleep = bilateral rhythmic jerks involving upper and lower limbs -can be reproduced by slow rocking of the infant in a head to toe direction -no autonomic changes seen, occur ONLY in sleep, stop with awakening -this helps differentiate these from seizures -resolve spont at 2-3 mo age
Following a first unprovoked seizure, what is the risk of epilepsy over the next 5 years?
40%
How can reflexes help you differentiate between muscle vs. nerve problem?
Nerve problem = reflexes will be completely absent -muscle problem = reflexes will be present or reduced
How can you differentiate between absence seizures (generalized) versus complex partial temporal seizures?
Both cause cessation of activity, staring spells, and automatisms -Absence seizures: no postictal confusion, simple automatisms, provoked by hyperventilation, last a few seconds -Complex partial temporal seizures: + postictal confusion, more complex automatisms, last several minutes ****Impt to differentiate between these since anticonvulsant treatment is different!
How can you differentiate between positional plagiocephaly vs. craniosynostosis?
In positional plagiocephaly, should have ipsilateral anterior displacement of the ear!!!
How can you distinguish between tics versus true involuntary movement disorders (ie. chorea or myoclonus)? (2)
With tics: 1. Presence of premonitory sensory urge that is then relieved by the tic 2. Ability to suppress tics voluntarily
How do you classify gray matter neuronal degeneration conditions? -what are the conditions in each class? (4 each)
Separate into disorders WITH or WITHOUT hepatosplenomegaly -With HSM: 1. Mucopolysaccharidoses (Hurler syndrome, Hunter syndrome, Sanfilippo syndrome) 2. Mucolipidosis 3. Niemann-Pick disease 4. Gaucher disease -Without HSM: 1. Tay-Sachs 2. Rett syndrome 3. Neuronal ceroid lipofuscinosis 4. Mitochondrial diseases: ie. MELAS
How do you confirm a diagnosis for malignant hyperthermia?
Caffeine contracture test: muscle biopsy tissue specimen is exposed to caffeine = muscle spasm is diagnostic for malignant hyperthermia
How do you differentiate post infectious cerebellitis from meningoencephalitis? (3) -what are 3 potential etiologic agents causing post infectious cerebellitis?
- No fever 2. No altered LOC 3. No seizures 3 potential etiologic agents: 1. VZV 2. Coxsackie 3. Echovirus
Hypsarrhythmia on EEG?
Infantile spasms/West syndrome 3-8 M ACTG or Vigabatrin