Rite Questions Flashcards
Breathing pattern and location?
Cheyne stoke
Ataxic
Apneustic
Cluster
Central neurogenic hypervent
Central neurogenic hypo
Breathing pattern locations?
Cheyne, ataxic, apneustic, cluster, hyper and hypoventilation
Dorsal Spinocerebellar tract pathway?
first-order neurons carry input from the periphery to a large nucleus in the posterior gray horn known as Clarke nucleus, which ranges from C8-L3 (Clarke column). From there, second-order neurons ascend in the ipsilateral DSCT and enter the cerebellum through the inferior cerebellar peduncle before terminating in the cerebellar cortex.
Onus Nucleus? rubrospinal tract pathway?
Onuf nucleus is a gray matter structure in the ventral horn of the sacral spinal cord. It is primarily involved in the conscious control of micturition and defecation and also plays a role in orgasm. Unlike the DSCT, it is not a sensory structure.
rubrospinal - red nucleus to LMN SC - inhibits extensor muscles - lesion causes decerebrate posturing
AED SE of oligohidrosis? Alopecia?
oligo- zonisamide
alopecia -VPA
dejerine rouse syndrome?
pain after thalamic stroke
tx w/ gabapentin
Balint syndrome?
Location of stroke?
triad of optic ataxia, oculomotor apraxia, and simultagnosia (can’t perceive more than one object at a time)
b/l parietal
Function of:
Lateral vestibular nucleus?
Medial?
descending?
Superior?
MVN/SVN Go UP to eyes for Vestibulocular reflex
descending go down FROM OTOLITHS TO Spinal cord via LINEAR MOVEMENTS
LVN - from cerebellum to spinal cord an extensor posturing
The MVN, along with the SVN, mediates the VOR, which aids in eye rotation to the opposite side during horizontal head movements. The MVN receives input from the lateral semicircular canals and sends efferent fibers through the MLF to coordinate eye movements in response to head movements. LVN efferent fibers regulate extensor tone and are not related to coordinating eye movements.
The MVN, along with the SVN, mediates the VOR, which aids in eye rotation to the opposite side during horizontal head movements. The MVN receives input from the lateral semicircular canals and sends efferent fibers through the MLF to coordinate eye movements in response to head movements. LVN efferent fibers regulate extensor tone and are not related to coordinating eye movements.
The lateral vestibular nucleus (LVN) receives input from the ipsilateral flocculonodular lobe (vestibulocerebellum) regarding head tilt and gravity. Fibers from the LVN then descend in the ipsilateral ventral horn of the spinal cord to regulate extensor tone of the back and extremity muscles on the ipsilateral side in response to head movements.
Buccal nerve is branch of what branch trigeminal?
Where inferior alveolar nerve located?
V2 maxillary
mandible, blocked during molar extraction
EEG findings in JME? Hz for interictal and myoclonic jerks?
The interictal EEG (pictured below) has 4- to 6-Hz spike or polyspike and slow-wave complexes. The ictal EEG with the myoclonic jerks typically shows 10- to 16-Hz polyspike discharges.
cauda equina vs conus?
conus usually numbness mainly perianal, less pain/sciatica
Developmental malformations that are due migration issues? organizational? Proliferation?
Migration: lissencephaly (type 2=cobblestone), heterotopia
organization: schizencephaly and polymicrogyri
proliferation - microcephaly, hemimegalacephaly
location of MLF, corticobulbar/spinal tract, pontocerebellar, vestibular nuclei?
Monomelic amyotrophy?
Neuralgic amyotrophy? (parsonage turner) - 97% involves which nerve?
Monomelic amyotrophy or Hirayama disease is a rare lower motor neuron disorder that typically presents with distal upper extremity weakness in juveniles of Asian descent. Proximal involvement is rare. Sensory symptoms are minimal.
S100 positive and verocay bodies?
Scwhannomas……Histologically, they are characterized by compact, hypercellular areas of elongated cells called Antoni A bodies and loosely arranged hypocellular areas called Antoni B bodies. Antoni A bodies also tend to palisade around hypocellular areas called Verocay bodies (2 nuclear palisading bodies w/ hypo cellular center)
Laminated calcifications with spindle cells in a whorled pattern ?
Pseudopalisading cells with central necrosis is consistent with ?
Round nuclei with clear cytoplasm is the typical presentation?
Laminated calcifications with spindle cells in a whorled pattern ? Meningioma
Pseudopalisading cells with central necrosis is consistent with ? GBM (also GFAP +)
Round nuclei with clear cytoplasm is the typical presentation? oligodendrolgioma (often frontal)
parasympathetic N that feeds submandibular gland?
The greater petrosal nerve provides parasympathetic innervation to the lacrimal, nasal, and palatine glands. The parasympathetic secretomotor fibers of the submandibular gland arise from the chorda tympani, which is distal to the greater petrosal nerve.
SCA1 vs 2 vs 7?
SCA 1 has cerebellar and brainstem atrophy
2 has MOST cerebellar atrophy
7 - younger (teen young adult)-myoclonus and seizures too, retinal degeneration
SCA1 is caused by a CAG repeat expansion in the ATXN1 gene on chromosome 6. It typically manifests around age 30-40 and is characterized by progressive cerebellar ataxia, dysarthria, and bulbar dysfunction
Dentatorubral-pallidoluysian atrophy?
similar to what other CAG repeat dz
Dentatorubral-pallidoluysian atrophy is caused by a CAG trinucleotide repeat expansion of the polyglutamine region of the atrophin-1 gene on chromosome 12p. Because it is most associated with ataxia, choreoathetosis, and dementia, it is most similar to Huntington disease (HD). In young-onset cases, seizures and myoclonus are also common. Although MRI may show cerebellar atrophy, as here, it also shows brainstem atrophy, calcification of the basal ganglia, and leukodystrophic changes.
Chromosome of HD? SCA? Dentatorubral-pallidoluysian atrophy?
4 - HUNT
SCA - 6
DP - 12
gamma motor N innervate?
Each skeletal muscle fiber is innervated by a single motor axon from the alpha motor neurons in the anterior horns of the spinal cord. The same axon may also innervate other muscle fibers. All the fibers innervated by the same axon are called a motor unit. Skeletal muscles contain specialized proprioceptive sense organs, called muscle spindles, which function to detect muscle stretch. Each muscle spindle consists of an encapsulated cluster of small striated muscle fibers (intrafusal muscle fibers). Each fiber has a mechanosensory nerve ending (the most prominent of these are called annulospiral endings) which wraps around the mid-region of the fiber; this sensor produces nerve impulses in response to stretch. Each fiber also receives motor innervation from a γ (gamma) efferent nerve fiber; impulses in this fiber cause the spindle muscle fiber to contract.
How to test for 4th nerve?
- vertical misalignment
- misalignment worse which direction? (opposite lesion)
- head tilt worse? (same side as lesion)
Trochlear nerve palsies present with (1) vertical misalignment (higher eye or hypertropia), (2) diplopia worsened in contralateral gaze to the higher eye, and (3) diplopia worsened in ipsilateral head tilt toward the higher eye.
The trochlear nucleus can be found in the caudal midbrain. The right superior oblique (trochlear nerve) nucleus is in the contralateral (left) midbrain. The occulomotor nerve nuclei (occulomotor nucleus and Edinger-Westphal nucleus) are both located in the rostral midbrain. The abducens nucleus is in the caudal pons.
ant dislocation of shoulder affects?
axillary nerve (arises from C5-6) - forms sup trunk before going to posterior cord and splits to axillary and radial
The axillary nerve innervates the deltoid (shoulder abduction >15°), teres minor (external rotation and shoulder adduction), and the triceps (long head only). The superior lateral cutaneous nerve is the sensory branch of the axillary nerve and supplies the upper lateral shoulder.
Histopathology of sarcoid? where lesions usually found?
Image B is a Masson trichrome stain that shows pink granulomas against dense blue collagenous connective tissue.
Sarcoidosis is an inflammatory granulomatous disease. Commonly described neurologic manifestations of sarcoidosis include cranial neuropathies (specifically II and VII), meningitis or encephalitis, peripheral neuropathy, and myopathy.
Lower trunk injury leads to?
claw hand
The most classic presentation of a lower trunk brachial plexus injury is the “claw hand,” in which the forearm is supinated and the wrist/fingers are flexed. This is also known as Klumpke palsy affecting the C8-T1 nerve roots. The C8-T1 roots form the lower trunk of the brachial plexus.
Illustration © TrueLearn, LLC
The hand and finger weakness are due to the effect on flexor muscles of the wrist and fingers, forearm pronator, and intrinsic hand muscles.
Sometimes patients will have sensory loss on the ulnar aspect of the hand and forearm. If the T1 root is damaged proximal to the sympathetic trunk, there may be an associated Horner syndrome. Biceps, brachioradialis, and triceps reflexes will be normal, and the finger flexor reflex will be absent.
Common causes include upward traction injuries (grabbing a branch during a fall from a tree or being dragged unconscious on a floor), thoracic outlet syndrome, and Pancoast syndrome. It also may also occur during a difficult delivery if the infant’s arm is pulled with upward traction.
Lesions in orbitofrontal cause?
ventrolateral?
rostral?
dorsolateral?
medial prefrontal?
Lesions to the orbitofrontal cortex of the prefrontal region can result in a lack of affect, inappropriate social behaviors, as well as risky behavior (ie, gambling, sexual acts, etc).
The rostral prefrontal cortex has been associated with disorganized behavior in everyday situations as well as difficulty with making everyday decisions.
Answer C: The dorsolateral prefrontal cortex is comprised of Brodmann areas 9 and 46. Lesions of the dorsolateral prefrontal cortex have been associated with deficits in working memory function, planning, and attention. The dorsolateral prefrontal cortex is also involved in encoding and retrieval of episodic memory.
Answer D: The ventrolateral prefrontal cortex is comprised of Brodmann areas 44, 45, and 47. This region houses the Broca area; therefore, lesions in this region can lead to Broca aphasia as well as spatial inattention and neglect.
Answer E: The medial prefrontal cortex is comprised of a dorsomedial region, which comprises Brodmann areas 8, 9, 10, 24, and 32, while the ventromedial region is comprised of Brodmann areas 10, 12, 14, and 25. Damage to the medial prefrontal cortex can cause a multitude of symptoms such as abulia, emotional lability, inattention, problems with decision-making, as well as akinetic mutism.
Good prognosis for GBM if O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation?
IDH mutation?
yes both are good
IDH WILD TYPE = BAD
Benedikt syndrome? Claude?
The case is describing Benedikt syndrome. It is due to a lesion in the ventral midbrain (on the right in this case) involving the following structures: red nucleus, oculomotor fascicles, cerebral peduncle, and substantia nigra. The syndrome presents with ipsilateral cranial nerve III palsy and contralateral involuntary movements (intention tremor, hemichorea, or hemiathetosis). It can also present with hemiparesis due to the involvement of the cerebral peduncle. This area of the brainstem is supplied by deep penetrating arteries from the posterior cerebral artery or paramedian penetrating branches of the basilar artery.
A similar syndrome is Claude syndrome which is when there is ipsilateral oculomotor nerve palsy and contralateral ataxia, suspected to be from involvement of the oculomotor fascicles and the dentato-rubro-thalamic tract.
Answer B: Weber syndrome is due to a lesion in the medial midbrain/cerebral peduncle. It presents with ipsilateral cranial nerve III palsy and contralateral hemiparesis. This area is supplied by the deep penetrating artery from the posterior cerebral artery.
Answer C: Foville syndrome is due to a lesion involving the caudal pontine tegmentum and the facial colliculus. It presents as ipsilateral cranial VI and VII palsies +/- contralateral hemiparesis. This area is supplied by the pontine perforator branches off of the basilar artery.
Answer D: Wallenberg syndrome is due to a lesion in the lateral medulla, which is supplied by the posterior inferior cerebellar artery. It presents with numerous symptoms including: Ipsilateral facial and contralateral body hypalgesia and thermoanesthesia; ipsilateral palatal weakness; dysphagia, dysarthria, nystagmus, vertigo, nausea/vomiting; ipsilateral Horner syndrome; skew deviation. With this, consider disease in the parent vertebral artery.
Answer E: Dejerine syndrome is due to a lesion in the medial medulla, which is supplied by the vertebral artery or anterior spinal artery. This presents with ipsilateral tongue weakness and contralateral hemiparesis, +/- contralateral loss of proprioception, and vibration.
Weber syndrome?
Weber syndrome is due to a lesion in the medial midbrain/cerebral peduncle. It presents with ipsilateral cranial nerve III palsy and contralateral hemiparesis. This area is supplied by the deep penetrating artery from the posterior cerebral artery.
foville syndrome?
Foville syndrome is due to a lesion involving the caudal pontine tegmentum and the facial colliculus. It presents as ipsilateral cranial VI and VII palsies +/- contralateral hemiparesis. This area is supplied by the pontine perforator branches off of the basilar artery.
foville syndrome?
Foville syndrome is due to a lesion involving the caudal pontine tegmentum and the facial colliculus. It presents as ipsilateral cranial VI and VII palsies +/- contralateral hemiparesis. This area is supplied by the pontine perforator branches off of the basilar artery.
Wallenburg syndrome? Artery? Sx?
nucleus ambiguous vs nucleus solitarius?
The nucleus solitarius is a purely sensory nuclei located in the medulla. The main role of the nucleus solitarius is autonomic regulation via its projections to the reticular formation, parasympathetic preganglionic neurons, hypothalamus, and thalamus. A lesion to the nucleus solitarius would not explain the motor findings in this patient, including dysarthria or uvula deviation.
The nucleus ambiguus is a collection of motor neurons and preganglionic parasympathetic neurons located deep in the medullary reticular formation. It is located dorsal to the inferior olive and is near the lateral upper medulla. Due to the presence of both motor and parasympathetic fibers, it functions to innervate the muscles of the soft palate, pharynx, and larynx and assists in speech and swallow. Additionally, the parasympathetic neurons innervate the heart to aid in control of the heart rate.
When there is a lesion of the nucleus ambiguus, nasal speech, dysarthria, and dysphagia may be seen. Additionally, contralateral deviation of the uvula may be present due to weakness of the laryngeal muscles.
3 areas that can cause palatal myoclonus? which contralateral?
CASPR (Morvan syndrome) sx? screening?
The classic triad of peripheral nerve hyperexcitability, dysautonomia, and encephalopathy,
Also LGI1
19:1 males to females
Screen for thymoma
muscle that abducts arm 15? 15-90? what is each nerve a branch of in brachial plexus?
first 15 - supraspinatus - upper trunk
deltoid - axillary- br off posterior cord
hereditary hyperekplexia gene?
GLRA1 (Glycine alpha receptor mutation on Chromosome 5), SLC6A5, GLRB, GPHN, and ARHGEF9.
Increased glycine in CSF causes what sx?
neonatal severe seizures and encephalopathy
nonketotic hyperglycinemia
tx for sydenhams chorea?
typical or atypical antipsychotic need D2 blocking
d2 agonist not first line
Treatment of Sydenham chorea includes antibiotic therapy with penicillin. The first-line treatment for the chorea is with a dopamine receptor antagonist, such as a typical or atypical antipsychotic.
rufinamide mechanism? can use in LGS?
Yes
modulation of activity in sodium channels, particularly prolongation of the inactive state.
felbamate mechanism?
inhibition of voltage-sensitive sodium and calcium channels, reduction of glutamergic transmission through modulation of NMDA receptors, and potentiation of GABA transmission
adies pupil pathophysiology? whee degeneration?
degeneration of post ganglioninic parasympathetic or ciliary ganglion, uopregulate post syndrome receptors so should respond to diluted pilocarpine. If need concentrated then medicine induced (atropine)
cocaine with Horner’s? what happens? amphetamine?
Answer C: Cocaine drops result in pupil dilation in 45 to 60 minutes of adminstration because they block norepinephrine reuptake by the presynaptic nerve terminal. Cocaine is used in the diagnosis of Horner syndrome. The affected pupil will minimally dilate or fail to dilate due to a lack of norepinephrine release.
Answer D: Hydroxyamphetamine is used to aid in the localization of lesions in Horner syndrome. It causes release of norepinephrine into the neuromuscular junction and, therefore, dilates the pupil with preganglionic lesions but not postganglionic ones.
cavernous malformation MRI findings?
On T2-weighted MRI, a typical cavernous malformation appears as a well-defined lesion with a central core of mixed-signal intensity surrounded by a rim of hypointensity (RIM is key)
histology schwannoma vs perineuroma vs neurofibroma vs ganglioneuromas
Neurofibroma – dark nuclei with wavy dark nuclei in a fibrotic storm
A: Schwannomas are the most common tumor of the peripheral nerves. They are made entirely of benign neoplastic Schwann cells. The presence of nerve fibers makes this answer incorrect.
Answer B: Perineuromas are composed of only perineural cells. These can clinically mimic schwannomas. On pathology, there is pseudo–onion bulbing that differentiates it from schwannomas. (ONOION)
Answer C: Ganglioneuromas are large slow-growing tumors that arise from sympathetic ganglion cells. The patient in this case has a tumor in her brachial plexus, which excludes this answer. (SYMPATHETIC!!!!)
IBM weakness pattern? mutation? histology?
The muscle biopsy finding most typical of inclusion body myositis is vacuole formation surrounded by a basophilic rim (rimmed vacuoles).
Inclusion body myositis is an inflammatory condition in older individuals (male predominant), which is slowly progressive. Clinical features of early weakness and atrophy of select muscles are found in quadriceps, flexors of the forearms, and ankle dorsiflexors. Muscle enzyme levels are normal or minimally increased, and EMG studies typically reveal an inflammatory myopathy.
Anti-NT5c1A protein has moderate sensitivity and high specificity for
mcardles disease abnormality?
myophosphorylase
The diagnosis of McArdle disease can be ascertained using four features that are commonly present which include: exercise intolerance, high serum creatine kinase (CK) levels, 1 or more episodes of elevated serum CK after exercise, and the “second wind” phenomenon. The “second wind” phenomenon is described as a sudden improvement in aerobic exercise after about 10 minutes if the patient takes a brief rest at the first signs of exercise intolerance. This phenomenon can be explained by the increased blood flow to muscles with better delivery of free fatty acids. Treatment strategies include a diet high in complex carbohydrates and consuming simple carbohydrates shortly before any strenuous exercise.
learn Sayre syndrome?
Of the choices offered in the question, only Kearns-Sayre myopathy is a mitochondrial disease. Kearns-Sayre syndrome is caused by large deletions in mitochondrial DNA. Neurologic manifestations include ophthalmoplegia, ptosis, and generalized weakness and often eventually progress to dementia, hearing loss, retinitis pigmentosa, and autonomic neuropathy. Systemic manifestations include heart block, cardiomyopathy, and diabetes. Mitochondrial DNA deletions (as opposed to mutations) are almost always sporadic.
Part (a) shows hematoxylin and eosin stain (arrows indicate fibers with an increase in basophilic staining indicative of mitochondrial hyperplasia), part (b) shows modified trichrome stain (arrows indicate ragged red fibers), and part (c) shows cytochrome c oxidase (COX) stain (asterisks mark COX-negative fibers). Electron microscopy (d-f) shows mitochondrial hyperplasia, variation in the mitochondrial shape and size, and mitochondria with crystalline arrays (arrowheads).
nightmare is REM or NREM parasomnia?
REM
toxicity with garlic breath? alopecia?
arsenic garlic
thallium alopecia
Arsenic toxicity acutely presents with abdominal pain, vomiting, and diarrhea. An axonal neuropathy of both motor and sensory fibers is evident after the first and second week of exposure, which tends to follow an ascending pattern, similar to that of Guillain-Barré syndrome. Pancytopenia and basophilic stippling of red blood cells (RBCs) can be seen. Dermatologic findings include oral ulcers, pruritic macular rash, and Mees lines in the fingernails and toenails. A persistent garlic breath is characteristic of arsenic toxicity. Treatment is mainly supportive in the chronic stage, but, in the acute setting, chelating agents such as dimercaprol and succimer may be recommended.
Answer A: Thallium toxicity presents with a severe axonal sensorimotor neuropathy with burning paresthesias of the feet. Alopecia is characteristic and can be seen weeks after intoxication (aids in the differential diagnosis). Patients can develop uremia and hepatic dysfunction. This type of intoxication does not present with a garlic smell.
Answer C: Lead toxicity classically presents with upper or lower extremity weakness, typically with wrist and finger drop. There is not an ascending pattern, as is seen with arsenic, and there are typically no sensory issues. Basophilic stippling of RBCs can be seen. It is treated with chelation.
Answer D: Garlic breath and dermatologic findings are not characteristics of Guillain-Barré syndrome.
Answer E: Mercury toxicity is characterized by intention tremor, paresthesias, ataxia, sialorrhea, changes in personality, and depression, anxiety, or both. The oral mucosa can show inflamed gums. Treatment includes penicillamine and 2,3-dimercaptopropane-1-sulfonate.
thalamic pain syndrome pseudonym? tx?
Dejerine-Roussy syndrome
gabapentin, lamictal, TCA
AED that can cause Parkinsonism?
Valproic acid use is associated with the development of a tremor, which is typically postural but can occasionally be resting; drug-induced parkinsonism has occurred as the result of chronic use of valproic acid.
Combined sensory index > —– is indicative of CTS?
In a carpal tunnel syndrome study, the combined sensory index (CSI) is used as an attempt to maximize sensitivity without reducing specificity. In the CSI, the results of several tests are taken into account, including the ringdiff (antidromic), thumbdiff (antidromic), and the palmdiff (orthodromic).
1.0 ms
taste for 2/3 ant tongue? post 1/3? pharynx?
First-order neurons for taste are present in the tongue, soft palate, pharynx, and upper esophagus. First-order neurons project from the following distributions as follows:
Anterior 2/3 taste buds -> Chorda tympani (a branch of CN VII)
Posterior 1/3 taste buds -> Lingual branch of CN IX
Pharynx/epiglottis -> CN X
These three branches of these three cranial nerves eventually synapse together in the nucleus solitarius, which has two portions: the rostral and caudal nucleus solitarius. The caudal nucleus solitarius assists in central cardiopulmonary regulation and is only fed by CN IX and X. The rostral nucleus solitarius is the gustatory nucleus and is (as above) fed by CN VII, IX, and X. Interestingly, the sections of CN IX and X that feed these two different nuclei have two different embryologic origins. Of note, the rostral nucleus solitarius is supplied by the PICA, and the caudal nucleus solitarious is supplied by the posterior spinal artery (PSA).
After coalescence in the rostral nucleus solitarius, second-order neurons project to the ventral posteromedial nucleus of the thalamus. Finally, third-order neurons project to the gustatory cortex, located in the insula and frontal opercular region.
spinomid contraindicated in what pts?
Siponimod was approved by the US Food and Drug Administration (FDA) in 2019 for the treatment of relapsing forms of multiple sclerosis. Siponimod is an oral disease-modifying agent from the same family of medications as fingolimod and, thus, acts as a sphingosine-1-phosphate receptor modulator, leading to receptor internalization and destruction of T and B cells. Siponimod is metabolized in the liver through the CYP2C9 enzyme.
Prior to initiation of the medication, it is recommended that patients be tested for CYP2C9 variants to determine CYP2C9 genotype and have a complete blood count with differential, liver function tests, varicella zoster virus titer level, an ECG, and ophthalmologic examination.
Additional contraindications include the following:
In the last 6 months: Myocardial infarction, unstable angina, stroke, TIA, decompensated heart failure requiring hospitalization, or Class III/IV heart failure
Presence of Mobitz type II second-degree, third-degree AV block, or sick sinus syndrome (unless the patient has a functioning pacemaker)
ANNA2?
Anti Ma2
VGCC
VGKC (potassium)
Anti MAG
ANNA-2 antibody (anti-neuronal nuclear autoantibody type 2), also known as anti-Ri, is associated with breast, gynecologic, lung, and bladder cancers and can cause ataxia with or without opsoclonus/myoclonus syndrome.
Answer A: Anti-Ma2, typically from testicular cancer, can cause limbic and brainstem encephalitis.
Answer C: Anti-VGCC (voltage-gated calcium channel), associated with small cell lung cancers, can cause Lambert-Eaton myasthenic syndrome.
Answer D: Anti-VGKC (voltage-gated potassium channel), associated with thymoma, prostate, and small cell lung cancer, can cause seizures, vertigo, peripheral neuropathy, and neuromyotonia.
Answer E: Anti-MAG (myelin-associated glycoprotein), associated with Waldenstrom macroglobulinemia, can cause peripheral neuropathy.
EMG with waxing and waning myotonia?
“dive bomber”
myotonic dystrophy type 1
progressive encephalomyelitis with rigidity and myoclonus (PERM)? sx and antibodies?
This case describes progressive encephalomyelitis with rigidity and myoclonus (PERM).
This is a rare autoimmune (occasionally paraneoplastic) neurologic disorder. Up to 30% of patients have a concurrent autoimmune disorder. It presents with a subacute encephalopathy with intermittent spells of rigidity and startle myoclonus. The patient frequently will have autonomic instability, presenting with fevers. Postinfectious causes have been seen following West Nile virus or brucellosis, but this exceedingly rare. This condition can be associated with thymomas. Treatment is with immunotherapy from high-dose steroids, intravenously administered immunoglobulins, plasma exchange or a combination of these therapies. The rigidity typically requires high doses of benzodiazepines.
Autoantibodies against the following targets have been found: GAD-65, glycine, DPPX, and amphiphysin.
stroke to where causes hemiballismus?
Lesions to the subthalamic nucleus from various causes classically cause hemiballismus.
what is jitter in single fiber emg?
Single-fiber EMG is the most sensitive test for myasthenia gravis. This technique allows simultaneous recording of the action potentials of 2 muscle fibers innervated by the same motor axon. The variability in time of the second action potential relative to the first is called “jitter.” Any disorder, such as myasthenia gravis, that reduces the efficacy of transmission at the neuromuscular junction will produce increased jitter.
The Boston Naming Test (BNT) consists of pictures of 60 items that the patient is asked to name. The patient is given 20 seconds to name each object. If the patient does not know the answer, they are given a phonemic cue (first syllable of the word). The BNT assesses naming and presence of aphasia. Localization is to the left temporal lobe.
Answer A: Trail making requires participants to connect items in increasing order and, at the same time, to alternate between tasks. For example, alternating numbers and letters from “1” to “A,” to “2” to “B,” to “3” then “C,” and so forth. This primarily tests executive function, which localizes to the prefrontal lobes.
Answer B: Serial subtraction primarily tests attention (which localizes to the frontal lobes) and calculation (which localizes to the left parietal/inferior parietal lobule).
Answer D: Similarities is a test in which participants are asked how 2 items are similar. For example, an apple and an orange are both fruits; a train and a bicycle are both modes of transportation. This tests primarily executive function and localizes to the prefrontal lobes.
Answer E: Although copying the Rey-Osterrieth complex figure does assess visuospacial skill, the localization is not restricted to the occipital lobe but also involves temporal and parietal lobes.
epidural vs subdural?
SUBDURAL CROSSES SUTURE LINES
why in IIH have diplopia? which nerve?
Cranial nerve VI has a unique course. As it exits the pons ventrally in the subarachnoid space, it makes a steep angle to ascend vertically along the clivus and makes another turn in Dorello’s canal via the petroclinoid ligament. Changes in intracranial pressure cause stretching in this portion, rendering cranial nerve VI susceptible to injury and leading to a sixth nerve palsy.
nervus intermedium branch of? which ganglion?
facial
geniculate
medial geniculate goes to superior or inferior temporal lobe? function of sup and inferior?
The projections of the medial geniculate nucleus terminate on the superior temporal gyrus. This network is part of the auditory circuit. Auditory inputs project from the organ of Corti to the ipsilateral cochlear nuclei. This then sends projections to the contralateral inferior colliculus via the lateral lemniscus. The inferior colliculus then sends bilateral projections to the medial geniculate body. The medial geniculate body is composed of several subnuclei. The predominant integrating nucleus is the medial geniculate nuclei. The other subnuclei include the lateral and dorsal nuclei of the medial geniculate body. The medial geniculate nuclei then terminate on the superior temporal gyrus, which is the primary auditory cortex.
Inf temporal can lead to PROSAPGNOSIA
The inferior temporal gyrus is involved in higher cortical visual processing and object recognition. The fusiform gyrus, also called the fusiform face area, is a substructure of the inferior temporal gyrus. Lesions of this gyrus can produce the classic prosopagnosia, which is the inability to distinguish faces.
dextrosemethoprhan-quinidine mechanism?
Dextromethorphan-quinidine is the first and only medication approved by the Food and Drug Administration for pseudobulbar affect/palsy. Dextromethorphan is a noncompetitive N-methyl-D-aspartate glutamate receptor antagonist and a serotonin and norepinephrine reuptake inhibitor. To block dextromethorphan hepatic metabolism, quinidine, a cytochrome P450 2D6 inhibitor, is administered with dextromethorphan.
shuddering vs breath holding? which usually larger stimulus?
shudder larger stimulus
Answer A: Breath-holding spells are common events in infants and young children from 6 months to 6 years of age. These usually run in families (about 30% of cases) and have 2 types: pallid breath-holding spells and cyanotic breath-holding spells. In the cyanotic variety, the child becomes angry or upset in response to a reprimand or a mild injury. The precipitant is often minimal, even trivial. There is a brief period of crying, typically followed quickly by breath-holding in forced expiration with apnea and cyanosis, which is then often followed by collapse with limpness and loss of consciousness.
Answer C: Hyperekplexia (stiff-baby syndrome or startle disease) is a rare genetic disease that has been associated with a variety of gene mutations usually affecting the glycine receptor. The disease is characterized by a triad of generalized stiffness while awake, nocturnal myoclonus, and an exaggerated startle reflex. These features are often apparent at birth. Episodes of hypertonia or tonic spasms occur upon awakening or with auditory or tactile stimuli.
Answer D: Infantile spasms are epileptic spasms that are usually part of West syndrome. Events are unprovoked and have no relation to feeding. Infants are almost always delayed in milestones, and their neurologic exam is abnormal.
Answer E: Shuddering attacks usually begin in infancy, less commonly in childhood. These brief episodes of altered muscle tone often manifest as a rapid tremor of the head, shoulder, and trunk reminiscent of a “shudder” or “shiver” from a chill. There may be stiffening, flexion, and elevation of the arms with a low-amplitude tremor. Episodes are provoked by strong emotions and sometimes by feeding.
Bottom Line: Sandifer syndrome refers to the intermittent paroxysmal spells of generalized stiffening and opisthotonic posturing that are caused by gastroesophageal reflux in infants.
possibility of Horners due to lesion above which level?
T1
tx of TD? mechanism?
For patients with a diagnosis of TD, additional pharmacologic interventions include the use of benzodiazepines, botulinum toxin injections, and vesicular monoamine transporter 2 (VMAT2) inhibitors.
The VMAT2 inhibitors may be useful therapeutic agents for TD. These agents act centrally by depleting dopamine storage in presynaptic vesicles. Examples include valbenazine, tetrabenazine, and deutetrabenazine.
ia type that causes cerebral sx?
Plasmodium falciparum is the causative agent of cerebral malaria, which is characterized by fever, chills, headache, seizures, and eventual coma.
balling syndrome?
This is the location of Bálint syndrome, which is characterized by optic ataxia (deficit of reaching objects under visual guidance), ocular apraxia (inability to voluntarily move the eyes when oculomotor function is intact), and simultagnosia (inability to visually perceive more than 1 object at a time).
parietal occipital region - bilateral
This is the location of Bálint syndrome, which is characterized by optic ataxia (deficit of reaching objects under visual guidance), ocular apraxia (inability to voluntarily move the eyes when oculomotor function is intact), and simultagnosia (inability to visually perceive more than 1 object at a time).
Anton syndrome?
This patient is showing signs consistent with Anton syndrome. Bilateral medial occipital lobe lesions produce visual anosognosia, characterized by cortical blindness, denial of such blindness, and confabulation.
panic disorder? how many attacks needed?
1 attack w/ 1 month of fearing further attacks
sx reach peak in 10 min
Polymyositis Antibodies? screening?
anti-Ro, anti-La, and anti-Jo-1 (myositis-specific antibody) levels
CT chest and PFT - r/o ILD
Visual evoked potentials P100 cutoff?
<117, if prolonged then nerve damage of pic nerve
methanol effects which part of brain/nerves/SC? CO? Manganese? B6?
methanol - putamen b/l
CO and manganese - globus pallidus
B6 - dorsal root ganglion (neuronopathy)
Nerves that are in inguinal canal?
iliohypogastric and external spermatic (genital br of genitofemoral nerve) - innervates anterior anterior scrotum (mons pubis/labia major in females)
and cremaster reflex
Localization of post ictal nose wiping? unilateral eye blinking?`
IPSALATERAL TO BOTH
Mechanism and common sode effects?
Fingolimid
Alemtuzemab
Glatirmer acetate
Nataluzimqb
Ocrelizumab
Teriflunomide
Dimethyl fumarate
Mechanism and common sode effects?
Fingolimid headache/cardiac
Alemtuzemab thyroid
Glatirmer acetate injection site pain, hypersensitivity
Nataluzimqb pml, hepatotoxicity
Ocrelizumab URI/infusion
Teriflunomide teratogenic, hepatotoxicity
Dimethyl fumarate flushing, angioedema, rare PML, N/D
sumatriptan receptors?
5HT1B - vessel constriction meninges
5HT1D - central nociceptive receptors
hyperventilation w/ SAH?
No can cause vasospasm
What is the wave w/ spike and wave? depolarization? hyper?
Spike and waves are generated by thalamocortical loops. Spikes represent cortical depolarization, whereas the wave is currently associated with hyperpolarization of pyramidal neurons.
neural crest forms CNS or PNS?
The neural crest participates in the formation of important peripheral nervous system structures, including the dorsal root ganglia and Schwann cells.
front’s sign?
contactin-associated protein-like 2 (Caspr2)? EMG findings?
Myokymic discharges are seen in Isaac syndrome.
Based on the clinical scenario as well as the results of blood work, the patient likely has a peripheral nerve hyperexcitability syndrome (ie, Isaac syndrome). Isaac syndrome is an autoimmune peripheral nerve hyperexcitability syndrome that is typically due to a voltage-gated potassium channelopathy. The limbs are most often affected, and there is usually myokymia (continuous muscle twitching described as a bag-of-worms). Additionally, patients have carpopedal spasms, increased sweating, and myotonia.
EMG–nerve conduction studies (NCS) typically show fasciculations, myokymic discharges, neuromyotonia, fibrillations, and cramp discharges, most commonly in the distal extremities. Treatment is symptomatic, as well as with intravenously administered immunoglobulin or plasma exchange.
nemaline myopathy histology findings?
NEMALINE RODS
trypanasomi causes what neuropathy?
Several years later, patients present with evidence of autonomic neuropathy, predominantly as a result of vagal denervation and depopulation of myenteric plexus neurons. Advanced cases can have dramatic complications resulting from mega-esophagus, mega-colon, cardiomyopathy, arrhythmias, and frank heart failure.
buprenorphine acts on with receptors?
mu and kapp
Methadone is a long-acting μ-opioid receptor agonist that may be used in the treatment of addiction and withdrawal.
Buprenorphine, a partial μ-opioid receptor agonist and kappa-opioid antagonist may also be effective in the treatment of opioid withdrawal.
Clonidine may also be used, which blocks the release of norepinephrine and is an alpha-2 agonist, suppressing autonomic symptoms of withdrawal. Lofexidine, another alpha-2 agonist, is FDA-approved for the treatment of opioid withdrawal.
lack of empathy lesion in brain?
HD results in repeats and increased expression of?
glutamate increase and loss of GABA neurons in nigrostriate lead to increase movement
smoking increases risk of MS?
true
also increases risk of neutralizing Ab w/ nataluzimab
most common area for aneurysm leading to third nerve plays affecting pupil size?
prom and ica
square wave jerks?
no fast or slow phase
if >9 min pathological
Featured snippet from the web
Square wave jerks are involuntary, horizontal, saccadic intrusions that interrupt fixation. Each square wave jerk consists of an initial saccade that moves the fovea away from the intended position of fixation, followed by a second saccade in the opposite direction, which refoveates the fixation position.
tx of SMA? What Chr?
KRIT-1 associated with? PTEN? ATM?
Lowe syndrome ?
Lowe syndrome is characterized by involvement of the eyes, CNS, and kidneys. It is an X-linked disorder due to a mutation on the OCRL1 gene.
Beck depression inventory goal?
The Beck Depression Inventory (BDI) is a self-report rating inventory that measures attitudes and symptoms of depression. It was originally developed to provide a quantitative assessment of the intensity of depression. Because it is designed to reflect the depth of the depression, it can monitor changes over time and provide an objective measure for judging improvement and the effectiveness or otherwise of treatment methods. The BDI is composed of 21 multiple choice questions that ultimately provide a score at the end that aids in measuring the severity of depression.
innervated adductor polices and adductor digit mini?
deep ulnar
sensitivity of SFEMG for ocular MG? general
97, 99
DBS for tics placed where?
Globus pallidus interna
Benign infantile seizures start and end when?
6 months to 2 years
can be assocuited with dyskinesia later in life
Benign familial infantile seizures are a benign, limited, autosomal-dominant epilepsy syndrome associated with SCN2A and SCN8A mutations.
those who later develop paroxysmal kinesigenic dyskinesia in adolescence or adulthood, most have mutations in the proline-rich transmembrane protein 2 (PRRT2) gene on chromosome 16p.
delayed sleep wake phase fall asleep when?
Patients with delayed sleep-wake phase disorder accounts for ~10% of insomnia, and there is a strong genetic component, with about 40% of cases having a family member also affected.
eeg findings in CJD?
freq of wicket spikes?
periodic sharp wave complexes
Six- to 11-Hz sharply contoured waves are also known as Wicket spikes. These usually last for a few seconds and are found in adults during drowsiness and light sleep. They are more common in the bilateral anterior and midtemporal regions. Wicket spikes are a benign variant and not typically found in patients with CJD.
LMN or UMN: progression bulbar palsy vs pseudo bulbar palsy
Progressive is LMN 0- involved neurons from brainstem nuclei
Pseudobulbar palsy refers to cranial nerve dysfunction caused by lesions of the corticobulbar pathways to the bulbar nuclei, resulting in an upper motor neuron pattern of weakness.
spina bifida due to lack of closure of post/caudal neuropore at which days gestation?
26-28 (4 weeks)
pallid breath holding ells last how long?
Pallid breath-holding spells are less common and can be mistaken for a seizure. The child stops breathing and becomes pale, diaphoretic, and limp; if the episode lasts more than a few seconds, this is followed by generalized increased tone of the trunk and extremities, often with incontinence and occasionally low-amplitude clonus. The entire episode lasts less than 1 minute, but the child is confused and/or sleepy for several minutes afterward.
Foam cells are noted in the image to the left, which mainly consist of monocytes with multiple lipid inclusion. In the image noted on the right, a plaque with fatty deposits is noted (atheroma), and a necrotic core is also seen. These are both classic histologic findings of atherosclerosis. In chronic plaques, one might notice new vessels around the plaque that are fragile and may rupture. This may cause an intraplaque hemorrhage and vessel occlusion.
Sup salivstory nucleus innervates which glands? nerve?
Inferior innervates which gland? nerve?
The salivatory nuclei include the superior salivatory nucleus and the inferior salivatory nucleus that provide parasympathetic innervation to the salivary glands. They are located in the pontine tegmentum in the brainstem. The superior salivatory nucleus innervates the submandibular gland and the sublingual gland. It is part of the facial nerve. The inferior salivatory nucleus innervates the parotid gland by way of the otic ganglion. It forms the parasympathetic component of the glossopharyngeal nerve.
where do sympathetic nerves travel from brain to SC? what level?
First-order sympathetic neurons arise in the posterolateral hypothalamus and travel through the lateral brainstem to synapse with second-order neurons at the ciliospinal center of Budge at the level of C8-T2 in the lateral spinal cord. These second-order neurons exit the spinal cord, traverse the pulmonary apex, travel through the stellate ganglion at level of the C6-C7 vertebrae, ascend the carotid sheath, and synapse with the third-order neurons in the superior cervical ganglia at the level of the carotid bifurcation. Third-order neurons that innervate sweat glands in the face will travel along the facial artery, a branch of the external carotid artery. Third-order neurons innervating the eye travel along the internal carotid artery to the cavernous sinus. In the cavernous sinus, third-order neurons join the abducens nerve briefly before traveling to the ophthalmic division of the trigeminal nerve to enter the orbit and innervate the iris dilator muscle controlling pupillary dilation as well as the Muller muscle which aids in eyelid elevation.
where do sympathetic nerves travel from brain to SC? what level?
First-order sympathetic neurons arise in the posterolateral hypothalamus and travel through the lateral brainstem to synapse with second-order neurons at the ciliospinal center of Budge at the level of C8-T2 in the lateral spinal cord. These second-order neurons exit the spinal cord, traverse the pulmonary apex, travel through the stellate ganglion at level of the C6-C7 vertebrae, ascend the carotid sheath, and synapse with the third-order neurons in the superior cervical ganglia at the level of the carotid bifurcation. Third-order neurons that innervate sweat glands in the face will travel along the facial artery, a branch of the external carotid artery. Third-order neurons innervating the eye travel along the internal carotid artery to the cavernous sinus. In the cavernous sinus, third-order neurons join the abducens nerve briefly before traveling to the ophthalmic division of the trigeminal nerve to enter the orbit and innervate the iris dilator muscle controlling pupillary dilation as well as the Muller muscle which aids in eyelid elevation.
most common periodic paralysis?
HYPOkalemic
fibrillation potentials?
Fibrillation potentials are characterized by spontaneous rhythmic membrane depolarizations due to instability of the muscle membrane. They are typically seen in myopathies and neuropathies and are an electrophysiologic marker of active denervation. On EMG, these are noted to be sharp spikes lasting 1–5 ms.
pick bodies - intracytoplasmic neuronal inclusion
Lewy body, intranuclear
hiring bodies?
Hirano bodies are oval-to-oblong eosinophilic inclusions seen in the hippocampus in Alzheimer disease (and, to a lesser extent, in some normal older individuals).
arterial supply of anterior IC?
recurrent branch of heubner off ACA
nodose ganglion?`
motor weakness c6 radiculopathy?
auditory pathways?
bunion bodies?
Bunina bodies are commonly found in amyotrophic lateral sclerosis. Bunina bodies are formed from abnormal aggregates of TDP-43 and are found in the motor cortex, brainstem, and spinal cord. They are not depicted in the exhibit.
lafora body dz?
Lafora disease is an inherited, severe form of progressive myoclonus epilepsy. The condition most commonly begins with epileptic seizures in late childhood or adolescence. Other signs and symptoms include difficulty walking, muscle spasms (myoclonus) and dementia.
Lafora body disease, which is a fatal autosomal-recessive condition. It leads to intracytoplasmic inclusions, which are typically found in the heart, liver, muscle, and skin. A Lafora body is an aggregate of polyglucosans.
benign variants on eeg
prosapgnosia due to stroke where?
fusiform gyrus on right, due to PCA
Dysembryoplastic neuroepithelial tumors (DNETs) are benign slow-growing glioneuronal neoplasms that are bubbly in appearance, as seen on this FLAIR brain MRI. The treatment of choice is excision, especially given medication resistance and low recurrence after excision.
DNETs most commonly occur in children and young adults, with a slight male predominance. They arise from either the cortical or deep gray matter, usually in the temporal lobe, and in 80% of cases are associated with cortical dysplasia. DNETs can present with medically intractable seizures and are the second most common tumors found in surgical resection cases for intractable epileptic seizures. Ninety percent of the cases of DNETs present with first seizure before the age of 20.
survival motor neuron (SMA), age of onset if 1, 2, 3, 4, 5, copies?
type/age/resp at birth/life exp/# SMA2
0 Prenatal Yes No < 6 months 1
1 < 6 months No No < 2 years 2
2 6-18 months No No 10-40 years 3
3 > 18 months No Assisted Adult 3-4
4 > 5 years No Yes Adult > 4
Grading syndrome?
Gradenigo Syndrome (GS) is classically described as a clinical triad of otitis media, facial pain and abducens palsy that is most commonly developed from infection in the petrous temporal bone (i.e., petrous apicitis) [1]. The full triad of GS however may not always be present especially in the post-antibiotic era .
lateral femoral cutaneous nerve roots?
Answer A: L1 give rise to the ilioinguinal, iliohypogastric, and genitofemoral nerves. These nerves supply small patches of sensation just lateral to the groin and some sensation to the groin itself. Nerve impingement at these locations is rare.
Answer B: L1-L2 gives rise to the ilioinguinal, iliohypogastric, genitofemoral, and less commonly, the lateral femoral cutaneous nerves.
Answer D: L3-L4 supplies a large portion of the femoral nerve (L2 also contributes). This ultimately branches to supply the iliacus and psoas muscle and to muscles for knee extension. Isolated femoral neuropathies commonly result from retroperitoneal hematoma. The lateral femor cutaneous nerve less commonly comes from L3-4.
Answer E: The S1-S2 nerve supplies the piriformis, and clinically the patient would have some associated weakness and gait impairment.
pt with cervical SC injury develops achy, diaphoresis and HTN, what to do?
accessory perineal N can innervate?
The extensor digitorum brevis (EDB) is normally innervated by the deep peroneal nerve. In normal conditions, one should get a fairly similar compound muscle action potential (CMAP) regardless of the stimulation site: ankle (deep peroneal), below the fibular head (common peroneal), or above the fibular head (common peroneal). In some individuals, a proportion of the axons will travel along the superficial peroneal nerve and branch off as an accessory deep peroneal nerve to innervate the EDB (see image below). The anomalous innervation, in this case, can be detected by stimulating the accessory nerve behind the lateral malleolus.
Noonan syndrome?
sx of pudendal N injury?
motor from S2-4, sensory DRG
The patient is experiencing pudendal neuropathy and was treated with a pudendal nerve block. The pudendal nerve supplies sensation to the perineal area and voluntary motor supply to the external urethral and anal sphincters (but not the internal urethral sphincter).
Pudendal neuropathy can occur due to physical nerve trauma as can occur during childbirth, pelvic surgery, and hip surgery. There can be infectious and immune causes as well.
Symptoms of pudendal neuropathy include burning pain in the distribution of the pudendal nerve (or its branches). Other symptoms include urinary frequency, incontinence, dyspareunia, and hyperalgesia of the affected area. Pain is worse when sitting and improved with lying down or standing. There can be a sensation of a foreign body in the vagina or rectum.
Ornithine transcarbamoylase deficiency inheritance?
The results of MRI can initially be normal. When they are abnormal, they will most commonly show symmetrical involvement of the paroccipital white matter or splenium of the corpus (Image A). This is a specific finding that appears in about 80% of boys with this disorder. Less common is involvement on the genu of the corpus callosum (Image C), and prominent rim contrast enhancement (Image D).
X-ALD is a peroxisomal disorder caused by a mutation in the ABCD1 gene on the X chromosome. This codes for an adenosine triphosphate-binding transporter on the peroxisomal membrane and is also called adrenoleukodystrophy protein. Defects in this transporter will prevent the transport of very long-chain fatty acids (VLCFAs) into peroxisomes. Consequently, there will be an increase in VLCFAs because they cannot participate in beta-oxidation and breakdown. A VLCFA panel is the initial screening test, and, if the results are elevated, the diagnosis is confirmed with genetic testing. Additional efforts have been made to include X-ALD in the newborn screen.
quantitate sudomotor axon reflex test, what is it and what does it test?
thermoregulatory sweat test?
The latter 3 tests are described as followed:
The Valsalva ratio is the maximum heart rate induced by Valsalva blood pressure reduction divided by the minimum heart rate that results from maneuver-induced blood pressure overshoot.
In the quantitive sudomotor axon reflex test, acetylcholine is administered via iontophoresis into the skin to stimulate sweat production. The latency and volume of sweat are recorded.
In thermoregulatory sweat tests, patients are in a regulated, hot, humid room with a sweat indicator powder applied to much of the body. Digital photographs are collected and report a percentage of the anterior body surface anhidrosis.
tx for complex regional pain syndrome? dx criteria?
clonidine, prazosin, ketamine, calcitonin
AIS grade A-D? which is worst?
with spinal shock, when reflexes start to return? what days?
1-3, sensory reflexes come back first before monosynaptic/dtr’s
splitter Martin scale?
Conservative medical management is recommended for Spetzler-Martin grade 4-5 lesions, which are considered to be a surgical high risk.
Meyers loop inf or superior?
inferior
pathology in brain w/ ALS?
`due to issue with zinc/copper superoxide dysmutase
Pathologic examination of motor neurons in amyotrophic lateral sclerosis (ALS) demonstrate astrogliosis and neuronophagia in the motor cortex.
intracystoplasmic bunion body in anterior horn cells
kufor-rekab syndrome
Kufor-Rekab syndrome (KRS), a rare, autosomal-recessive subtype of JPD caused by a genetic mutation of ATP13A2, presents in patients younger than age 21 years.
early onset PD <60
young onset 21-40
juvenile <21
Aromatic acid decarboxylase deficiency?
The diagnosis of aromatic acid decarboxylase (AADC) deficiency is often delayed. It it often misdiagnosed as cerebral palsy, myasthenia gravis, or mitochondrial disorders.
AADC is a protein that aids in the production of various neurotransmitters, including dopamine, epinephrine, norepinephrine, and serotonin. Signs and symptoms of the rare autosomal-recessive disorder of AADC deficiency generally appear in the first year of life; these include developmental delay, tone abnormalities (ie, axial hypotonia and appendicular hypertonia), athetosis, lethargy, irritability, and poor feeding. Patients may also be susceptible to oculogyric crises and autonomic crises. Symptoms tend to worsen late in the day.
medical errors, latent vs active?
latent is inherent error in system that is destined to cause issue
active is a direct action w/ immediate harm
where CN 3 exit in midbrain?
