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+Neurology UWORLD ROUND 1*

Terms in this set (110)

stoke with has a sudden onset of contralateral sensory loss involving all sensory modalities (pure sensory stroke)

weeks to months later --> patients can develop what ???--> characterized by severe paroxysmal burning pain over the affected area that is exacerbated by light touch (allodynia)

ddx?
Lacunar stroke of posterolateral thalamus

develop thalamic pain syndrome
presents with headache and systemic symptoms (fatigue, fever) in patients age >50 yrs

potential complications include anterior ischemic optic neuropathy, which can lead to blindness

threatened (vision change) or confirmed vision loss --> needs what treatment??
treatment high dose intravenous glucocorticoids

Giant cell arteritis
characterized by sings and symptoms of upper motor neuron dysfunction distal to the site of compression

these include weakness, hyperreflexia, extensor plantar response

it is considered a medical emergency requiring prompt diagnosis by what?

ddx?
Spinal MRI

Spinal Cord compression
tick born paralysis is characterized by rapidly progressive ascending paralysis (which may be asymmetrical), absence of fever and sensory abnormalities, and normal CSF examination

ticks must feed for 4-7 days and are typically found on patients after meticulous searching

removal of the tick results in spontaneous improvement in most patients
...
patients with large or embolic ischemic strokes and those treated with thrombolytics are at high risk for hemorrhagic transformation

this condition usually occurs within 48 hrs of the stroke and often manifests with deteriorating mental status

diagnosis requires what?
emergent noncontrast CT scan of the head
patient develops acute bladder paralysis, lower extremity paresis, crude touch/pain sensation loss and diminished reflexes (initially)

vibrioception and deep touch sensation are generally preserved because these are carried in the posterior columns and receive some blood flow from the intact posterior spinal arteries

descending (type B) aortic dissections can be associated with interruption of blood flow to which artery?
Anterior spinal cord ischemia (particularly in the lower thoracic levels)
Hemorrhage and neuro findings
a distal, symmetric polyneuropathy that can cause a "stocking and glove" pattern of sensory loss, impaired proprioception and vibratory sense, and diminished ankle reflexes; motor findings may be a late manifestation in severe disease; ddx?

hyperactive reflexes and an up going plantar reflex are inconsistent with diabetic neuropathy and are suggestive of what?
diabetic neuropathy

upper motor neuron process
patients with dementia have an increased risk of developing agitated delirium in the hospital

what are the most common causes of delirium in a hospitalized patient?
what is the treatment?
what is avoided?
toxic-metabolic and infectious etiologies

treat with typical and atypical antipsychotics for acute agitation in elderly

avoid benzodiazepines
myasthenia gravis is caused by ?
most patients with acetylcholine receptor antibodies have what abnormalities evident on the anterior mediastinal mass on chest imaging?
autoantibody-mediated degradation of acetylcholine receptors at the neuromuscular junction, which often leads to fatigable ocular and bulbar muscle weakness

Thymoma
characterized by bilateral motor function loss at and below the level of the injury with diminished pain and temperature sensation bilaterally that begins 1-2 levels below the cord injury, whereas proprioception, vibratory sensation, and light touch are unaffected

ddx?
anterior cord syndrome usually occurs when there is injury to the anterior spinal artery from trauma
disorder that can present with headaches,k muscle spasms, jaw fatigue, and facial pain

physical exam can reveal tenderness of muscles of mastication, as well as pain, crepitus, and/or audible clicks form the joints

ddx?
Temporomandibular joint disorder
a potential complication of both mechanical and bioprosthetic valves. Patients can have a new murmur, heart failure due to valvular obstruction(stenosis) or regurgitation, or a thromboembolic event (transient ischemic attack)

ddx?
next best step?
Prosthetic valve thrombosis (PVT)

promptly evaluated with echocardiography (assess the degree of the thrombus, and evaluate for prothetic valve dysfunction)
recurrent forceful contraction of the eyelid muscles

bright lights trigger symptoms, whereas touching or brushing the skin around the eye may terminate the spasm

mild cases may be managed with trigger avoidance but what is needed for more significant symptoms?

ddx?
botulism toxin injection may be needed for more significant symptoms

belpharospasm (form of focal dystonia)
a common electrolyte abnormality that causes weakness, fatigue, and muscle cramps

when severe it can lead to paralysis and arrhythmia

ecg: u waves, flat and broad T waves, and premature ventricular beats

ddx?
hypokalemia
Thrombolytic therapy (IV alteplase) improves neurologic outcomes in patients with acute ischemic stroke when given within how long?

before thrombolytics what should be performed to rule out hemorrhagic stoke?
4.5 hours of symptoms onset

noncontrast CT and screed for other contraindications to therapy
how do we treat foodborne botulism?
equine serum heptavalent botulinum antitoxin
rapidly progressive dementia, myoclonus, mood changes, and sleep disturbances

no effective disease-modifying treatment

rapidly decline until die, which occurs less than 12 mo post diagnosis

ddx?
Creutzfeldt-Jakob disease
autosomal dominant disorder caused by expansion of the cytosine-thymine-guanine trinucleotides

typically presents in late adolescence or early adulthood with facial and distal muscle weakness and myotonia (delayed muscle relaxation after contraction); cataracts, testicular atrophy, and sleep distrubances are also common

ddx?
Myotonic dystrophy
Wernicke encephalopathy
a group of immune-mediated polyneuropathies that are caused by molecular mimicry

characterized by ophthalmopledia, ataxia, and areflexia; strengthn often preserved

highly associated with anti-GQ1b antibody

ddx?
Miller Fisher Synfrome (MFS) a variant of Guillian-Barre syndrome
Interpretation of cognitive screening tests should take what into account?
educational level (individuals with high educational levels may score in the unimpaired range despite experiencing clinically significant cognitive decline)
REM sleep behavior disorder is characterized by dream enactment during REM sleep atonia. Most patients with idiopathic RBD eventually develop a disorder of alpha-synuclein neurodegeneration, most commonly in what disease?
Parkinson disease
management options for acute pain (including opioids) will be similar for all patients regardless of substance use history, although those with a history of opioid use disorder who are given opioid analgesics may need close follow-up care to avoid relapse

what can we give?
IV morphine
Restless leg syndrome is a neurologic condition that can interfere with the quality and duration of sleep

characterized by urge to move the legs, with discomfort that is temporarily relieved by movement

how do we treat?
dopamine agonists (pramipexole) are often effective
patients with frequent attacks or disabling symptoms that do not respond to abortive treatment of migraines should be considered for prophylactic treatment including what?
topiramate, beta blockers, or amitriptyline
phenytoin is highly protein-bound and metabolized hepatically by the cytochrome p450 system --> medications that inhibit the CYP450 (TMP-SMX, fluconazole) or that displace phenytoin from plasma proteins (valproic acid) increase the risk of drug toxicity

common symptoms of acute pheytoin tox include?
- horizontal nystagmus
- ataxia
- nausea/vomiting
- significant tox results in AMS, coma, and death
common with advanced aids and generally manifests with signs of elevated intracranial pressure, progressive headache, nausea/vomiting, and confusion

in minority of patients, elevated ICP compresses the 6th cranial nerve and results in diplopia and lateral gaze palsy.

the diagnosis is generally made using what?\
ddx?
lumbar puncture with CSF analysis and india ink stain or capsular polysaccharide antigen testing

Cryptococcal meningoenchephalitis
unilateral headache , neck pain, and partial horner syndrome following a fall and likely also had a what?

presentation suggestst what which is the reason for common cause of stroke in young adults ?

what is a common complication?

what is the cause of the partial horner syndrome?

diagnose with?

management?
transient ischemic attack (TIA)

Internal carotid artery dissection

cerebral ischemia

distension of the sympathetic fibers that travel along the internal carotid artery --> lead to the partial horner syndrome (ptosis and miosis (anhidrosis which is the 3rd characteristic component of horner syndrome does not occur with partial horner syndrome as the sympathetic fivers responsible for the facial diaphoresis travel along the external carotid artery)

CT or MR angiography

Management of stroke due to dissection is similar to other ischemic strokes and includes thrombolysis in approperiate patients, and mechanical thrombectomy for large vessel occulsions (MCA) --> although there is a risk of extension of the hematoma, thrombolysis is generally considered to have similar risks as non-dissection-related ischemic strokes
- further management of both ischemic stroke and tia inclides antithrombotic therapy (aspirin, anticoagulation)
criteria for thrombolytics in stroke
dementia with executive dysfunction (difficulty planning meetings) and neurologic findings ( asymmentric reflexes, urinary frequency, and gait abnormalities) is most consistent with the subtype known as which dementia?

what is the difference between large artery infarction vs small arterial distribution?
vascular dementia

large artery infarction --> cortical type VaD --> neuro sings reflect the cortical region involved --> follows a step wise worsening course

small arterial distribution --> subcortical type VaD --> focal motor deficits, abnormal gait, urinary symptoms and psychiatric symptoms --> gradual declining course
Cluster headaches
presenting with symmetric, descending motor paresis that can progress to involve the diaphragm, causing hypoventilation and respiratory acidosis

ddx?

what is impaired?
food born botulism

impairied neuromuscular transmission
symptoms including headache (worse at night), nausea/vomiting, and mental status changes.

papilledema and focal neurologic deficits may be seen on examination

cushing reflex ( htn, bradycardia, resp depression) is worrisome finding suggesting brain stem compression

ddx?
Intracranial hypertension
a patient with pronator drift which is relatively sensitive and specific sign for what?
upper motor neuron or pyramidal tract disease affecting the upper extremities.
Acute unilateral motor weakness without sensory deficits or higher cortical dysfunction (pure motor hemiparesis) is suggestive of a what ?

what is this type of infarct most commonly associated with?
lacunar stroke affecting the posterior limb of the internal capsule.

lacunar infarcts are most commonly associated with chronic HTN--> which leads to arteriolar sclerosis and occlusion of deep penetrating branches of the major cerebral arteries
Clinical features of sellar masses
Clinical features of metastatic brain cancer
Distal symmetric polyneuropathy
Initial work up for suspected cognitive impairment
Montreal cognitive assessment ( score <26/30)
Clinical features of corneal abrasions
Parkinson disease should be suspected in patients with a resting tremor of 5-7 hz that is asymmetric and associated with rigidity

tremor is often the presenting symptom of PD

what is the treatment?
Trihexyphenidyl is an anticholinergic medication sometimes used in the treatment of PD --> generally in younger patients where tremor is the primary symptom (tremor-dominant PD)
Bell palsy, a peripheral neuropathy involving the facial nerve (CN VII), presents with acute, progressive, unilateral facial weakness

patients with a classic presentation require what next?
no further diagnostic workup is needed, and many other causes of facial weakness can be eliminated through a thorough history and physical exam
Complications of infective endocarditis
Tetanus
Concussion
essential tremor is most often presents as a tremor of the hands that is supressed at rest, exacerbated by outstretched arms, and more pronouned at the end of goal-directed movement

often hereditary and can be associated with a head tremor, but is not associated with other neurologic symptoms

diagnosis how?
clinical
single cerebral infarct may lead to vascular dementia if it damages key areas involved in cognition

patients with Vascular dementia due to a strategic infarction typically experience an abrupt decline in cognitive functioning
which medications in the treatment of Parkinson disease may cause hallucinations?
Dopamine agonists and, to a lesser extent, levodopa are associated with this adverse effect
involving muscle contractions that often involve the small muscles of the. head and neck; oculogyric crisis results in a forced upward gaze deviation

typically occur as extrapyramidal symptoms of a high-potency, first generation antipsychotic (haloperidol, fluphenazine)

ddx?
management?
Acute dystonias

management with benztropine and diphenhydramine
classicaly manifests as the triad of ataxia, encephalopathy, and oculomotor dysfunction

associated with long-standing alcohol abuse; however, it may be caused by any disorder that causes chronic malnutrition ( short gut syndrome); ddx?

Wernicke encephalopathy is a neurologic complication of which vitamin deficiency?
Wernicke's encephalopathy

thiamine deficiency

treatment with IV thiamine
...
...
a monocular painless acute vision loss most commonly associated cause by an embolized atherosclerotic plaque from the ipsilateral carotid artery

problem where ?
central retinal artery occlusion
patient with myasthenia gravis with severe respiratory muscle weakness leading to resp failure

individuals often have increasing generalized and bulbar muscle weakness (difficulty coughing up sputum) prior to the onset of crisis

the condition may be precipitated by infection, surgery, or various meds (azithromycin)

ddx?
management?
after stabilization?
myasthenic crisis

management:
- first intubate for airway protection
- then acetylcholinesterase inhibitor (pyridostigmine) (used in daily management of MG are held to reduce excess airway secretions and the risk of aspiration)

once stabilized:
- treat with IV immunoglobulins or plasmapheresis (preferred) in addition to corticosteroids
- other immunomodulatory therapy (mycophenolate mofetil or azathioprine) (especially if corticosteroids ineffective)
Exertional heat stroke
what is the first line medical treatment for idiopathic intracranial htn ?
Acetazolamide =/- furosemide
characterized by acute-onset, fluctuating consciousness, most frequently seen in elderly hospitalized patients.

resolves with treatment

ddx?
ddx increases risk of what?
Delirium

cognitive dysfunction can be persistent and a single episode of delirium increases the risk of further cognitive decline
what kind of tremor are typically a fine, fast, symmetric action tremor of the hands that increases with sympathetic activity (stress, anxiety)

certain medications (SSRI, B agonist) can cause enhanced physiologic tremor, which generally improves with dose reduction or medication cessation

what is the tremor?
enhanced physiologic tremor
when should a patient be referred to carotid endarterectomy to reduce future strokes?
symptomatic patients (TIA, or ischemic stroke in the distribution of the addected vessel) with high-grade carotid stenosis ( 70-99%)
nonfatigable nystagmus which cannot be suppressed by visual fixation

head imaging should be performed to evaluate for cerebellar stroke or hemorrhage in patients who have persistent, new-onset central vertigo and vascular risk factors, neurologic signs/symptoms, or accompanying headache

ddx?
central vertigo
Neurological deficits related to an acute demyelinating plaques of multiple sclerosis usually lasts for days to weeks whereas transient ischemic attack symptoms usuallt last for <24 hrs

what is the initial treatment of TIA?
modifying risk factors, starting aspirin and statin, and improving blood pressure control
what is used for the treatment of acute ischemic stroke that causes a significant neurological deficit (hemiparesis) and presents within 4.5 hrs from onset of symptoms ?
TPA (tissue plasminogen activator)
what is the treatment for the different time phrames of herpes zoster pain ?
usually characterized by the acute onset of monocular vision loss with central scotoma, afferent pupillary defect, changes in color perception, and pain with eye movement

strong association between ddx and multiple sclerosis

ddx?
treatment?
Optic neuritis

IV corticosteroids
Huntington disease in what part of the brain?
caudate nucleus
contralateral hemiballismus is injury to what part of brain ?
subthalamic nucleus
may be seen in Parkinson disease, dementia with lewy bodies, and multiple system atrophy

what part of brain?
substantia nigra
involved in processing of reward, among other roles, and recieves significant dopaminergic inputs

may have atrophy in Parkinson disease

what part of brain?
nucleus accumbens
primary output nucleus of the basal ganglia

what part of brain?
globus pallidus
patient with headache associated with nausea and vomiting and/or focal neurologic deficits

symptoms often worse at night and with body positioning that increases the intracranial pressure

MRI is usually diagnostic

ddx?
intracranial mass
patient with vascular disease risk factors and often present with unilateral weakness

ddx?
lacunar infarctions
presents with bulbar symptoms (dysphagia, dysarthria) with examination revealing both upper motor neuron signs (pathologic jaw jerk reflex) and lower motor neuron signs (tongue fascuculations and atrophy)

ddx?
ALS
brain lesions and clinical presentation
brown-sequard syndrome
Guillian barre syndrome
long term (>5 yrs) use of what therapy can cause vitamin B12 deficiency due to alterations in calcium hemostasis leading to impaired absorption of vit B12 in the terminal ileum
?
Metformin
Huntington disease is an autosomal dominant disease of CAG trinucleotide repeat expansion characterized by psychiatric symptoms, cognitive impairment and chorea

it is associated with preferential degeneration of what kind of neurons in the caudate nucleus and putamen?
GABA-producing neurons
what causes fetal hydantoin syndrome ( orofacial clefts, microcephaly, nail/digit hypoplasia, cardiac defects, dysmorphic facial features)?
phenytoin
associated with alzheimer dementia and occurs due to the beta amyloid deposition in the walls of small to medium cerebral arteries that leads to increased fragility of the vessels

most common manifestation is a spontaneous, lobar intracranial hemorrhage

ddx?
cerebral amyloid angiopathy
...
patient experienced a syncopal event with a trigger (prolonged standing), a prodrome (nausea, feeling of warmth), and a rapid reveovery (within 1-2 min)

common triggers include pain, anxiety, and strong emotion

what is this type of syncope?

when vasovagal syncope is repeatedly triggered by certain activity (coughing, micturition) it is referred to as??
Vasovagal syncope

situational syncope
reflex syncope
vagal and situational syncope ( a type of reflex syncope)
breif (<1 min) episodes of vertigo trigged by changes in head position (looking up at high shelves, bending down )

shorter episodes, fatiguable nystagmus inhibited by gaze fixation, preserved ambulation, and no other central nervous symptoms ( headache, diplopia, dysarthria) --> Peripheral vertigo

ddx?
most common cause?
what is the confirmatory test?
Benign paroxysmal positional vertigo (BPPV)

crystalline debris (canaliths) in the semicircular canals that disrupt the normal flow of fluid in the vestibular system --> leads to contradictory signaling from the corresponding canals in each side--> which interpreted as a spinning sensation (vertigo)

Dix-Hallpike maneuver ( best known provocative head positioning maneuver and assesses the posterior semicircular canals)
occupational exposure in a battery manufacturing palnt

clinical presentation of:
- fatigue
- neuropsychiatric manifestions ( short-term memory loss, sensorimotor neuropathy, headaches, ataxia)
- GI manifestations (abdominal pain, constipation)
- HTN and possible nephrotoxicity (elevated creatinine)

microcytic anemia with basophilic stippling and impaired purine metabolism can result in hyperuricemia

ddx?
Lead poisoning in adults
Lead poisoning
patient presents with hepatic, neurologic, and psychiatric symptoms

diagnosis is supported by low serum ceruloplasmin, increased urinary copper, and Kayser-Fleischer rings on slit lamp exam

ddx?
Wilson disease
initial diagnostic work- up of a first time seizure in an adult should include?
- basic blood tests ( serum electrolytes, glucose, calcium, magnesium, complete blood count, renal and liver function tests)
- a toxicology screen to evaluate for metabolic and toxic causes

- UNPROVOKED SEIZURE --> requires further evaluation with neuroimaging and EEG
...
presents with low back pain that radiates down the leg associated with sensory loss over the lateral thigh, calf, and dorsal root and weakness of great toe extension and foot dorsiflexion, inversion, and eversion

ddx?
L5 radiculopathy
acute exacerbations of multiple sclerosis should be treated with ?
high-dose IV glucocorticoids

Plasma exchange is reserved for patients who do not respond to high-dose glucocorticoids
...
patients with ALS (asymmetric muscle strength, muscle atrophy) has manifestations of early resp insufficiency

affects both lower and upper motor neuron functions

what can we give to prolong survival and improve quality of life?
noninvasive positive-pressure ventilation
what should be suspected when trigeminal neuralgia presents bilaterally?

what is the pathophys?
multiple sclerosis

demyelination of the trigeminal nerve axon or the nerve root --> which leads to improper signaling of the nerve and paroxysms of severe pain
...
patient elderly with symmetric bilateral, sensorineural hearing loss

can hear but not understand others

ddx?
Presbycusis
generalized convulsive status epilepticus is defined as a seizure lasting >5 mins or >2 seizure events in which the patient does not completely regain consciousness.

what should be administered for seizure termination?

what should be administered to prevent seizure recurrence?
IV benzodiazepines (lorazepam, diazepam)

Nonbenzodiazepine antiepileptic (fosphenytoin, phenytoin, levetiracetam, or valproic acid)
bell palsy is a peripheral neuropathy of the facial nerve (cranial nerve 7) characterized by facial weakness that involves both the upper and lower face.

it is thought to be caused by a reactivation of what?
neuropathic viruses (herpes simplex virus)--> resulting in inflammation, edema, and degeneration of the myelin sheath
alzheimer disease initially presents with memory impairment

neuroimaging generally demonstrates what?
temporal lobe atrophy, which is most prominent in the hippocampi and surrounding medial temporal lobes (seen in later stages)
guillian barre syndrome is characterized by ascending weakness, bulbar symptoms (dysarthria) and respiratory compromise after antecedent illness such as resp or GI infection

CSF analysis shows albuminocytologic dissociation

treatment of GBS includes?
IV immunoglobulin or plasmapheresis
Lumbar spinal stenosis
amitriptyline side effects
- orthostatic hypotension
- lethargy
- anticholinergic symptoms (dry mouth, constipation, urinary retention)
presents with both upper (spasticity, hyperreflexia) and lower (fasciculations, atrophy) motor neuron lesions
als
where is the multiple sclerotic plaque that results in paraplegia, bladder incontinence, and absent sensation below the level of the lesion?
upper thoracic spinal cord
...
...
idiopathic intracranial HTN (young, obese women with headache, vision changes, papilledema, and abducens nerve (CNVI) palsy

diagnosis is confirmed by?
lumbar puncture showing elevated opening pressure and normal cell counts
...
what is recommended for solitary brain metastasis in patients with good performance status and stable extracranial disease?
surgical resection

**in multiple brain metastases, whole brain radiation therapy is typically used**
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