Neurology Flashcards
Carries a risk of permanent visual loss in 20-50% if untreated. Associated s/sx include polymyalgia rheumatica, headache (in 40% to 90%), weight loss (16% to 76%), scalp tenderness (28% to 91%), anorexia (14% to 69%), fever, leg claudication (2% to 43%), and jaw claudication (4% to 67%)
Giant Cell Arteritis
Headaches associated with ___ may be unilateral or bilateral and are often temporal; Seldom throbbing and more often dull or boring with superimposed episodic icepick-like, lancinating pain. The headaches are often worse at night and often aggravated by exposure to cold. Scalp tenderness is often present
Giant Cell Arteritis
Hemisection of the spinal cord, most often produced by an extra medullary lesion, results in ___ which consists of s/sx: loss of pain and temperature sensation contralateral to the hemisection, ipsilateral loss of proprioceptive, ipsilateral spastic weakness, and segmental lower motor neuron and sensory signs
Brown-Sequard syndrome
____ most likely presents with variable quadriparesis with the upper extremities more severely involved than the lower extremities, and with some degree of sensory sparing
Central Cord Syndrome
Compression of the third nerve by ___ characteristically causes dilatation and unresponsiveness of the pupil. Oculomotor paresis may be incomplete with at least one element of nerve dysfunction (i.e., ptosis, mydriasis, or extraocular muscle weakness) absent
Aneurysm
___ is characterized by macular degeneration with visual hallucinations
Charles-Bonnet Syndrome
Indicated for the tx of Crohn’s and MS; There is an increased risk of the development of progressive multifocal leukoencephalopathy (due to JC virus infection) with its use
Natalizumab
A 20 y/o man p/w acute onset of HA, and pain surrounding his left eye; also w/ nasal stuffiness, rhinorrhea, and redness of the sclera of the left eye. He has about 1-2 headaches per day. In addition to sumatriptan and steroids, what is effective in acute tx?
High-flow oxygen treatment (Cluster headache)
The 3 classes of medications that are most effective for migraine prevention are ___
antiepileptics, antidepressants, and antihypertensives
___ result in an inability to depress either eye fully in adduction; a positive Bielschowsky test on tilt to either shoulder
Bilateral fourth nerve palsies
___ results in weakness and atrophy of the quadriceps and sensory loss of the anteromedial thigh
Femoral neuropathy
___ typically involves variable motor loss in the lower extremities with loss of bowel and bladder function
Conus medullaris syndrome
Patients with ___ develop unilateral or bilateral asymmetric buttock, thigh, or leg pain, and occasionally neurologic signs (e.g., numbness, loss of reflexes, or paresis) after exertion
Lumbar stenosis
___ p/w inability to walk, gaze palsy without hemiplegia, and absence of unilateral limb paresis
cerebellar infarct or hemorrhage
In acute ischemic stroke, aspirin, if not contraindicated, should be given within ___
first 48 hours
In acute ischemic stroke, IV rt-PA should be given within ___
first 3 hours
Drunk person, compressing the ___; p/w severe wrist drop with marked paresis of elbow and wrist extension, supination of the forearm, extension of all 5 metacarpophalangeal joints, and extension and abduction of the thumb + sensory loss over the entire extensor
radial nerve
Severe unilateral head or eye pain lasting minutes and associated with autonomic manifestations with frequent attacks per day, more likely in women
Paroxysmal hemicrania
Most characteristic visual aura of migraine is a ___ (occurring in about 64% of cases), beginning as a hazy spot from the center of a visual hemifield followed by shimmering light of different patterns expanding peripherally
Scintillating scotoma
Pain due to nociceptor dysfunction that persists after the rash subsides in a region affected by herpes zoster (HZ) virus reactivation (i.e., shingles).
Postherpetic neuralgia
A 42 y/o man initially complained of tingling of the feet and fingers. Over the next 1-2 weeks –> progressive symmetrical weakness of the legs and then the arms, both proximally and distally, accompanied by pain in the low back and legs. Reflexes were diffusely absent and toe signs are flexor. What is dx and tx?
GBS; tx/ IV immunoglobulin or plasma exchange
SOD-1 protein is abnormally deposited in ___
Amyotrophic lateral sclerosis
Degeneration of the large anterior horn cells of the spinal cord is seen in ___
Amyotrophic lateral sclerosis
The pattern of subacute, progressive proximal and symmetric limb muscle weakness, without involvement of the cranial nerves and preservation of sensation and reflexes, should raise the possibility of ___. The diagnosis is supported by an increase in the serum level of creatine kinase and EMG findings for an inflammatory myopathy
Polymyositis
Polymyositis dx is confirmed by ___; Tx is ___
Muscle biopsy; tx w/ high dose PO corticosteroids
CSF in viral meningitis is often characterized by ___
elevated protein levels, clear CSF, normal glucose, normal opening pressure
Laboratory CSF findings suggestive of bacterial meningitis are ___
high WBC count (especially neutrophils), high protein level, low glucose level.
A 75 y/o man presents with recent right fronto-temporal headaches and sudden, persistent loss of vision in his R eye. Exam shows severe visual impairment in all 4 quadrants and relative afferent pupillary defect of the right eye. Fundoscopy of the right eye shows that the optic disc is not swollen or pale, however the retina appears white and there is a cherry-red spot. What is dx?
Central retinal artery occlusion
Which one of the following antiepileptic drugs (AEDs) can cause aplastic anemia and liver failure, and is therefore a last resort medication?
Felbamate
A 75-year-old man states that when he is exposed to bright light, his right eye will go blind for several minutes. Monocular transient visual loss (TVL) lasting 5 to 60 minutes (usually up to 30 minutes) is strongly suggestive of ___
Carotid occlusive disease
Pt p/w mild weakness and decreased sensation in all extremities. The neurological examination demonstrates decreased proprioception and vibration sensation, hyperreflexia, and mild weakness, but normal tone, in all extremities. Dx?
subacute combined degeneration, which is demyelination of the dorsal and lateral spinal cord
The FDA also has approved ___ for the treatment of relapsing-remitting MS
a synthetic form of myelin basic protein, called glatiramer acetate
A 42-year-old woman presents with acute onset of headache, vertigo, nausea, and vomiting. Neurologic exam reveals left-beating nystagmus, left Horner syndrome, absent left gag reflex, left arm ataxia, and anesthesia to pinprick in the left face and right upper extremity.
vertebral artery dissection
The subacute onset of ataxia in a woman with normal MRI findings should strongly suggest a ___
paraneoplastic disorder
___ occur especially with ovarian, fallopian tube, endometrial, surface capillary, and breast carcinomas, and occasionally with lymphoma. They cause paraneoplastic cerebellar degeneration with a subacute ataxia that may be associated with downbeat nystagmus.
Anti-Purkinje cell antibodies (anti-Yo antibodies)
Most common cause of bitemporal hemianopsia
pituitary tumor
Right-side homonous hemianopsia is due to damage to ___
left optic tract
Damage to ___ leads to dilated pupil, lid ptosis, and eye stays lateral (abducted)
3rd cranial nerve
Damage to ___ leads to contralateral lower face paralysis
upper motor neurons of CN 7
Damage to ___ leads to ipsilateral upper and lower face paralysis
lower motor neurons of CN 7
___ is one of the most common etiologies of episodic vertigo and is brought on by head movements or changes in posture and often associated with nausea and nystagmus. It is due to abnormal stimulation of the semicircular canals by otoliths from the utricle. Diagnosis may be aided by provoking nystagmus and vertigo with ___
Benign paroxysmal positional vertigo (BPPV); the Dix-Hallpike maneuver
___ may also result in episodic vertigo, but is typically associated with tinnitus, sensation of ear fullness, and low-frequency hearing loss.
Meniere’s disease
Episodes of transient visual loss (TVL) lasting less than 60 seconds in an older patient is concerning for ___
amarousis fugax (embolus from cardiac source)
Paroxysmal hemicranias (a subtype of trigeminal-autonomic cephalgias), hemicrania continua, valsalva-induced headaches, and primary stabbing headache are responsive to ___
Indomethacin
___ is a degenerative neurologic disorder characterized by a combination of cognitive, behavioral, balance, and visual symptoms. Among the most common signs are difficulty in the voluntary movement of the eyes in the vertical plane and impairment in voluntary eye saccades. Both difficulties with eye movement and balance are thought to contribute to sudden falls
progressive supranuclear palsy (PSP)
___ is due to destruction of the acetylcholine receptors at neuro-muscular junctions
myasthenia gravis
Progressive multifocal leukoencephalopathy (PML) is caused by the opportunitistic and ubiquitous ___ in immunodeficient individuals (e.g., those with HIV/AIDs) and involves destruction of ___, which create myelin in the central nervous system, leading to progressive demyelination of subcortical white matter.
JC virus (Polyomavirus JC);
Which one of the following frontal lobe areas would most likely be involved with a seizure semiology of swallowing, salivation, mastication, epigastric aura, and speech arrest often associated with clonic facial movements?
Operculum
Which one of the following findings of brainstem auditory evoked potentials (BAEPs) is consistent with a peripheral hearing disorder?
Absent wave 1 with normal 3 and 5
Clinical characteristics include: patients are usually middle aged men; proximal weakness of limbs, especially in the legs, which may improve with exercise; absent or decreased tendon reflexes; cranial nerve tends to be spared (vs. myasthenia gravis), except mild ptosis; often autonomic impairment-impotence, urinary dysfunction, dry mouth, orthostasis; repetitive stimulation study reveals a decremental response at low rates of nerve stimulation and an incremental response at high rates; often associated with small cell lung cancer
Lambert-Eaton myasthenic syndrome
___ should be considered for people with tremor where essential tremor cannot be clinically differentiated from Parkinsonism.
Single-photon emission computed tomography (SPECT)
___ occurs in children and is defined by the following triad: multiple seizure types (usually including generalized tonic-clonic, atonic, and atypical absence seizures); EEG showing slow (3 Hz) spike-and-wave discharges. impaired cognitive function in most but not all cases This syndrome is associated with CNS disease or dysfunction from a variety of causes, including developmental abnormalities, perinatal hypoxia/ischemia, trauma, infection, and other acquired lesions.
Lennox-Gastaut Syndrome
___ is a disorder of copper transport that is characterized by inadequate biliary copper excretion. This inadequacy leads to accumulation of copper in liver, brain, kidneys, and corneas. Neurologic presentation includes movement disorders such as tremors and ataxia, and spastic dystonia such as masklike face, rigidity, gait disturbance, dysarthria, drooling, and dysphasia.
Wilson’s disease
A 52-year-old woman has a 3- to 4-week history of progressive, asymmetrical, mainly distal more than proximal sensory loss and weakness of the legs, with pain in the back, buttocks, and thighs. The knee reflexes are absent and the ankle reflexes are reduced, with flexor toe signs. Dx?
Inflammatory polyradiculoneuropathy
Spinal cord neoplasms frequently affect the ___, leading to areflexia and extensor plantar responses
Pyramidal tract
“Pseudobulbar or suprabulbar palsy that occur due to interruption of the ___ as they descend through the corona radiata and internal capsule, and present with deficits in cranial nerve function and, additionally, can present with ““emotional incontinence.”””
corticobulbar tracts
Corticobulbar tract carries upper motor neuron input to motor nuclei of trigeminal, facial, glossopharyngeal, vagus, accessory, and hypoglossal nerves. The motor component of trigeminal nerves supplies muscles of mastication. The facial nerve supplies the muscles of facial expression.
___: clinical characteristics of this entity: usually middle aged men; proximal weakness of limbs, especially in the legs which may improve with exercise; reduced or absent reflexes; cranial nerve tend to be spared (vs. myasthenia gravis) except mild ptosis; often autonomic impairment-impotence, urinary dysfunction, dry mouth, orthostasis; repetitive stimulation study reveals a decremental response at low rates of nerve stimulation and an incremental response at high rates; often associated with ___
Lambert Eaton Myasthenic Syndrome; associated w/ small cell lung cancer
Mesial temporal lobe epilepsy syndrome (MTLE) is the most common epilepsy syndrome associated with ___. High-resolution MRI characteristically reveals ___ that appears essential in the pathophysiology of MTLE for many patients.
Complex partial seizures; hippocampal sclerosis
___ was the first drug approved in the US for ALS since it modestly protracted ALS progression. In 2017, ___, the second-drug for ALS treatment, was approved by the US FDA.
Riluzole; Edaravone
Bilateral lesions of the ___ cause Klüver-Bucy syndrome leading to hypersexuality, hyperorality (fixation on oral exploration), and disinhibited behavior, but primitive reflexes will be absent.
amygdala
Lacunar infarct of subthalamic nucleus leads to ___
hemiballismus
___ is also very short-acting cholinesterase inhibitor that is used for diagnosing myasthenia gravis
Edrophonium
___ is an acetylcholinesterase inhibitor that does not cross the blood-brain barrier and is used largely for treating myasthenia gravis.
Pyridostigmine
__ is the test of choice to examine ___ to diagnose Huntington’s disease. The HD gene resides on the short arm of ___
PCR; trinucleotide repeats (CAG); Chromosome 4 at 4p16.3
Huntington’s disease is transmitted by ___ pattern. If one parent is an affected carrier, the likelihood of transmission to any given child is ___
autosomal dominant inheritance; 50%
The clinical hallmark of Guillain-Barre is ___. Test of choice are ___
loss of deep tendon reflexes in the extremities; EMG and nerve conduction studies
___ presents with classic tetrad of acalculia, agraphia (without alexia), R and L confusion, and finger agnosia (inability to name fingers). Lesion localizes to ___
Gerstmann’s syndrome; left angular gyrus (in left parietal lobe)
___ is when a patient gets up from the floor/chair by using hands bc of muscle weakness in the legs. It is a classive indicator of ___
Gower’s maneuver; Duchenne’s muscular dystrophy
Tx for trigeminal neuralgia (lancinating, brief sharp pain, usually unilateral)
Carbamazepine (Tegretol) or Oxycarbazepine (Trileptal)
About 25% pts with temporal arteritis also have comorbid ___
polymalgia rheumatica
Patient with ___ p/w with progressive proximal muscle weakness, generalized fatigue, and red nonpruritic rash on the face and body, esp on the knees and elbows
Dermatomyositis
Intoxicated man p/w limp right wrist and fingers (cannot lift them) and weakness when trying to extend arm from a bent to straight position. He has trouble turning his forearm over when placed palm down on a flat surface. The lesion is most like to the ___
Radial nerve due to entrapment from prolonged compression of axilla
Compression of ___ leads to weakness of flexor carpi ulnaris, intrinsic hande muscles, and 4th/5th finger deep flexor weakness
Ulnar nerve
Compresion of ___ leads to classic carpal tunnel syndrome. There is sensory loss, thenar atrophy and + percussion of nerve over wrist leading to paresthesia (=___) in 60% cases
Median Nerve; Tinel’s sign
Biceps and brachialis weakness is the hallmark of injury to ___
Musculocutaneous nerve
musculocutaneous nerve originates from the lateral cord of the brachial plexus (C5-C7) at the inferior border of pectoralis minor muscle.
___ is screening test for Sarcoidosis
Serum ACE level
32 y/o man with HIV p/w fever (101 F), headache, stiff neck, photophobia, and lethargy. CD4 count is 0. Dx is __, most useful immediate dx test would be ___
Cryptococcal meningitis; LP for CSF analysis and India ink staining
Diagnostic test of choice for Creutzfeldt-Jakob disease (CJD)/Prion Disease:
LP with CSF assay of 14-3-3 and tau proteins’
Lesion to ___ of brain stem leads to internuclear ophthalmoplegia in multiple sclerosis, p/w abnormal horizontal ocular movements with absence/delayed adduction of the eye ipsilateral and coarse horizontal nystagmus in the abducting eye
Medial longitudinal fasciculus
___ is a sensation of an electric shock descending down the spine or the extremities upon neck flexion. It is most often suggestive of ___.
Lhermitte’s sign; Multiple sclerosis
IV Immunoglobulin therapy and plasmapharesis are treatments for what two neurological conditions?
Myasthenia gravis and Guillain-Barre Syndrome
MRI scan for patient with ___ revelas numerous subcortical white matter demyelinating lesions
Multiple sclerosis
Locked-in syndrome is the result of a ___ lesion that is clinically devastating, producing quadriplegia, mutism, and lower cranial nerve palsy
pontine
34 y/o AAF p/w 6 weeks of intermittent bifrontal headache and vague visual obscurations. Immediate dx test is ___
LP, showing elevated opening pressure; Dx: Pseudotumor cerebri
Inclusion criteria for use of r-TPA:
ischemic stroke with onset <3 hrs prior; neuro deficits measurable on NIH stroke scale; CT scan showing no intracranial hemorrhage
Exclusion criteria for use of r-TPA:
rapidly improving/minor stroke sx; seizure at onset of stroke; prior intracranial hemorrhage; pretreatment BP > 185/110; major surgery within 14 days; prior stroke or head injury w/in 3 months, recent MI
Patient p/w ipsilateral Horner syndrome, ipsilateral loss of pain and temperature sensation in face, cerebellar ataxia, weakness of vocal cords/pharynx, contralateral loss of pain and temperature sensation to the hemibody; Dx is ___ due to location___and vessel__
Lateral Medullary Syndrome; occlusion of one of vertebral arteries (infarct of lateral medulla)
___ is the most common offending infectious agent in AIDS-related retinopathy. It accounts for 30% cases of HIV-related retinopathy
Cytomegalovirus
Lesion at ___ causes a foot drop from dorsiflexor muscle weakness
L5
Leg extension is a function of the quadriceps muscles, which are innervated by nerves emanating from the ___ nerve roots
L3, L4
Hip flexion (raising knee in the air) is a function of the iliopsoas muscles, which are innervated by ___ nerve roots
L1, L2, L3
The Achilles or ankle jerk motor reflex is a function of the ___ motor nerve root
S1
Classic Brown-Sequard syndrome presents as ___
ipsilateral loss motor control, propioception, and vibration——-contralateral loss of pain and temperature
The ___ irrigates the medial frontal lobes; when affected, causes preferential leg greater than arm and face weakness contralateral to the side of the lesion
Anterior cerebral artery
___ is one of the classic lacunar stroke syndromes and would be expected from an infarct in the area of the ___
Pure motor hemiparesis; internal capsule
Lesion to ___ will produce hemiballismus. Most often results from an acute stroke
contralateral subthalamic nucleus
Ataxia is indicative of lesion to ___, which is perfused by the vertebrobasilar arterial system
cerebellum
Patient p/w transient ipsilateral monocular blindness (amaurosis fugax), contralateral body weakness or sensory loss, aphasia w/ dominant hemisphere involvement, and contralateral homonymous visual field deficits. Dx = ___
Carotid territory transient ischemic attack or stroke
___ involves parenchymal invasion of the brain by the larval stage pork tapeworm T. solium. This is caused by ingestion of undercooked pork. presentation ranges from epilepsy, focal neuro deficits, hydrocephalus, cognitive decline, meningitis, or myelopathy
CNS cysticercosis
___ is more often seen in AIDS patients with low CD4 counts. CNS lesions present as ring enhancing on both CT and MRI. Tx: pyrimethamine and sulfadiazine with folinic acid
Toxoplasmosis
___ presents with classic triad of gait ataxia, areflexia, and opthalmoplegia. Variant of Guillain-Barre syndrome
Miller-Fisher syndrome
Dermatome at the level of the umbilicus
T10
Large stroke in the (territory)___ can result in hemineglect, visual and tactile extinction, impaired speech, anosognosia, and behavioral problems such as delirium/confusion
right MCA territory
A third nerve palsy resulting from an aneurysm (originating at internal carotid artery near origin on posterior communicating artery) is usually associated with ___, as contrasted with a diabetic third nerve palsy, which usually ___
aneurysm –> dilated pupil, eyelid ptosis; diabetic –> spares pupillary function
Early infection can appear as meningitis, uni/bilateral Bell’s palsy, painful radiculoneuritis, optic neuritic, or GBS. After initial infection, about 2/3 develop rash (erythema chronicum migrans), painless expanding macular patch. Dx = ___
Lyme Disease (Borrelia burgdorferi)
Condition starts with fever, headache, muscle aches, and GI sx about 2-14 days after tick bite. There is a rash that appears initially around wrists and hands, then spreads over days to the feet and forearms. Dx = ___
Rocky Mountain spotted fever (Rickettsia rickettsii)
Primary auditory cortex localizes to the ___
superior temporal gyrus in both temporal lobes
Epidural hematomas result most often from head trauma and skull fracture that cause a tear in the ___ or one of its branches
middle meningeal artery
Subdural hematoma is believed to result from tearing of the ___ over the cortical surface or from trauma to the venous sinuses or their tributaries
bridging veins
Creutzfeldt-Jakob disease presents with ___ on EEG
periodic sharp-wave complexes
Periodic lateralizing epileptiform discharges (PLEDs) on EEG is characteristic of ___
herpes simplex encephalitis
Midline cerebellar vermian lesions cause ___
truncal ataxia
___ is maneuver to dx BPPV
Dix-Hallpike maneuver
On EEG, triphasic sharp waves are characteristic of ___
Creutzfeldt-Jakob Disease
Compression of ___ results in a painful sensory syndrome known as meralgia paresthetica, which p/w pain and sensory loss to the lateral thigh
lateral femoral cutaneous nerve
___ are common characteristics of anterior horn cell diseases such as poliomyelitis and amyotrophic lateral sclerosis
fasciculations
___ occurs 1 day - 4 weeks postpartum, p/w puerperal headache, seizures, neuro deficits and behavior/personality changes
Cerebral venous thrombosis
___ is a brief period of transient hemiparesis or hemiplegia following a seizure. Sx usually dissipate within 48 hours and treatment is expectant and supportive
Todd’s paralysis
___ is a hematoma overlying the mastoid that results from a basilar skull fracture extending into the mastoid portion of the temporal bone
Battle sign
Hypocalcemia that is chronic may result in clinical observation of ___, positive when the cheek is tapped with the examiner’s finger and the corner of the mouth involuntarily contracts
Chvostek sign
Triphasic waves on EEG are characteristic of ___
hepatic or metabolic encephalopathy
___ results from bilateral destruction of the amygdaloid bodies and the inferior temporal cortex, p/w hypersexuality, placidity, and hyperorality
Kluver-Bucy syndrome
Pt p/w sudden onset of transient loss of vision that manifests as a curtain or shade or veil usually over central visual field. Brief (about 1-5 min) then generally returns to normal. Dx __ caused by ___
Amaurosis fugax; carotid artery territory ischemia
___ affects the cerebellum and/or brain stem. Classive sx of ischemia include ataxia, nystagmus, vertigo, dysarthria, and dysphagia
Vertebrobasilar territory ischemia
___ ischemia affects the small penetrating branching arteries off of the MCA that feed the striatum. Ischemia produces lacunar infacts of the internal capsule, resulting in pure contralateral motor hemiparesis
Lenticulostriate territory
___ ischemia would be expected to cause contralateral hemiparesis of the leg preferentially
Anterior cerebral artery
___ ischemia can take different forms. If lesion is in dominant hemisphere, aphasia may result. If nondominant –> hemineglect, anosognosia, visual/tactile extinction, aprosody of speech, contralateral limb apraxia
Middle cerebral artery
Patient p/w rapid onset bilateral leg weakness with clear-cut sensory level below level of the lesion. Urinary and/or bowel incontinence are common. Pain and temperature are usually affected, but propioception and vibration sensation are spared. Often follows an infection or vaccination or is the direct result of dymyelination 2/2 MS. Dx =
Transverse myelitis
Horner’s syndrome p/w ___; lesion usually localizes to ___
ptosis, miosis, anhidrosis; internal carotid artery
Prosopagnosia, the inability to recognize faces, is associated with lesions to ___
Fusiforme gurus in the occipital/temporal lobes
Difference between paralysis of Bell’s palsy vs facial paresis of hemispheric stroke
Bell’s palsy - entire face; stroke - spares upper 1/3 of face (brow, upper eyelid)
Patient p/w dissociated suspended sensory deficit usually in a cape or shawl pattern over the arms and upper trunk. There is impairment of pain and temperature, but preserved light touch, vibration, and propioception
Syringomyelia
___ is herpetic cranial neuritis that affects CN VII and VIII. Pathogen is varicella zoster virus and p/w painful facial palsy, vertigo, ipsilateral hearing loss, and vesicles in the external auditory canal
Ramsay Hunt Syndrome
Person p/w cortical blindness and often denies that they are blind and confabulate.
Anton’s syndrome (localizes to bilateral occipital lobes)
Most common CNS cancer noted in patients with advanced AIDS
Lymphoma
Most frequent CNS opportunistic infection in AIDS patients
CNS toxoplasmosis
Patient with AIDS and Cd4 count 50 p/w rapidly progressive onset gait difficulties, spasticity, leg weakness, sphincter dysfunction, and loss of propioception to both feet and legs. Dx ___
Vacuolar myelopathy
___ is most common peripheral nerve syndrome that complicates AIDS. Patient p/w diminished ankle jerk reflexes, decreased pain/temp/vibration sensation, possible paresthesia of feet
Distal sensory polyneuropathy
___ is demyelinating d/o affecting AIDS pts with low CD4 count. Results from opportunistic infection by the JC virus. P/w hemiparesis, aphasia, sensory deficits, ataxia, and visual field deficits.
Progressive multifocal leukoencephalopathy (PML)
___ p/w headache, ipsilateral Horner’s syndrome, and contralateral hemiparesis
carotid artery dissection
Bell’s palsy is believed to be result of infection from ___
Herpes simplex virus, Lyme disease
Patient p/w 4-week hx headache, vague fever, and paresthesias. Temp is 103.5. He complains of difficulty swallowing with pharyngeal spasms for past 3 days. Usually progresses to encephalitis with possible autonomic hyperactivity, seizures, agitation, and psychosis. Dx ___
Rabies virus
Trismus, or lockjaw, is a primary sx in over 75% cases of people infected with ___. Can p/w risus sardonicus, a sustained involuntary grimace resulting from uncontrollable facial muscle spasm. Laryngospasm can lead to respiratory depression.
Clostridium tetani
Classic sx of ___ include dysphagia, dysarthria, ptosis, and diplopia. These sx rapidly progress to limb paralysis and eventually to paralysis of respiratory muscles that can lead to death if the condition is untreated
Botulism (Clostridium botulinum)
“Damage to ___ p/w proximal forearm, mild paresis of the pronator quadratus, paresis of the flexor digitorum profundus I and II and flexor pollicis longus, and a ““pinch attitude”” in attempt to make a complete circle by applying pulp of thumb to that of index finger”
Anterior interosseous nerve
Infection with ___ can be asymptomatic or p/w splenomegaly, pharyngitis, and cervical lymphadenopathy
Epstein-Barr virus (EBV)
60 y/o woman p/w progressive downward course over 6 months characterized by behavioral disinhibition, emotional lability, severe name and word-finding difficulties, hyperorality, stubbornness, inability to plan, and poor judgment. Autopsy of brain would show:
Pick’s inclusion bodies and gliosis (Pick’s disease - one of frontotemporal dementias)
The neuropatholical hallmark of idiopathic Parkinson’s disease:
Lewy bodies in parts reticulata of substantia nigra
___ are eosinophilic neuronal inclusions seen in Creutzfeldt-Jakob disease and Alzheimer’s dementia
Hirano bodies
Mesial temporal sclerosis is pathological hallmark of brain tissue in those with ___
temporal lobe epilepsy
Atrophy of the head of the caudate nucleus is found in ___, but it does not correlate with illness severity
Huntington’s disease
Pt p/w acute onset of pure right hemiparesis that affects face, arm, and leg equally. Stroke most likely localizes to ___; Dx __
Left internal capsule (Lacunar Stroke Syndrome)
Infant consumes unpasteurized honey and then p/w weak cry, lethargy, floppiness, poor suck, and constipation. Dx ___
Botulism (Clostridium botulinum)
___ is rare stroke syndrome 2/2 ischemia to bilateral parietal-occipital lobes or occiptal lobes alone. Often complication of vascular dementia. P/w ocular apraxia, optic ataxia, and deficits in visual attention
Bulint’s syndrome
Pt with ___ p/w cortical blindness. Hallmark is patient being unaware or denying blindness and confabulating. Results from lesion to bilateral occipital lobes
Anton’s syndrome (localizes to bilateral occipital lobes)
___ is inability to recognize familiar faces
Prosopagnosia
___ p/w combination of blepharospasm (involuntary eyelid blinking) and oromandibular dystonia
Meige’s syndrome
Meige syndrome is a rare neurological movement disorder characterized by involuntary and often forceful contractions of the muscles of the jaw and tongue (oromandibular dystonia) and involuntary muscle spasms and contractions of the muscles around the eyes (blepharospasm).
Inability to recognize and identify objects
Agnosia
Inability to perform a skilled, learned, purposeful motor behavior
Apraxia
Near muteness with normal reading, writing, and comprehension
Aphemia
Positive grasp reflex is usually patholigical sign in an adult. It is a normal infantile reflex present at birth and usually disappears by 6 months. Persistent or redevelopment of the reflex in adulthood would indicate damage to ___
grasp reflex past the age of infancy is associated with focal lesions in the central nervous system (CNS) or diffuse neurodegeneration,
grasp reflex is fairly specific for a lesion of the supplementary motor area on the medial surface of the contralateral frontal lobe.
___ is positive when a patient suspected of psychogenic hemiparesis does not give effort in the contralateral (unaffected) lower extremity when asked to push down on the bed with the paretic (affected) lower extremity
Hoover’s sign
___ is present when straight-leg raising in a recumbent position results in reproduction of pain or paresthesia in the sciatic distribution
Lasegue’s sign
___ is positive when a patient is asked to stand up straight with eyes closed and subsequently loses balance. This indicates deficit to either posterior columns of the spinal cord or to cerebellar pathology, or both
Romberg’s sign
Vagal nerve stimulation is FDA approved for ___
adjunctive tx of refractory partial complex epilepsy and intractable MDD
___ p/w intermittent headaches that result from increased intracranial pressue b/c of the ball-valve blockage of the passage of CSF in the ventricular system. Blockage may lead to brief intermittent drop attacks
Colloid cyst of the 3rd ventricle
Isoniazid can cause ___, leading to sensory polyneuropathy
Vitamin B6 (pyridoxine) deficiency
___ is fungus that colonizes in paranasal sinuses, can cause hypersensitivity pneumonitis. Has predilection for invading the posterior circulation and can cause vertebrobasilar strokes
Aspergillus
___ presents as dementia, dermatitis, and diarrhea
Niacin deficiency (pellagra)
Pimozide (Orap) is approved for treatment of Tourette’s. It’s MOA is ___
dopamine antagonist
___ is most common manifestation of neurosarcoidosis, occuring in up to 75% cases
Cranial neuropathies, in particular facial nerve palsy
___ typically leads to large hemispheric infarction w/ contralateral hemiparesis, conjugate eye deviation toward side of infarct, hemianesthesia, and homonymous hemianopia
Stem occlusion of MCA
What neuro deficit is more common in upper division MCA infarct?
Broca aphasia
Pure alexia (inability to read) without agraphia (inability to communicate through writing) is due to stroke in ___. Patients can write but cannot read their own writing. Speech, comprehension, and repititon are intact
posterior cerebral artery
Stroke to ___ are usually responsible for sensory deficits of cerebral origin. presents with hemianesthesia with gradual return of sensory function and pain
Thalamus
___ results from intracranial bleed that causes brief loss of consciousness, followed by initial lucid period and then further deterioration afterwards. This results from ___
Epidural hematoma; middle meningeal artery
Damage to ___ results in subdural hematoma, which is often seen in alcoholics who have a head injury
bridging veins
___ results from occlusion of posterior inferior cerebellar artery, producing sensory loss on ipsilateral face (from trigeminal involvement), contralateral loss of pain and temperature sensation, bulbar weakness (resulting in dysphagia, dysarthria) and Horner’s syndrome
Lateral Medullary Syndrome (Wallenburg Syndrome)
Person with ___ p/w fluctuating rigidity of axial musculature with superimposed spasms. 80% of patients develop this as a nonparaneoplastic phenomenon and in association with diabetes and polyendocrinopathy and often antibodies to ___
Stiff-Person Syndrome; glutamic acid decarboxylase
Bilateral lesions of the brain stem or the thalamus results in ___
coma
Patient suffers injurious fall down the stairs. Neuro exam reveals right biceps weakness, absent right biceps reflex, and right shoulder and arm pain radiating to the right thumb. The likely dx is ___
C6 radiculopathy
Defiency in ___ is associated with subacute combined degeneration of the posterior columns of the spinal cord
Vitamin B12
Most reliable CSF finding in patients with chronic progressive phase of Multiple sclerosis
oligoclonal
___ is caused by JC virus. Occurs in pts with impaired immunity (AIDS, Chronic lymphocytic leukemia, Hodgkin’s lymphoma). P/w focal neuro deficits, seizure activity, and cognitive impairment characterized by memory impairment, psychomotor retardation, and inattentiveness
Progressive Multifocal Leukoencephalopathy (PML)
The etiology for ___ is Epstein-Barr virus. P/w progressive personality changes, seizures, and signs of increased ICP
CNS Lymphoma
__ presents with classic triad of abdo pain, peripheral neuropathy, and psychiatric disturbance (delirium, psychosis, depression, anxiety).
Acute intermittent porphyria
Young man p/w several days of progressive BLE weakness and numbness. Has hx flu-like illness about 10 days prior. Also with loss of sensation to all sensory modalities below the middle of the thorax and DTR are brisker below the waist. Dx ___
Acute/subacute transverse myelitis
“Patient states ““thot blegging at bremull fee felking””. He says this with normal intonation but no one can understand. He is able to respond to other questions with similar expressions but cannot execute any instructions. Dx ___”
Wernicke’s aphasia
___ p/w ocular motor apraxia (difficulty directing eyes away from central fixation), optic ataxia (inability to coordinate extremity movement under visual control), and impaired visual attention. Often associated with bilateral hemisphere lesions
Bulint’s syndrome
Pt p/w sudden onset R foot pain and leg paralysis. Right arm/hand/face are only slightly affected. No visual deficits. Over the ensuing weeks, pt is found to have lack of spontaneity, abulia, and loss of bladder controol. Stroke affected ___
Left anterior cerebral artery
In ___, an otherwise cognitively intact individual suddenly loses memory for recent events, asks repetitive questions about his/her environment, and sometimes confabulates. Lasts several to 24 hours.
Transient Global Amnesia
Treatment of choice for lancinating pain of Trigeminal Neuralgia
Carbamazepine
35 y/o pt p/w headache, fever, seizures, confusion, stupor that evolve ove 1 week. EEG shows periodic lateralized high-voltage sharp waves emanating from left temporal region. CT shows low-density lesion in the left temporal lobe. Most likely dx ___. (Fever in 90%; Headache in 80%, disorientation in 70%, personaity changes in 70-85%; focal or generalized seizures in 40-67%)
Herpes simplex virus-1 (HSV-1) encephalitis
70 y/o pt p/w leg stiffness w/ ambulation and LE spasms while sleeping. Neuro exam shows markedly stiff-legged gait with leg adduction while walking. LE tone is increased with a spastic catch and knee jerk reflexes are hyperactive and ankle jerk reflexes elicit clonus. Dx ___
Cervical spondylosis
___ commonly manifests with neck/face/head pain ipsilateral, frequently associated with ipsilateral Horner’s syndrome and often follows head or neck trauma.
Carotid artery dissection
Clostridium tetani causes trismus and dysphagia by ___
inhibiting GABA and glycine release in the brain and spinal cord
Broca’s aphasia results from lesion to ___ and p/w ___
left posterior inferior frontal gyrus; broken, stuttering, staccato speech, with inability to repeat. reading often impaired
Wernicke’s aphasia results from lesion in ___ and p/w ___
superior temporal gyrus; fluent speech but comprehension impaired, speech may be logorrheic or overproductive
Conduction aphasia results from lesion from ___ and p/w ___
arcuate fasciculus; inability to repeat, relatively normal spontaneous speech and possibility of paraphasic errors and hesitancy
Wallenburg syndrome is due to lesion in ___ and p/w ___
lateral medulla; ipsilateral Horner syndrome, ipsilateral loss of pain/temp sensation in face, cerebellar ataxia, weakness of vocal cords, contralateral loss of pain/temp sensation to the hemibody
A large stroke in the ___ can result in hemineglect, visual and tactile extinciton, impaired speech prosody (loss of musical and emotional inflection), anosognosia (not knowing you have a deficit) and behavioral problems (delirium, confusion)
Right MCA territory
Boy goes to summer camp and p/w sudden onset facial diplegia. Causative agent ___
Borrelia burgdorferi (Lyme disease)
Truncal ataxia or instability can result specifically from lesion to ___
cerebellar vermis
Lesion to ___ cause ipsilateral limb ataxia and/or dysmetria of either the arm/leg or both. can result in dysdiadochokinesis
cerebellar hemisphere
Lesions in ___ can result in cranial neuropathy, particularly V, VII, VIII. can p/w ipsilateral bells palsy, ipsilateral facial numbness, ipsilateral hearing loss/tinnitis/vertigo
cerebellopontine angle area
___ is a common characteristics of anterior horn diseases such as polymyositis and ALS
fasciculations
D2 overstimulation in ___ leads to tics and extraneous motor movements (parkinsons, tourettes, tics)
Caudate nucleus
Subacute combined degeneration of the spinal cord is due to ___
Deficiency of vitamin B12 (cobalamin)
Onset of muscle weakness is more common in the upper than the lower extremities, but in approximately 25% of patients, weakness begins in bulbar-innervated muscles. Pseudobulbar palsy may present with inappropriate or forced crying or laughter, which is often a source of great emotional distress for patients. The EMG examination characteristically reveals a combination of acute (positive sharp waves and fibrillation potentials) and chronic (reduced neurogenic firing pattern with evidence of increased amplitude and duration, polyphasic motor unit potentials) changes in a widespread distribution
ALS
___ commonly manifests with neck, face, and head pain ipsilateral to the dissection, frequently is associated with an ipsilateral Horner’s syndrome, and often follows head or neck trauma
Carotid artery dissection
3 core clinical criteria are fluctuations in cognitive function with varying levels of alertness and attention; visual hallucinations; and Parkinsonism
Lewy Body Disease
frontotemporal lobar degeneration –> pt can become aggressive and socially inappropriate, and demonstrate a lack of concern, apathy, abnormal self-awareness, and an inability to appreciate meaning. Speech and language abnormalities often begin early and progress rapidly.
Pick’s Disease
Asymmetric resting tremor in UE’s, lack of coordination, soft voice, decreased facial expression, progressive bradykinesia, rigidity and gait difficulty. Neurocognitive disorder generally occurs late
Parkinson’s Disease
A score of ___ on MMSE is consistent with moderate-severe dementia; appropriate for memantine
10-20 out of 30
In one study with neurocognitive disorder due to Parkinson’s disease, 90% of patients reported reduction in visual hallucinations with ___
cholinesterase inhibitors (Donepezil)
Which regions of the brain are most affected by HIV-associated dementia (HAD)?
Basal ganglia and hippocampus
___ is caused by infectious protein agents known as prions
Creutzfeldt-Jakob disease (CJD)
A 79 y/o man displays fluctuating levels of cognitive function. Which one of the following neurocognitive disorder diagnoses is most likely?
Neurocognitive disorder with Lewy bodies
Hyperphosphorylated tau protein found in the hippocampus is most common in which one of the following neurocognitive disorders?
Alzheimer’s Disease
___ characterized by rapidly progressive cognitive decline and behavioral change, as well as gait disturbance, myoclonic jerks, and fatigue
Creutzfeldt-Jakob disease
___ is the etiologic agent of progressive multifocal leukoencephalopathy (PML)
JC Virus
___ is deposited in disorders such as Lewy body disease, Parkinson ‘s disease, and REM sleep behavior disorder
Synuclein
Neurofibrillary tangles (aggregates of hyperphosphorylated tau) are seen in ___
Alzheimer’s disease
Pick cells (distinctive, enlarged vacuolar neurons with argentophilic neuronal inclusions in the cytoplasm bodies) are seen in ___
Neurocognitive disorder due to frontotemporal lobar degeneration
On autopsy, ___ can be seen in vascular dementia
Lacunar infarcts
On autopsy, ___ can be seen in Alzheimer’s disease
Neuritic plaques
In patients with neurocognitive disorder due to frontotemporal lobar degeneration, ___ is often seen on autopsy
TDP-43 proteinopathy
Which one of the following chromosomes is involved in the development of frontotemporal neurocognitive disorder?
Chromosome 17
___ are primarily used to treat the cognitive symptoms of neurocognitive disorder withLewy bodies, but they may also be of some benefit in reducing psychiatric and motor symptoms
Acetylcholinesterase inhibitors, such as donepezil and rivastigmine
___ is caused by the bacterium Tropheryma whipplei, which creates a malabsorption syndrome, as well as skin rashes, diarrhea, arthritis, and cognitive decline.
Whipple’s disease
The rapid decline of neurocognitive function over the course of a few months, along with the presence of a movement disorder like myoclonus (sudden, uncontrolled movement of limbs), strongly suggests ___. It is associated with co-morbid mental health disorders, including mood, anxiety, and panic disorders. EEG shows ___
prion disease
In an elderly patient with no other concerning history, ___ can present as disinhibition and impairment in judgment, sexually disinhibition, social inappropriateness, and psychosis (hallucinations and delusions). Stroke, neoplasm, or well-circumscribed focal infectious processes can cause this
orbitofrontal syndrome
Patient with neurocognitive dysfunction and 14-3-3 protein in CSF has ___
Creutzfeldt Jakob Disease
Genetic predisposition is the strongest risk factor in decreasing the age of onset of Alzheimer’s Disease in homozygous individuals having polymorphism of ___
apolipoprotein E4 gene
___ have been proven to be safe and efficacious for treating neuropsychiatric component of NCD with Lewy bodies
Cholinesterase inhibitors (Rivastigmine, donepezil, and galantamine)
In Creutzfeldt Jakob disease, EEG shows ___ at a certain point during the course of the disease but is not present in all patients. Comprises of movement disorders like ___
typical periodic, sharp, synchronous triphasic waves at a rate of 0.5-2 Hz
Carriers of ___ (which resides on chromosome 19) are at increased risk of posttraumatic dementia as well as Alzheimer’s disease
apo E4
MRI finding for patients with Huntington’s Disease
caudate and cerebral atrophy
Gait disturbance characterizied by involuntary acceleration found in Parkinson’s disease
Festination
Pt with ___ p/w motor weakness, areflexia, paresthesias with minor sensory loss, and increased protein in the CSF w/o pleocytosis (albuminocytological dissociation)
Guillan-Barre Syndrome
