Neuropathology Flashcards
(356 cards)
1
Q
Brain lesion that causes disinhibition, deficits in concentration, orientation and judgement
A
Frontal lobe
2
Q
Brain lesion that causes reemergence of primitive reflexes
A
Frontal lobe
3
Q
Brain lesion that causes eyes to look toward side of lesion (ipsilateral)
A
Frontal eye fields
4
Q
Brain lesion that causes eyes to look away from lesion
A
Paramedian pontine reticular formation
5
Q
Brain lesion that causes impaired adduction of ipsilateral eye with nystagmus of contralateral eye with abduction
A
Medial longitudinal fasciculus
6
Q
Internuclear ophthalmoplegia
A
Impaired adduction of ipsilateral eye with nystagmus of contralateral eye with abduction
7
Q
Demyelinating disease that causes internuclear ophthalmoplegia
A
Multiple sclerosis
8
Q
Brain lesion that causes agraphia, acalculia, finger agnosia, left-right disorientation
A
Dominant parietal cortex
9
Q
Neuropsychological disorder characterized by dyscalculia, dysgraphia, finger agnosia and left-right disorientation
A
Gerstmann syndrome
10
Q
Brain lesion that causes agnosia of the contralateral side of the world
A
Non-dominant parietal cortex
11
Q
Syndrome characterized by agnosia of the contralateral side of the world
A
Hemispatial neglect syndrome
12
Q
Inability to make new memories
A
Anterograde amnesia
13
Q
Brain lesion that causes anterograde amnesia
A
Hippocampus (bilateral)
14
Q
Brain lesion that causes tremor at rest, chorea, athetosis
A
Basal ganglia
15
Q
Neurodegenerative conditions that cause resting tremors, chorea, and athetosis
A
Huntington and Parkinson disease
16
Q
Brain lesion that causes contralateral hemiballismus
A
Subthalamic nucleus
17
Q
Brain lesion that causes confusion, ataxia, ophthalmoplegia, nystagmus memory loss, confabulation and personality changes
A
Mammillary bodies (bilateral)
18
Q
Neurological disorder characterized by confusion, ataxia, ophthalmoplegia, nystagmus memory loss, confabulation and personality changes
A
Wernicke-Korsakoff syndrome
19
Q
Syndrome characterized by hyperphagia, hypersexuality, hyperorality
A
Kluver-Bucy syndrome
20
Q
Brain lesion that causes Kluver-Bucy syndrome of disinhibited behavior
A
Amygdala (bilateral)
21
Q
Syndrome characterized by paralysis of conjugate vertical gaze
A
Parinaud syndrome
22
Q
Brain lesion that causes paralysis of conjugate vertical gaze
A
Superior colliculus
23
Q
Viral infection that causes of Kluver-Bucy syndrome
A
HSV-1 encephalitis
24
Q
Complications that cause Parinaud syndrome
A
Stroke, hydrocephalus, and pinealoma
25
Brain lesion that causes reduced levels of arousal and wakefulness
Reticular activating system (midbrain)
26
Brain lesion that causes intention tremor, limb ataxia, loss of balance toward side of lesion
Cerebellar hemispheres
27
Brain lesion that causes truncal ataxia and dysarthria
Cerebellar vermis
28
Degeneration of vermis is associated with what
Chronic alcohol use
29
After how many minutes does hypoxia begin to cause irreversible brain damage
5 minutes
30
Areas of brain most vulnerable to hypoxia
Hippocampus, neocortex, cerebellum, watershed areas
31
Which area of the brain is most vulnerable to ischemic hypoxia
Hippocampus
32
Best imaging modality to use to detect ischemia within 3-30 minutes
Diffusion-weighted MRI
33
Best imaging modality to use to detect ischemic changes within 6-24 hours
CT
34
Imaging modality used to exclude hemorrhage
Noncontrast CT
35
What needs to be done before tPA can be given in stroke patient
Exclude hemorrhage with noncontrast CT
36
Ischemic changes seen within 12-24 hours
Red neurons
37
Eosinophilic neuron with pyknotic nuclei
Red neuron
38
Ischemic changes seen after 24-72 hours
Necrosis plus neutrophils
39
Ischemic changes seen after 3-5 days
Macrophages (microglia)
40
Ischemic changes seen after 1-2 weeks
Reactive gliosis plus vascular proliferation
41
Ischemic changes seen after 2 weeks or more
Glial scar (cystic lesion with gliosis)
42
Type of necrosis seen after ischemic attack
Liquefactive necrosis
43
Types of ischemic strokes
Thrombotic, Embolic, Hypoxic
44
Type of stroke common in cardiovascular surgeries affecting watershed areas due to hypoperfusion or hypoxemia
Hypoxic stroke
45
Type of stroke due to a clot forming at site of infarction commonly affecting MCA or basilar and carotid bifurcation
Thrombotic stroke
46
Type of stroke affecting multiple vascular territories caused by a-fib, DVT with patent foramen ovale
Embolic stroke
47
Source of embolus in embolic stroke
Another part of the body
48
Ischemic stroke treatment
tPA if within 3.5 hours and no risk of hemorrhage
49
Steps to reduce risk of ischemic stroke
Give aspirin or clopidogrel, optimum control of BP, blood sugar, lipids and control conditions that increase risk (a-fib)
50
Brief reversible episode of focal neurologic dysfunction without acute infarction resolving within 15 minutes
Transient ischemic attack
51
Common locations of hemorrhagic strokes
Thalamus, basal nuclei, pons, cerebellum
52
Clopidogrel MOA
ADP receptor inhibitor
53
Common blood vessel injured in epidural hematoma
Middle meningeal artery (foramen spinosum)
54
Area of skull damaged in epidural hematoma
Pterion (thinnest area of lateral skull)
55
Skull bones that meet at pterion
Frontal, parietal, temporal, sphenoid bones
56
CT findings in epidural hematoma
Biconvex (lentiform) hyperdense blood collection not crossing suture lines
57
Common finding prior to symptoms in epidural hematoma
Lucid interval
58
Common cause of subdural hematoma
Rupture of bridging veins
59
Predisposing factors for subdural hematoma
Brain atrophy, trauma, older age, alcoholism
60
Common cause of subdural hematoma in infants
Shaken baby syndrome
61
CT findings in subdural hematoma
Crescent-shaped hemorrhage that crosses suture lines
62
Type of herniation seen in epidural hematoma
Transtentorial herniation causing CN III palsy
63
Type of herniation seen in subdural hematoma
Midline shifting of ventricles
64
Common cause of subarachnoid hemorrhage
Bleeding from trauma or rupture of aneurysms
65
Types of aneurysms that commonly rupture in subarachnoid hemorrhage
Saccular or Berry aneurysms
66
Symptoms of subarachnoid hemorrhage
"Worst headache of my life", bloody or yellow spinal tap
67
Complication of subarachnoid hemorrhage
Blood breakdown or rebleed cause vasospasms resulting in ischemic infarct
68
Treatment in subarachnoid hemorrhage to reduce or prevent vasospasms
Nimodipine
69
What genetic diseases are associated with saccular and Berry aneurysms
Marfan syndrome and ADPKD
70
What risk is increased in subarachnoid hemorrhages
Risk of developing communicating and/or obstructive hydrocephalus
71
Brain hemorrhage most commonly caused by systemic HTN
Intraparenchymal hemorrhage
72
Conditions associated with intraparenchymal hemorrhage
Amyloid angiopathy, vasculitis, neoplasms
73
Intraparenchymal hemorrhage may be secondary to what type of injury
Ischemic stroke
74
Common location of intraparenchymal hemorrhage
Basal ganglia and internal capsule
75
Condition that develops in perforating vessels as a result of HTN
Charcot-Bouchard microaneurysm of lenticulostriate vessels
76
Presentation of intraparenchymal hemorrhage
Presents with headache, nausea, vomiting, and eventually coma
77
Effects of MCA stroke to temporal lobe (Wernicke); frontal lobe (Broca)
Aphasia if dominant (left hemisphere)
| Hemineglect if non-dominant (right hemisphere)
78
Effects of MCA stroke to frontal lobe
Broca's aphasia
79
Effects of MCA stroke to motor and sensory cortices
Contralateral paralysis and sensory loss (face and upper limb)
80
Area of brain associated with Wernicke aphasia
Right superior quadrant
81
Area of brain involved in MCA stroke causing visual field deficits
Temporal lobe
82
Effects of Anterior cerebral stroke to motor and sensory cortices
Contralateral paralysis and sensory loss (lower limb)
83
Effects of Lenticulostriate artery stroke to striatum, internal capsule
Contralateral paralysis and/or sensory loss (face and body)
| Absence of cortical signs (neglect, aphasia, visual loss)
84
Common cause of lacunar infarcts
Hyaline arteriosclerosis due to unmanaged hypertension
85
Effects of Anterior spinal artery stroke to Lateral corticospinal tract
Contralateral paralysis (upper and lower limbs)
86
Effects of Anterior spinal artery stroke to Medial lemniscus
Decreased contralateral proprioception
87
Effects of Anterior spinal artery stroke to Caudal medulla-hypoglossal nerve
Ipsilateral hypoglossal dysfunction (tongue deviates ipsilaterally)
88
Ipsilateral deviation of tongue, contralateral limb weakness, contralateral sensory loss
Medial medullary syndrome
89
Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting nucleus ambiguus
Dysphagia, hoarseness, decreased gag reflex
90
Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting Vestibular nuclei
Vomiting, vertigo, nystagmus
91
Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting lateral spinothalamic tract, spinal trigeminal nucleus
Decreased pain and temperature from contralateral body, ipsilateral face
92
Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting Sympathetic fibers
Ipsilateral Horner syndrome
93
Effects of Posterior inferior cerebellar artery stroke to lateral medulla affecting Inferior cerebellar peduncle
Ataxia, dysmetria
94
Artery specific to Nucleus ambiguus effects
PICA
95
Artery supplying Inferior cerebellar peduncle
PICA
96
Syndrome characterized by Ataxia, dysmetria; Ipsilateral Horner syndrome; Decreased pain and temperature on contralateral body and ipsilateral face; Vomiting, vertigo, nystagmus; Dysphagia, hoarseness, decreased gag reflex
Lateral Medullary syndrome
97
Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Facial nucleus
Face paralysis, decreased lacrimation, salivation, and taste from anterior 2/3 of tongue
98
Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Vestibular nuclei
Vomiting, vertigo, nystagmus
99
Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Spinothalamic tract, spinal trigeminal nucleus
Decreased pain and temperature from contralateral body and ipsilateral face
100
Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Sympathetic fibers
Ipsilateral Horner syndrome
101
Effects of Anterior inferior cerebellar artery stroke to lateral pons affecting Middle and Inferior cerebellar peduncles
Ataxia, dysmetria
102
Syndrome characterized by Face paralysis, decreased lacrimation, salivation, and taste from anterior 2/3 of tongue; Vomiting, vertigo, nystagmus; Decreased pain and temperature from contralateral body and ipsilateral face; Ataxia, dysmetria
Lateral pontine syndrome
103
Facial nucleus affects are specific to a lesion of what artery
AICA
104
Effects of Basilar artery stroke to Pons, medulla, lower midbrain
Completely paralyzed but RAS spared, therefore preserved consciousness
105
Condition in which patient is fully aware and can only move their eyes
Locked-in syndrome
106
Effects of Basilar artery stroke to Corticospinal and corticobulbar tracts
Quadriplegia with loss of voluntary facial, mouth and tongue movements
107
Effects of Basilar artery stroke to Ocular cranial nerve nuclei and PPRF
Loss of horizontal, but not vertical eye movements
108
Effects of Posterior cerebellar artery stroke to Occipital lobe
Contralateral hemianopia with macular sparing
109
Initial paresthesias followed in weeks to months by allodynia and dysesthesia due to thalamic lesions in 10% of stroke patients
Central post-stroke pain syndrome
110
Lesion to inferior frontal gyrus of frontal lobe in which patient has non-fluent speech but intact comprehension
Broca's aphasia
111
Lesion to arcuate fasciculus with intact comprehension and fluent paraphrasic speech
Conduction aphasia
112
Lesion to arcuate fasciculus affecting Broca and Wernicke areas with impaired comprehension and non-fluent speech
Global aphasia
113
Lesion to superior temporal gyrus of temporal lobe with impaired comprehension and non-sensical fluent speech
Wernicke's aphasia
114
Lesion affecting frontal lobe with sparing of Broca's area with intact comprehension and non-fluent speech
Transcortical motor aphasia
115
Lesion affecting watershed areas with sparing of Broca's, Wernicke's and arcuate fasciculus with impaired comprehension and non-fluent speech
Transcortical, mixed aphasia
116
Lesion affecting temporal lobe with sparing of Wernicke's area with impaired comprehension and fluent speech
Transcortical sensory aphasia
117
Most common site of Berry aneurysms
Junction of anterior communicating artery and ACA
118
Diseases associated with berry aneurysms
ADPKD and Ehlers-Danlos syndrome
119
Risk factors for berry aneurysms
older age, HTN, smoking, race
120
Race at increased risk of berry aneurysms
African-Americans
121
Symptoms of Anterior communicating artery compression aneurysm
Bitemporal hemianopia (optic chiasma compression)
122
Symptom of posterior communicating artery compression
Ipsilateral CN III palsy (mydriasis, ptosis, "down and out" eye
123
Disorder of recurrent seizures
Epilepsy
124
Continuous or recurring seizures that may result in brain injury lasting 5-30 minutes
Status epilepticus
125
Seizure with impaired consciousness
Complex partial seizure
126
Seizure with motor, sensory, autonomic, psychic auras with preserved consciousness
Simple partial seizure
127
Seizures affecting single area of brain, often preceded by auras
Partial seizures
128
Lobe commonly involved with partial seizures
Medial temporal lobe
129
Seizure characterized by blank stare and no postictal confusion
Absence seizure
130
Seizure characterized by quick, repetitive jerks
Myoclonic seizure
131
Seizure characterized by alternating stiffening and movement
Tonic-clonic seizure
132
Seizure characterized by 3 Hz spike-and-wave discharges
Absence seizure
133
Seizure characterized by stiffening
Tonic seizure
134
Seizure characterized by "drop" to floor and commonly mistaken for fainting
Atonic seizure
135
Common causes of seizures in children
Genetic, febrile seizures, trauma, congenital metabolic
136
Common causes of seizures in adults
Tumor, trauma, stroke, infection
137
Common causes of seizures in elderly
Stroke, tumor, trauma, metabolic, infectioin
138
Repetitive, brief, unilateral headaches with severe periorbital pain with lacrimation and rhinorrhea
Cluster headache
139
Treatment for acute cluster headaches
Sumatriptan, 100% O2
140
Prophylactic treatment for cluster headaches
Verapamil (CCB)
141
Unilateral headache that last 15 min to 3 hours, is repetitive and may present with Horner syndrome
Cluster headache
142
Unilateral headache with pulsating pain, nausea, photophobia and phonophobia; possible aura lasting 4 to 72 hours
Migraine headache
143
Cause of migraine
Irritation of CN V, meninges or blood vessels
144
Substances released from blood vessels causing migraine
Substance P, calcitonin gene-related peptide and vasoactive particles
145
Acute treatment for migraine
NSAIDs, triptans, dihydroergotamine
146
Prophylactic treatment for migraines
Lifestyle changes, beta-blockers, CCBs, amitryptaline, topiramate, valproate
147
Bilateral headache with steady pain and constant lasting 4 to 6 hours
Tension headache
148
Treatment for tension headaches
Analgesics, NSAIDs, acetaminophen; (amitriptyline for chronic pain)
149
Repetitive, unilateral, shooting pain in the distribution of CN V lasting less than 1 minute
Trigeminal neuralgia
150
Treatment for trigeminal neuralgia
Carbamazepine
151
Restlessness and intense urge to move; cannot sit still
Akathisia
152
Common causes of akathisia
Neuroleptics use or Parkinson disease
153
Extension of wrists causes flapping motion
Asterixis
154
Basal ganglia lesion causing slow, snake-like, writhing movements, especially in fingers
Athetosis
155
Basal ganglia lesion causing sudden jerky, purposeless movements
Chorea
156
Type of chorea seen in acute rheumatic fever and Huntington disease
Sydenham chorea
157
Sustained, involuntary muscle contractions like torticollis, writer's cramp or blepharospasm
Dystonia
158
High-frequency tremor with sustained posture worsened with movement or when anxious
Essential tremor
159
Patients with essential tremors often self-medicate with which substance
Alcohol
160
Treatment for essential tremors
Primidone or non-selective beta-blockers like propranolol
161
Contralateral subthalamic lesion causing sudden, wild flailing of 1 arm; maybe ipsilateral leg
Hemiballismus
162
Cerebellar dysfunction causing slow, zigzag motion when pointing/extending toward a target
Intention tremor
163
Sudden, brief, uncontrolled muscle contraction
Myoclonus
164
Substantia nigra lesion causing uncontrolled movement of distal appendages alleviated by intentional movement
Resting tremor
165
Disease causing intention tremor characterized by "pill-rolling tremor" at rest
Parkinson disease
166
Signs and symptoms of Parkinson disease
Tremor at rest, Rigidity, Akinesia, Postural instability, Shuffling gait (TRAP)
167
Description of Lewy bodies
Made of alpha-synuclein
168
Intracellular eosinophilic inclusions seen in Parkinson disease made of alpha-synuclein
Lewy bodies
169
Cause of movement disorders in Parkinson disease
Loss of dopaminergic neurons of substantia nigra pars compacta
170
Illegal street drug contaminant that can cause Parkinson disease
MPTP
171
Autosomal dominant trinucleotide repeat in Huntington disease
CAG
172
Chromosome affected in Huntington disease
Chromosome 4
173
Brain structure affected in Huntington disease
Atrophy of caudate and putamen with ex vacuo ventriculomegaly
174
Cause of neuronal death in Huntington disease
NMDA-R binding and glutamate excitotoxicity
175
Neurotransmitter changes in Huntington disease
Increased dopamine
| Decreased GABA, ACh
176
Signs and symptoms of Huntington disease
Chorea, Dementia, Athetosis, Depression, Aggression (C DADA)
177
When does anticipation occur
Spermatogenesis
178
Most common cause of dementia in elderly
Alzheimer disease
179
Who is at risk of developing early onset dementia
Down syndrome patients
180
Cause of Alzheimer disease in Down syndrome
Amyloid precursor protein is located on chromosome 21
181
What are neurofibrillary tangles
Hyperphosphorylated tau protein
182
What are senile plaques
Beta-amyloid core
183
Location of neurofibrillary tangles
Intracellular
184
How is A-beta (amyloid-beta) made
Cleavage of amyloid precursor protein (APP)
185
Which Apo protein is associated with decreased risk of sporadic form of Alzheimer's
ApoE2
186
Location of senile plaques
Extracellular space
187
Gross findings in Alzheimer disease
Widespread cortical atrophy, especially hippocampus with narrowing of gyri and widening of sulci
188
Microscopic findings in Alzheimer disease
Intracellular neurofibrillary tangles and extracellular senile plaques
189
How many copies of APP gene are found in Down syndrome
3 copies on chromosome 21
190
Familial form of Alzheimer's is associated with which proteins
Presenelin-1 and presenelin-2
191
Which Apo protein is associated with sporadic form of Alzheimer's
ApoE4
192
Gross findings in frontotemporal dementia
Frontotemporal degeneration
193
Microscopic findings in frontotemporal dementia
Inclusions of hyperphosphorylated tau
194
Appearance of tau protein in frontotemporal dementia
Round Pick bodies
195
Signs and symptoms of frontotemporal dementia
Changes in personality and behavior or aphasia; possible movement disorders
196
Microscopic findings in Lewy body dementia
Intracellular Lewy bodies primarily in cortex
197
Signs and symptoms of Lewy body dementia
Dementia and visual hallucinations, parkinsonian features
198
Cause of vascular dementia
Multiple arterial infarcts and or chronic ischemia
199
Second most common cause of dementia in elderly
Vascular dementia
200
Signs and symptoms of vascular dementia
Step-wise decline in cognitive ability with late-onset memory impairment
201
Cause of vascular dementia
HTN, atherosclerosis or vasculitis
202
Image findings in vascular dementia
Multiple cortical and or subcortical infarcts
203
Rapidly progressive dementia with myoclonus
Creutzfeldt-Jacob disease
204
Common findings in Creutzfeldt-Jacob disease
Periodic sharp waves on ECG and increases 14-3-3 protein in CSF
205
Common gross and microscopic findings in Creutzfeldt-Jacob disease
Spongiform cortex and prions
206
What are prions made of
Beta-pleated sheet resistant to proteases
207
Increased ICP with no apparent cause
Idiopathic ICP
208
Another name of idiopathic ICP
Pseudotumor cerebri
209
Risk factors associated with pseudotumor cerebri
Female gender, obesity, vitamin A excess, tetracycline, danazol
210
Signs and symptoms of pseudotumor cerebri
Headache, diplopia, papilledema with no mental status change
211
Lumbar puncture findings in pseudotumor cerebri
Increased opening pressure with headache relief
212
Treatment for pseudotumor cerebri
Weight loss, acetazolamide, topiramate
213
Treatment for refractory pseudotumor cerebri
Repeat lumbar puncture, CSF shunt placement, or optic nerve sheath fenestration surgery
214
Common findings in communicating hydrocephalus
Increased ICP, papilledema and herniation
215
Common cause of communicating hydrocephalus
Arachnoid scarring post-meningitis
216
Common symptoms in normal pressure hydrocephalus
Urinary incontinence, ataxia, cognitive dysfunction and magnetic gait (feet appear stuck on floor)
217
Who is at risk for normal pressure hydrocephalus
Elderly
218
Image findings in normal pressure hydrocephalus
Expansion of ventricles
219
Common findings in normal pressure hydrocephalus
Episodic elevated CSF pressure and no increased subarachnoid space volume
220
Appearance of increased CSF on imaging but actually due to decreased brain tissue and neuronal atrophy with normal ICP
Ex vacuo ventriculomegaly
221
Acute paralysis, dysarthria, dysphagia, diplopia, loss of consciousness secondary to osmotic changes
Osmotic demyelination syndrome
222
Other name for osmotic demyelination syndrome
Central pontine myelinolysis
223
Complication of osmotic demyelination syndrome
Locked-in syndrome
224
Common cause of osmotic demyelination syndrome
Iatrogenic from overly rapid correction of hyponatremia
225
Result of correcting hypernatremia too quickly
Cerebral edema/herniation
226
Autoimmune inflammation and demyelination of oligodendrocytes in CNS
Multiple sclerosis
227
Most common clinical course of multiple sclerosis
Relapsing and remitting
228
Population commonly affected by multiple sclerosis
Caucasian women in 20s and 30s living farther from equator
229
Symptoms of MS
Scanning speech, Intention tremor, Incontinence, Internuclear ophthalmoplegia, Nystagmus, Hemiparesis, Hemisensory symptoms
230
Charcot triad
Scanning speech, Intention tremor, Nystagmus (SIN)
231
Findings in MS
Increased IgG levels and myelin basic protein in CSF
232
Diagnosis of MS
Oligoclonal bands on MRI, paraventricular plaques with preservation of axon and multiple white matter lesions
233
Paraventricular plaques
Areas of oligodendrocyte loss and reactive gliosis
234
Treatment for MS to slow progression and modify disease
Beta-interferon slows progression, glatiramer and natalizumab
235
Acute flair treatment for MS
IV steroids
236
Symptomatic treatment for neurogenic bladder in MS
Catheterization, muscarinic antagonists
237
Symptomatic treatment for spasticity in MS
Baclofen, GABA receptor antagonist
238
Symptomatic treatment for pain in MS
TCAs, anticonvulsants
239
Autoimmune condition that destroys Schwan cells causing inflammation and demyelination of peripheral nerves and motor fibers
Guillain-Barre syndrome
240
Type of paralysis seen in Guillain-Barre syndrome
Symmetric, ascending paralysis beginning in lower extremities
241
Prognosis of Guillain-Barre syndrome
Almost all patients survive with recovery in weeks to months
242
CSF findings in Guillain-Barre syndrome
Increased CSF protein that may cause papilledema
243
Bacteria associated with Guillain-Barre syndrome
Campylobacter jejuni
244
Treatment for Guillain-Barre syndrome
Respiratory support, plasmapheresis, IV immunoglobulins
245
Multifocal inflammation and demyelination after infection or vaccination with rapidly progressive multifocal neurological symptoms and altered mental status
Acute disseminated encephalomyelitis
246
Disease also known as hereditary motor and sensory neuropathy
Charcot-Marie-Tooth disease
247
Typical inheritance pattern of Charcot-Marie-Tooth disease
Autosomal dominant
248
Mechanism of Charcot-Marie-Tooth disease
Defective proteins involved in structure and function of peripheral nerves or myelin sheath
249
Typical findings associated with Charcot-Marie-Tooth disease
Foot deformities (pes cavus, hammer toe), lower extremity weakness and sensory deficits
250
Inheritance pattern for Krabbe disease
Autosomal recessive
251
Defect in Krabbe disease
Galactocerebrosidase deficiency
252
Mechanism of Krabbe disease
Galactocerebroside and psychosine buildup destroy myelin
253
Findings in Krabbe disease
Peripheral neuropathy, developmental delay, optic atrophy, globoid cells
254
Inheritance pattern for metachromatic leukodystrophy
Autosomal recessive
255
Defect in metachromatic leukodystrophy
Arylsulfatase A deficiency
256
Mechanism of metachromatic leukodystrophy
Buildup of sulfatides impairs production and destroys myelin
257
Findings in metachromatic leukodystrophy
Central and peripheral demyelination with ataxia and dementia
258
Most common leukodystrophy
Metachromatic leukodystrophy
259
Demyelinating disease of CNS seen in reactivation of latent JC virus infection that is rapidly progressive and usually fatal
Progressive multifocal leukoencephalopathy
260
Drugs that increase risk of progressive multifocal leukoencephalopathy
Natalizumab and rituximab
261
Population affected by progressive multifocal leukoencephalopathy
AIDS patients
262
Inheritance pattern of adrenoleukodystrophy
X-linked
263
Mechanism of adrenoleukodystrophy
Disruption of very-long-chain fatty acid metabolism increases fatty acids damaging adrenal glands, testes and nervous system
264
Complications of adrenoleukodystrophy
Long-term coma and death in adrenal crisis
265
What is the defect in adrenoleukodystrophy
Cannot add coenzyme A to long-chain fatty acids
266
Sturge-Weber syndrome inheritance
Congenital, non-inherited
267
Mechanism of Sturge-Weber syndrome
Mutation in GNAQ gene causes developmental anomaly in neural crest cell derivatives
268
Sturge-Weber syndrome acronym for symptoms
STURGE: Sporadic, Tram track calcifications, Unilateral, Retardation, GNAQ, Glaucoma, Epilepsy
269
Genetic defect of tuberous sclerosis
TSC1/TSC2 mutation on chromosome 16
270
Inheritance pattern of tuberous sclerosis
Autosomal dominant, variable expression
271
Common findings in tuberous sclerosis
```
HAMARTOMAS:
Hamartomas in CNS and skin
Angiofibromas
Mitral regurgitation
Ash-leaf spots
Rhabdomyoma
Tuberous sclerosis
autosomal dOminant
Mental retardation
Angiomyolipoma
Seizures, Shagreen patches
```
272
Inheritance pattern for NF1
Autosomal dominant, 100% penetrance
273
Mutation in NF1
NF1 tumor suppressor gene on chromosome 17
274
Function of NF1
Codes for neurofibromin
275
Function of neurofibromin
Negative regulator of RAS
276
Signs and symptoms in NF1
Café-au-lait spots, cutaneous neurofibromas, optic gliomas, pheochromocytoma, Lisch nodules
277
Pigmented iris hamartomas
Lisch nodules
278
Inheritance patter for NF2
Autosomal dominant
279
Mutation in NF2
NF2 tumor suppressor on chromosome 22
280
Signs and symptoms in NF2
Bilateral acoustic schwannomas, juvenile cataracts, meningiomas, and ependymomas
281
NF2 affects what
2 ears, 2 eyes, and 2 parts of brain
282
Inheritance pattern for von Hippel-Lindau disease
Autosomal dominant
283
Mutation in von Hippel-Lindau disease
Deletion of VHL gene on chromosome 3
284
Findings in von Hippel-Lindau disease
```
HARP:
Hemangioblastomas - retina, brain stem, spine cerebellum
Angiomatosis - skin, mucosa, organs
Renal cell carcinoma - bilateral
Pheochromocytoma
```
285
Common, highly malignant brain tumor that can cross corpus callosum
Glioblastoma
286
Cell of origin in glioblastoma
Astrocyte
287
Microscopic findings in glioblastoma
GFAP positive, "pseudopalisading" pleomorphic tumor cells around central area of necrosis
288
Mass with "fried egg" cells in frontal lobe white matter and "chicken wire" capillary pattern presenting with seizures
Oligodendroglioma
289
Benign brain tumor with dural attachment, spindle cells in whirled pattern and psammoma bodies presenting with seizures
Meningioma
290
Cerebellar tumor associated with von Hippel-Lindau syndrome with thin-walled capillaries presenting with polycythemia
Hemangioblastoma
291
Cell of origin in meningioma
Arachnoid cell
292
S-100 positive tumor at the cerebellopontine angle localized to CN VIII
Schwannoma
293
Cell of origin in schwannoma
Schwan cells
294
Cell of origin in hemangioblastoma
Blood vessel
295
Most common adult tumor
Meningioma
296
Well circumscribed, GFA positive tumor in posterior fossa with eosinophilic, corkscrew fibers
Astrocytoma
297
Most common malignant brain tumor in childhood
Medulloblastoma
298
Most common benign CNS tumor in childhood
Astrocytoma
299
Cell of origin in astrocytoma
Glial cell
300
Cell of origin in medulloblastoma
Neuroectodermal
301
Brain tumor in cerebellum causing non-communicating hydrocephalus with Homer-Wright rosettes and small blue cells on histology
Medulloblastoma
302
Malignant brain tumor in 4th ventricle causing hydrocephalus with perivascular rosettes and basal ciliary bodies near the nucleus on histology
Ependymoma
303
Most common childhood supratentorial tumor
Craniopharyngioma
304
Cell of origin in ependymoma
Ependymal cells
305
Cell of origin in craniopharyngioma
Remnants of Rathke pouch
306
Brain tumor causing bitemporal hemianopia with calcification and cholesterol crystals in motor oil-like fluid within tumor on histology
Craniopharyngioma
307
Brain tumor causing vertical gaze palsy, obstructive hydrocephalus, increased beta-HCG production in boys
Pinealoma
308
Artery compressed with cingulate herniation under falx cerebri
Anterior cerebral artery
309
Complications of transtentorial herniation
Caudal displacement of brain stem rupturing paramedian basilar artery branches leading to Duret hemorrhages
310
Complications of Uncal herniation to ipsilateral CNIII
Blown pupil - down and outward gaze
311
Complications of Uncal herniation to ipsilateral PCA
Contralateral homonymous hemianopia with macular sparing
312
Complications of Uncal herniation to contralateral crus cerebri
Ipsilateral paresis
313
Complications of cerebellar herniation into the foramen magnum
Coma and death when brain stem compressed
314
UMN sign lesions
Weakness, Up reflexes, tone, & Babinski; spastic paresis, clasp knife spasticity
315
LMN sign lesions
Weakness, atrophy, fasciculations, Down reflexes, tone; flaccid paralysis
316
Congenital degeneration of anterior horns of spinal cord causing "floppy baby"
Werdnig-Hoffmann disease
317
Motor nerves affected in Werdnig-Hoffmann disease
LMN's
318
Symptoms in Werdnig-Hoffmann disease
Symmetric weakness, hypotonia and tongue fasciculations
319
Inheritance pattern of Werdnig-Hoffmann disease
Autosomal recessive
320
Degeneration of anterior horns of spinal cord causing asymmetric weakness
Poliomyelitis
321
Combined UMN and LMN deficits from loss of cortical and spinal cord neurons presenting with asymmetric limb weakness, fasciculations and eventual atrophy
Amyotrophic lateral sclerosis (ALS)
322
Other name for amyotrophic lateral sclerosis
Lou Gehrig disease
323
Enzyme defective in familial ALS
Superoxide dismutase I
324
Treatment for ALS
Riluzole
325
Presentation of complete occlusion of anterior spinal artery
Below lesion: UMN deficits and loss of pain and temp
| Level of lesion: LMN deficits
326
Sensory and Motor tracts affected in complete occlusion of anterior spinal artery
Cortical spinal tract below lesion - UMN deficits
Spinothalamic tract below lesion - pain and temp
Anterior horn at level of lesion - LMN deficits
327
Artery that supplies anterior spinal artery at T8 spinal level
Artery of Adamkiewicz
328
Lesion that caused by tertiary syphilis resulting in demyelination of dorsal columns and roots presenting with absence of DTRs and positive Romberg sign
Tabes dorsalis
329
Patient with Argyll Robertson pupils and poor coordination most likely has what defect of spinal cord
Demyelination of dorsal columns and roots
330
Lesion causing bilateral loss of pain and temp in cape-like distribution and associated with Chiari I malformation
Syringomyelia
331
Area of spinal cord affected by syringomyelia
Anterior white commissure of spinothalamic tract
332
Sensory and motor tracts affected in patient with ataxic gait, paresthesia and impaired position/vibration sense with vitamin B12 deficiency
Spinocerebellar tracts, lateral Corticospinal tracts and Dorsal columns
333
Person with compression of spinal roots from L2 and below is at risk for what complications
Loss of bladder and anal sphincter control, radicular pain, saddle anesthesia
334
Motor nerves affected in cauda equina syndrome
LMNs
335
Cauda equina syndrome
Radicular pain, absent knee and ankle reflex, loss of bladder and anal sphincter control
336
Mechanism of poliomyelitis
Poliovirus replicates in oropharynx and small intestine then spreads to bloodstream and CNS
337
Area of CNS affected by poliomyelitis
Anterior horn of spinal cord causing LMN death
338
Diagnosis of patient presenting with fever, malaise, headache, nausea, LMN signs with increased WBCs and slight protein increase in CSF
Poliomyelitis
339
Symptoms present with damage to oculosympathetic pathway
Miosis, ptosis, anhydrosis
340
Horner syndrome
Miosis, ptosis, anhydrosis
341
Presentation of hemisection of spinal cord
Ipsilateral loss of all sensation at lesion level
Ipsilateral LMN signs at lesion level
Ipsilateral UMN signs below lesion
Ipsilateral loss of fine touch and proprioception below lesion
Contralateral pain and temp loss below lesion
Horner syndrome if lesion above T1
342
Brown-Sequard syndrome
Lesion affecting hemisection of spinal cord
343
Inheritance pattern of Friedreich ataxia
Autosomal recessive
344
Defect in Friedreich ataxia
Trinucleotide GAA repeat on chromosome 9
345
Mechanism of Friedreich ataxia
Defective frataxin gene impairs mitochondria leading to degeneration of spinal cord tracts
346
Cause of death in Friedreich ataxia
Hypertrophic cardiomyopathy
347
Presentation of Friedreich ataxia in childhood
Kyphoscoliosis with staggering gait, falling, diabetes, pes cavus, hammer toes, nystagmus and dysarthria
348
Presentation of CN V motor lesion
Jaw deviates toward side of lesion from unopposed pterygoid muscle on opposite side
349
Presentation of CN X lesion
Uvula deviates away from side of lesion
350
Presentation of CN XI lesion
Contralateral weakness turning head and ipsilateral shoulder droop
351
Presentation of CN XII lesion
Tongue deviates to side of lesion
352
Motor neuron affect with CN XII lesion
LMN
353
Motor neuron lesion causing ipsilateral paralysis of upper and lower muscles of face with taste loss to anterior 2/3 of tongue and hyperacusis
LMN lesion
354
Cause of LMN lesion causing ipsilateral paralysis of upper and lower muscles of face
Destruction of facial nucleus or CN VII anywhere along its path
355
Cause of UMN lesion causing lower facial paralysis
Contralateral destruction of motor cortex or connection between motor cortex and facial nucleus
356
Motor neuron lesion causing contralateral paralysis of lower face muscles with sparing of forehead
UMN lesion
