Exam 7 Flashcards

(268 cards)

1
Q

Migraines effect up to…

A

12% of the population, more women

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2
Q

Migraine pathophysiology

A

Genetically predisposed hyperexcitable neurovascular system

Cortical spreading, vasodilation and inflammation

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3
Q

Migraine prodrome

A

60% of migraine sufferers

24-48 hours before HA

Euphoria/ depression/ irritability
Food cravings
Constipation
Neck stiffness
Increased yawning
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4
Q

Migraine aura

A

25% of migraine sufferers

Visual (most common)
Sensory
Verbal
Motor

Less than one hour!! Completely reversible

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5
Q

Visual aura of migraine

A
Field defects
Luminous visual hallucinations
Scintillating scotomas (field defects and luminous visual hallucinations)
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6
Q

Motor aura

A

Familial hemiplegic migraine- autosomal dominant, fully reversible motor weakness

Sporadic hemiplegic migraine- no relative has had an attack

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7
Q

Migraine HA characteristics

A

4-72 hours

At least 2:
Unilateral
Pulsating or throbbing
Moderate or severe
Aggravated by physical activity

NV, photophobia or photophobia

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8
Q

Migraine postdrome

A

Sudden head movement causes pain, exhaustion, mild elation or euphoria

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9
Q

Migrainous vertigo

A

Recurrent episodic vertigo in a patient with migraines

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10
Q

Basilar type migraine

A

Migraine with aura symptoms that come from the brainstem and/or both hemispheres simultaneously

NO MOTOR WEAKNESS

At least two of these fully reversible stroke like symptoms

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11
Q

Retinal/ocular migraine

A

Repeated attacks of monocular scotoma or blindness, less than 1 hour

Followed by HA

Irreversible visual loss is common here

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12
Q

Ophthalmoplegic migraine

A

Lateralized eye pain with NV, diplopia

HA for a week or more, latent period of around 4 days before ophthalmoplegia

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13
Q

Consider prophylaxis for migraines if…

A

2 or three migraines per month or significant disability with attacks

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14
Q

Tension headache epidemiology

A

86% prevalence, more women than men

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15
Q

TTH patho

A

Heightened sensitivity of pain pathways

Increased muscle tenderness

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16
Q

TTH presentation

A

Vise like, tight
Neck or back of head
Generalized and bilateral
Mild to moderate

No neuro symptoms!

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17
Q

Three main types of TTH

A

Infrequent episodic- less than 1 day a month

Frequent episodic- 1-14 days per month

Chronic- greater than 15 days per month

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18
Q

Duration and at least 2 of these for TTH

A

30 min -7 days for episodic

Pressing/tightening
Mild to moderate
Bilateral
Not aggrated by routine physical activity

*no NV, no photobia or phonophobia

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19
Q

Cluster headache presentation

A
Severe pain
Orbital, temporal, supraorbital
Strictly unilateral
At night, awake the patient
Autonomic phenomena
Restless, pacing, rocking back and forth
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20
Q

Cluster headache timing

A

Up to 8 times a day

Short lived, 15 minutes to 3 hours

Cluster period can last 6-12 weeks

Remissions can last 12 months even

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21
Q

Autonomic symptoms in clusters

A

Ipsilateral to side of pain

Ptosis
Miosis
Lacrimation
Conjunctival injection
Rhinorrhea
Nasal congestion
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22
Q

Horner’s syndrome

A

Ptosis, miosis, anhidrosis

Common in cluster headaches

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23
Q

Types of cluster headaches

A

Episodic- most common, 2 cluster periods lasting 7 days to 1 year separated by pain free periods of 1 month or more

Chronic- more than 1 year without remission or with remissions less than 1 month

Probable- fulfilling all but one criteria

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24
Q

Workup of cluster

A

Unlike other headaches, have to get brain CT or MRI on these patients

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25
Acute cluster traetment
SubQ sumatriptan and oxygen, upright position with 12 L/min 100%
26
Meds and surgical procedures to prevent cluster HA
Verapamil, Li, steroids and topiramate Radio frequency, gamma knife, nerve sectioning, cocaine and alcohol
27
Headache red flags
Progressive HA Disturbs sleep Exertional HA Associated neurologic symptoms, focal deficiency Fever Onset of new or different HA Onset of HA after age of 50
28
Primary cough HA
HA provoked by coughing or straining in the absence of intracranial disorder >40 years old
29
Primary cough HA features
Sudden Bilateral Seconds to 30 minutes Not associated with NV, photo/phonophobia
30
Primary cough HA workup
Must do imaging, could have low ICP, IC hypervolemia, carotid artery occlusion, posterior fossa lesion, chiari type 1 malformation, unruptured aneurysm
31
Imaging primary cough HA
CT or MRI repeated annually for several years
32
Giant cell arteritis
Chronic vasculitis effecting large and medium sized arteries Blindness is the worst complication
33
GCA HA diagnosis
72 years old average, almost never younger than 50 If over 50 and new laterlized HA, abrupt visual disturbance, polymyalgia rheumatica, jaw claudication, fever or anemia, anorexia, weight loss
34
Diagnostic tests of GCA
Temporal artery biopsy Halo sign on ultrasound Elevated ESR
35
GCA treatment
Start prednisone right away!
36
HA from intracranial mass lesions
Posterior fossa- occipital HA Supratentorial lesions- bifrontal HA Symptoms MAY be nonspecific and variable, worsen with activity and postural change, or have associated NV
37
Medication overuse headache
Chronic daily headache from someone with a pre existing HA disorder Addictive personalities, anxiety and fear of HA
38
MOH most commonly results from overuse of...
Opioids Butabital containing combo analgesics ASA/APAP/caffeine combos
39
MOH criteria
More than 15 days a month Regular overuse for more than 3 months of: Ergotamine, Tristan's, opioids or combo for more than 10 days, or simple analgesics, opioids/ergotamine/triptan combinations greater than 15 days a month Headache markedly worsened during medication overuse
40
MOH treatment
Weaning of analgesic overused Preventive therapy Avoid abrupt discontinuation
41
Neuralgia
Neuropathic pain that is paroxysmal, breif, shock like or lightning like Follows peripheral or cranial nerve distribution No neuro deficit Nonpainful stimuli can provoke it Refractory period following the attack
42
Trigeminal neuralgia
>50 years old, women Vascular compression of trigeminal nerve root
43
TN definition
Paroxysmal attacks of intense, sharp, superficial stabbing pain in the distribution of one or more branches of the trigeminal nerve, usually unilateral Facial muscle spasms One-several seconds and repetitive Refractory or continuous dull pain
44
TN triggers
``` Lightly touching trigger zone Chewing Talking Brushing teeth Cold air Smiling Grimacing ```
45
TN diagnostics
CT, MRI, MRA MRI if trigeminal sensory loss, bilateral, <40 years old Electrophysiologic trigeminal reflex testing Jaw jerk reflex
46
TN prognosis
6-12 months may remit 25-50% stop responding to drug therapy 90% pain free immediately or soon after surgery
47
Atypical facial pain
Depressed middle aged women Constant, often burning pain Restricted distribution, then spreads to face on affected side and sometimes to the opposite side, neck or back of the head
48
Glossopharyngeal neuralgia
Pain similar to TN Throat, sometimes deep in ear and back of tongue Accompanied by syncope sometimes
49
Glossopharyngeal neuralgia triggers
Swallowing Chewing Talking Yawning MS?
50
Postherpetic neuralgia
Pain beyond 4 months from initial onset of herpes zoster 15% of patients with hx of shingles develop this
51
PHN incidence increases with...
Advanced age Greater acute pain Greater rash severity V1 trigeminal nerve involvement
52
Neurons vs. neuroglia
Neurons area typical cells within nervous system, info transmitters Neuroglia are metabolic, immunologic and structural support
53
Microglia
Activated with immune system and brain injury, clean up cells
54
Oligodendrocytes and Schwann cells
Oligodendrocytes- myelinate in the CNS Schwann cells- myelinate in the PNS
55
Astroctye
Act on blood vessels, are the blood brain barrier
56
Ependymal cells
Line the ventricles of the brain, produce CSF
57
Afferent nerve
Arrive in the CNS Sensation, sensory nerves
58
Efferent nerves
Exit the CNS, motor nerves
59
Ascending versus descending tracts
Ascending deliver info to the brain, sensory Descending deliver info to the periphery, they are motor
60
If the tract begins with spino...
It is sensory, starts in the spine and goes to the brain
61
If the tract ends in spinal...
Motor, starts in the brain and ends up in the spine
62
Posterior column tract is...
A sensory tract
63
Corticobulbar tract
Motor Facial expressions, voluntary movement
64
Extrapyrimadal tracts and symptoms
Info from brainstem to involuntary parts of spine Dystonia Akathisia Parkinsonism Tardive dyskinesia
65
Upper motor neuron lesion
Problem with the pyramidal tracts Loss of the upper motor inhibition allows for exaggerated motor stretch reflex of the lower motor unit
66
UMN lesion presentation
``` Spastic paralysis Hyperreflexia Babinski Clonus Clasped knife reflex ```
67
LMN lesion presentation
Flaccid paralysis Atrophy Fasciculations/fibrillations Hyporeflexia
68
LMN lesion examples
``` Spinal cord injury/nerve root compression Motor neuron disease Peripheral neuropathy Diabetic neuropathy Poliomyelitis ```
69
UMN lesion example
Cerebrovascular accident (stroke) Cervical or thoracic spine injury of the cord Intracranial tumor
70
Spasticity
Velocity dependent increase in tonic stretch reflex Results from abnormal input from dorsal/posterior horn causing the UMN lesion symptoms
71
Treatment of spasticity
Botox injection Dorsal rhizotomy
72
ASIA impairment scale
5 grades from complete, sensory, motor, normal
73
Glad standard for spinal cord injury
MRI
74
Spine fx concerning for SCI
Posterior element disruption Compression fx with posterior wall of vertebral body displaced into spinal canal Dens fx
75
SCIWORA
Spinal cord injury without radiographic abnormality Usually cervical spine Rare, usually kids under the age of 8 30 min-4 day latent period
76
Syndromes
5 types, most common in central cord
77
Treatment of SCI
Stabilize spine and immobilize High dose IV methylprednisone WITHIN 8 HOURS OF INJURY
78
Sympathetic autonomic dysreflexia
Sweating | Increased BP
79
Parasympathetic autonomic dysreflexia
Bradycardia
80
Autonomic dysreflexia
Occurs with spinal cord lesions above T6 After spinal shock has resolved, around 6 months More common in quadriplegia
81
Syringomyelia
Cyst in the spinal cord, destroying a portion of the cord from the inside out
82
Chiari malformation
Congenital condition, anatomic defect in skull base Varying degrees of cerebellum and brainstem herniation through foramen magnum
83
findings in chiari
Pain, numbness, gait ataxia, HA, weakness Burns from loss of pain sensation, hyperhidrosis, glossy skin, pallor coolness Charcot joints Long tract signs IMPAIRED PAIN AND TEMP WITH PRESERVATION OF LIGHT TOUGH
84
Delirium
Disorientation, bewilderment, difficulty following commands
85
Lethargy
Mild drowsiness, aroused by moderate stimuli then drift back to sleep
86
Obtunded
Moderate drowsiness, sleep more than normal, slow response to stimuli
87
Stupor
Severe drowsiness, only vigorous and repeated stimuli arouse the individual
88
Glasgow coma scale
15 is good <8 intubated 3 is coma Eyes, verbal, motor
89
Locked in syndrome
Patient unable to move or speak, but normal cognitive skills
90
A kinetic mutism
Move or speak very slowly, but are alert
91
Psychogenic unresponsiveness
Hold eyes closed and resist opening, normal eye responses Faking a coma
92
Minimally conscious state
Intact awareness but poor response Intact wakefulness
93
Vegetative state
No awareness of self or environment Intact wakefulness
94
Brain death
Irreversible and unresponsive coma Absence of brainstem reflexes Apnea test- cannot sustain appropriate oxygen level
95
Traumatic brain injury
Structural or physiologic disruption of brain function Any period of confusion, disorientation, change in consciousness Neurologic dysfunction
96
Basal skull fracture
Hemotympanum Panda eyes CSF leakage from ear or nose Battle sign (behind ear)
97
Systems effected by dysautonomia
``` Cardiac GI GU Sexual skin ```
98
Postural tachycardia syndrome
Lightheadedness, palpitations, feeling faint >30 bpm increase upon standing, no fall in BP
99
Baroreflex failure
Stress induced systolic BP surges of more than 300, vagus nerve giving no input
100
Familial dysautonomia
Ashkenazi Jewish descent
101
Multi system atrophy
Progressive neurodegenerative disorder with autonomic, extrapyrimidal, cerebellar and pyramidal features Fatal
102
Autonomic neuropathy
Autonomic dysfunction from postganglionic autonomic nerves
103
Neruocardiogenic syncope
Symptomatic, sudden drop in BP with simultaneous bradycardia @ 10 minutes
104
Dysautonomia for tilt table
Continuous drop in BP and no compensatory increase in HR
105
MS epidemiology
Autoimmune, 20-40 peak incidence Most common demyelinating disease
106
Classically... MS
Lesions are separated by space and time Areas of demyelinating arise, cause problems, and slowly resolve
107
MS mechanism of disease
Demyelination occur Microglia activation increases tissue damage Lesions re-myelinate with time
108
Examples of MS symptoms
``` Weakness Numbness Tingling Spasticity Paresthesias Imbalance Optic neuritis Diplopia Urinary issues Pain ```
109
Lhermitte sign
MS Sensation of electric shock radiating down spine or to the limbs with neck movement
110
Uhthoff phenomenon
Worsening of MS symptoms with an increase in temperature
111
Optic neuritis and MS
Acute, monocular vision loss Painful, central
112
Transverse myelitis
Inflammatory demyelination of 1-2 segments of the thoracic spinal cord Weakness pain and sensory disturbance that is rapidly evolving
113
MS diagnosis
Can be clincal MRI to confirm/support
114
CSF in MS
Increase gamma globulin synthesis, mostly IgG Oligoclonal bands seen in 90% of patients even if IgG is normal
115
Tabes dorsalis
Seen in neurosyphilis Sensory ataxia, foot drop, wide based/ataxic gate Lancinating/stabbing pains Argyll Robertson pupils
116
Basal ganglia function
Planning and executing movement (and inhibiting movement when it is not desired)
117
Dopamine from...
The substantia nitro modulates the function of the striatum
118
Parkinsonism is one or more of these signs:
Tremor Rigidity Bradykinesia Postural instability **PD is the most common cause
119
Patho of Parkinson's disease
Degernation of dopamine producing neurons in substantia nigra 60-80% of these neurons are lost before the onset of symptoms Lewy bodies- eosinophilic inclusions created
120
Parkinson's tremor
At rest Stops or decreases with purposeful movement Pill rolling
121
Parkinson's bradykinesia
Gait changes, like shuffling or festination (quick short steps) Micrographia
122
Parkinson's rigidity
Cogwheel from numerous hitches Clasp knife contractions
123
Other PD symptoms
``` Depression Sleep disturbance Autonomic dysfunction Dementia Seborrheic dermatitis Masked facies/lack of expression ```
124
Huntington disease general
Autosomal dominant Trinucleotide repeat on chromosome 4 30-50 year old onset
125
Patho of Huntington
Atrophy of the striatum 9-20 years before onset of sx
126
Huntington movement changes
Fidgety early Then chorea, dystonia, rigidity
127
Cognitive changes in Huntington
Irritable Antisocial Psychiatric issues Dementia
128
Huntington diagnosis
Genetic testing
129
Benign essential tremor
Occurs at any age, worsened by stress, nicotine and caffeine Rhythmic movements, only occurs with movement
130
Benign essential tremor traetment
Not necessary, but deep brain stimulation showed promise
131
Restless leg syndrome general
Increases with age Irresistible urge to move limbs, intense sensory disturbances like itching burning or crawling Occurs during sleep or inactivity
132
Idiopathic torsion dystonia
Painful, prolonged muscle contractions Often manifest in childhood
133
Examples of idiopathic torsion dystonia
Torticollis Facial grimacing Forced opening and closing of the mouth Posturing Blepharospasm
134
Myoclonus
Spontaneous muscle contractions
135
Seizure definition
Sudden change in cortical electrical activity
136
Epilepsy definition
Any disorder characterized by recurrent unprovoked seizures
137
Primary generalized seizure definition
Widespread electrical activity involving both sides of the brain at once
138
Simple partial seizure
Patient remains conscious, secondary to electrical discharge from one specific area of the brain
139
Complex partial seizure
Electrical discharge from a specific area of the brain, patient loses responsiveness or consciousness
140
Todd's paralysis
Transient neurologic deficit that lasts for hours or rarely days after a seizure
141
Aura in seizures
Can be sensory like sensation Somatosensory like singling Visual like hallucinations/flashing lights Auditory like buzzing, drumming Taste
142
Affective aura
Fear, depression and anger
143
Mnemonic aura
Memory phenomena like deja vu
144
Absence seizure
Impairment of awareness and responsiveness Abrupt onset, often begin in childhood Generalized seizure
145
Atomic seizure
Abrupt loss of muscle tone, loss of control of muscles Most commonly fall forward
146
Myoclonic seizure
Single or multiple myoclonic jerks lasting less than 1 second, clustered in a few minutes
147
Tonic clonic seizures
Sudden loss of consciousness Tonic phase- rigid and drop to ground, falling backward, sudden LOC Clonic- jerking of musculature Posictal- abnormal behavior, no memory of event, headache, confusion, fatigue
148
Epilepsy diagnosis
At least 2 unprovoked seizures, occuring greater than 24 hours apart May treat after 1 seizure if it is likely that they will have another If they have had no seizures in 10 years and off meds for 5, no more diagnosis
149
A normal EEG...
Does NOT rule out an epilepsy or seizure disorder
150
Status epilepticus
Emergency! Single seizure of >30 minutes
151
TIA definition
Transient episode of neuro dysfunction Ischemic without acute infarction **warning sign for true ischemic stroke
152
TIAs occur most often in the ...
48 hours prior to the stroke More likely to follow a carotid TIA
153
Large artery low flow TIA
Stenotic atherosclerotic lesions Often at internal carotid artery
154
Lacunar or small penetrating vessel TIA
Middle cerebral artery stem, basilar or vertebral artery, circle of Willis
155
Low flow TIA presentation
Short lived, minutes Several times per year-day Marked symptoms dependent on artery affected
156
Embolic TIA presentation
Hours Infrequent Anterior or posterior cerebral circulation dependent
157
Lacunar presentation TIA
Brief Repetitive Stepwise and progressive rather than abrupt Pure motor or pure sensory symptoms
158
TIA imaging
Within 24 hours of symptom onset! Diffusion weighted brain MRI is preferred
159
Neurovascular imaging for TIA
Carotid duplex US CTA or MRA Gold standard: cerebral arteriography
160
When TIA is caused by embolism, get a...
EKG asap!
161
ABCD score for high risk stroke post-TIA
>60 1 point >140/90 1 point Unilateral weakness 2 points Isolated speech disturbance 1 point More than 60 min 2 points 10-59 minutes 1 point Diabetes 1 point
162
Hospitalization for TIA
TIA AND have ABCD score of greater than 3, OR 0-2 score plus Uncertainty that workup can be completed outpatient or focal ischemia
163
Carotid circulation supplies...
The anterior and middle of the brain
164
Vertebrobasilar circulation provides...
To the posterior part of the brain
165
Anormalities of language
Anterior circulation/middle cerebral artery
166
Vertigo, ataxia, bilateral deficits
Posterior circulation
167
Pure motor or pure sensory deficits
Lacunar
168
Ischemic stroke imaging
Noncontrast CT initially MRI better for early US of carotid CTA or MRA
169
Lacunar stroke
Small infarcts in the deeper parts of the brain and brainstem caused by the occlusion of a single penetrating branch of a large cerebral artery 1/4 of ischemic strokes Less severe
170
Lipohyalinosis
90-95% of lacunar strokes, small vessel disease from diabetes
171
Lacunar infarct presenation
Lack cortical signs Pure motor or pure sensory Clumsy hand dysarthria syndrome Internuclear ophthalmoplegia Abrupt or gradual onset, progress
172
CT and diffusion weighted MRI findings for lacunar infarct
Ct- small punched out hypodense areas MRI- small, punched out CSF filled spaces
173
MCA infarct
Largest proportion of the brain, severe stroke Usually embolism
174
MCA infarct findings
Contralateral hemiplegia/hemisensory loss Contralateral homonymous hemianopsia Eye deviation to side of lesion Anosognosia Aphasia, wernicke or broca
175
Wernicke aphasia
Posterior branch, defect in understanding what others are saying
176
Broca aphasia
Anterior branch, motor defect, cant express what they are thinking
177
ACA infarct
Usually embolism
178
ACA infarction presentation
Contralateral findings of leg weakness and sensory loss, grasp reflex Paratonic rigidity- resisted movement Abulia- no motivation Frank confusion Urinary incontinence
179
Ophthalmic artery infarct
Asymptomatic in most Transient emblic obstruction- amaurosis fugax
180
Posterior cerebral artery infarct
Around 5% Contralateral strength and sensory deficits are MILD Contralateral homonymous hemianopsia Color anomia (can't name them) Visual agnosia (can't name objects)
181
Lateral medullary syndrome
Posterior inferior cerebellar artery occlusion Facial weakness, Horner syndrome, ataxia/incoordination IPSILATERAL Sensory loss in trunk and limbs CONTRALATERAL
182
Thalamic pain syndrome
Stoke within posterior circulation Chronic pain on the same side of the stroke Could also have intention tremor and hand spasm
183
Vertebrobasilar infarct
``` Vertigo Vertical nystagmus Contralateral hemiplegia and sensory deficit Ipsilateral CN palsy Ipsilateral ataxia ```
184
Cerebellar stroke
``` Vertigo, dizziness NV Nystagmus Ipsilateral limb ataxia Contralateral sensory loss in limbs ``` Coma, brain herniation and death!
185
Door to stroke unit admission
Less than 3 hours!
186
Avoid lowering BP in the first...
2 weeks after CVA After 2 weeks can be reduced to <140/90
187
Intracerebral hemorrhage
Usually from HTN Spontaneous, nontraumatic sudden onset Putamen is the most common site If amyloid of blood- lobar/cortex most common
188
Intracerebral hemorrhage presentation
Loss or altered level of consciousness Vomiting Headache May terminate fatally within 2 days
189
Intracerebral hemorrhage imaging
CT without contrast CTA MRA later when stable
190
What should you not do with intracerebral hemorrhage?
Lumbar puncture! Monitor ICP
191
ICH treatment
Mechanical ventilation Treat HTN Control fever FFP if blood dyspraxia
192
ICH score and mortality rates
``` 1- 13% 2- 26% 3- 72% 4- 97% 5- 100% ```
193
Subarachnoid hemorrhage
Rupture of aneurysm or arteriovenous malformation Worst headache of my life, thunderclap Meningismus
194
Mycotic aneurysms
Septic emboli more distal vessels Often at cortical surface
195
AVM
Congenital vascular malformation Smaller ones are more likely to bleed! Usually supratentorial
196
Imaging in SAH
CT is positive in 85% LP positive in 95% Cerebral angiogram is the gold standard!
197
Degenerative motor neuron dz presents as...
Weakness and muscle wasting with sensation intact 30-60 years old
198
Bulbar/pseudobalbar palsy
Degeneration of medulla and CN IX-XII nuclei Bulbar affects the motor nuclei causing dropping palate, decreased gag, pooling secretions, etc Pseudobulbar is fits of laughing and crying, UMN defect
199
ALS general
Upper and lower motor neuron disease ``` In 3 of 4 of these tracts: Bulbar Cervical Thoracic Lumbosacral ```
200
ALS presentation
Asymmetric limb weakness Hand weakness, foot drop
201
Degenerative motor neuron dz diagnosis
Electromyography
202
Myasthenia gravis general
Limit ability to reach action potential threshold in the NMJ from autoantibodies against AchR
203
Myasthenia gravis features
Thymoma in 15% of patients Insidious onset Significant fatigability of muscles, ptosis and diplopia common
204
Myasthenia gravis diagnosis
Tensilon/edrophonium challenge or ice pack challenge Serum AchR antibody testing
205
MG prognosis
Respiratory failure, progressive
206
Lambert eaton syndrome
Defective release of Ach Paraneoplastic in 40% of cases Handgrip to differentiate from MG, LE pateints will get stronger as the squeezes continue
207
Botulism
Canned foods , clostridium botulinum toxin prevents Ach release Resp difficulty and limb weakness later
208
Organophosphate poisoning
Pesticides, buildup of Ach Parasympathetic overload (SLUDGE/BBB)
209
Organophosphate poisoning treatment
Wash with soap, aspirate gastric contents Atropine reverses parasympathetic sx 2-PAM reverses binding to AchE if quickly given
210
Myotonic dystrophy
Autosomal dominant Muscle stiffness and delayed relaxation
211
Duchenne muscular dystrophy general
All males Absent dystrophin protein from muscle fiber plasma membrane CK elevations Degenerative and regenerative changes DNA probe
212
DMD presentation
2-3 years of age Slow to attain motor milestones Calf hypertrophy and proximal leg weakness Gower sign- climbing up legs and body to stand
213
Charcot Marie tooth disease
Autosomal dominant Progressive polyneuropathy with weakness and muscle wasting
214
Charcot Marie tooth disease presentation
Foot drop, foot deformities, early gait disturbance
215
Guillan barre general
Campylobacter jejuni enteritis, demyelination 2-3 weeks post insult Ascending weakness and paralysis Can involve respiratory muscles! Protein in CSF
216
Mononeuritis multiplex
Nerve infarcts Multiple nerve trunks Painful, asymmetrical and sensory!
217
Saturday night palsy
Compressive, paralysis of distal limb Sleeping with arm over back of chair Improves over a few months
218
Bell's palsy
LMN facial nerve paresis Abrupt onset, can be preceded by ear pain and taste disturbance Steroids Most have full recovery
219
CVA versus bell's
If you cannot elevate 1 eyebrow, its a facial nerve issue. Bell's!
220
General dementia considerations
Gradual and progressive Previously normal cognition Risk doubles every 5 years after 50 yeras old
221
Dementia signs and symptoms
Short term memory loss(hippocampal damage) Aphasia (wernickes) Visuospatial dysfunction Apraxia (loss of learned behaviors)
222
With dementia presentation you must rule out...
Reversible causes!! Not a normal part of aging
223
Dementia testing
Mini cog- 3 word recall, clock drawing MMSE, Montreal
224
Testing for dementi
MRI indicated for progressive decline in cognition Rules out other CNS dz
225
Alzheimer's disease general
Most common cause of dementia Very rare if younger than 60 Different proteins, Ach levels drop
226
AD presentation
Memory impairment- immediate memory preserved early on, then lose recent memory first Visuospatial skills lost early Personality changes
227
AD diagnosis
Clinical Genetic testing Analysis of brain tissue
228
Vascular dementia
2nd most common dementia Multiple infarcts, like lacunar
229
Frontotemporal dementia
Focal degeneration of frontal and or temporal lobes Changes in personality social behavior or language
230
Kluver bucy sydrome
Emotional blunting Hyperphagia Visual agnosia In frontotempral dementia
231
Lewy body dementia general
2nd most common cause of degenerative dementia Lewy bodies are intraneuronal protein tangles, seen in Parkinson's and LBD
232
LBD presentation
Hallucinations Parkinsonism Cognitive fluctuations Antipsychotic sensitivity
233
Normal pressure hydrocephalus
Cause of dementia symptoms Impaired absorption of CSF Wet, wacky and wild Magnetic gait, apathy, incontinence
234
Prion disease
Misfolding prion proteins causing neurodegenerative disease Difficult to sterilize! Fatal in 1 year
235
CJD
Mad cow disease Rapidly progressive dementia is the main sign
236
Meningitis general
Inflammatory dz of the meninges and CSF Rapid clincal progression
237
Big causes of meningitis
Strep pneumo Neisseria meningitidis *listeria in adults over 50 and neonates or those with immunodeficiency
238
Meningitis presentation
Fever Stiffness altered mental status headache
239
Austrian triad
Pneumonia, meningitis and endocarditis from strep pneumo
240
Meningococcal rash
Fever and rash, if it doesn't blanch its meningitis Could lead to Waterhouse Frederick son syndrome
241
Less common meningitis pathogens
H flu in kids Staph Aureus and gram negative in nosocomial and health care settings
242
Neonatal meningitis
More common in the first month than any other time of life Always LP!
243
Aseptic meningitis
Signs and symptoms of meningitis but negative cultures, need a LP Self limited
244
Causes of aseptic meningitis
``` Enterovirus HIV HSV Syphilis- later onset Tb ```
245
Encephalitis
Impaired cerebral function Viral often times AMS, seizures, neuro deficits
246
West Nile
Mosquitoes Flaccid paralysis, maculopapular rash, tremor
247
Primary intracranial tumor types
Gliomas are half of all brain tumors, from glial cells Then meningiomas
248
Symptoms of primary intracranial tumors
``` Progressive CNS deficits Headache Personality changes Malaise Seizures Brain herniation ```
249
Frontal lobe signs
Intellectual decline, mental activity slowing, personality changes, grasp reflexes
250
Temporal lobe symptoms
Seizures, hallucinations, motor phenomena, depersonalization
251
Parietal lobe symptoms
Contralateral disturbances of sensation, asterognosis, depressing apraxia, loss of tactile discrimination
252
Occipital lobe changes
Crossed homonymous hemianopia or partial field defect, visual agnosia, color anomia, visual hallucinations
253
Brainstem and cerebellum symptoms
CN palsies, ataxia, incoordination, nystagmus
254
Intracranial tumor imaging
MRI with contrast!
255
Most common source of intracranial metastasis is carcinoma of...
The lung
256
Most cerebral metastasis are located...
Supratentorial
257
Leptomeningeal metastases
Cancer of the pia and arachnoid mater
258
Leptomeningeal metastases causes neuro deficits by..
Infiltration of cranial and spinal nerve roots Invasion of brain or spinal cord Hydrocephalus
259
Leptomeningeal mets diagnosis
CSF confirms: increase pressure, pleocytosis, increased protein and decreased glucose
260
Most common spinal tumor is...
Extradural Then intradural/extramedullary
261
Signs/symptoms of spinal tumor
Focal pain aggravated by valsalva Radicular pain Motor and sensory deficits Sphincter disturbances
262
Brain abscess general
Within brain parenchyma, from infection, trauma or surgery Bacteria is the most common pathogen
263
Brain abscess symptoms
Dx after 14 days of symptoms Headache usually, fever in half, AMS, seizures, CN deficits
264
Imaging of brain abscess
CT/MRI with contrast Earlier recognition with MRI
265
How big can an abscess be before it needs to be drained?
Less than 2 cm and can be treated medically
266
Modified dandy criteria
To dx idiopathic intracranial hypertension Signs and symptoms of ICP No neuro abnormalities Normal CSF Normal imaging
267
IIH symptoms
Headache, lateralized, throbbing, ass't with NV Diplopia, photopsia, loss of vision Pulsatile tinnitus Changes precipitated by standing and valsalva
268
3 hallmark signs of IIH
Papilledema Visual field loss 6th nerve palsy