Neurology Flashcards
1
Q
Frontal lobe function
A
Planning and execution of movement
speech
smell
problem solving
PPSS
2
Q
Parietal lobe function
A
Sensory input - touch
pressure
Body orientation
3
Q
Temporal lobe function
A
Understanding language
Behavior
Hearing
Memory
4
Q
Occipital lobe function
A
Vision
Color perception
5
Q
Cerebellum function
A
Balance
Coordination of voluntary movement
Fine muscle control
6
Q
Brain stem function
A
Breathing
Temperature
Digestion
Alertness/sleep
Swallowing
7
Q
Hippocampus function
A
Memory
8
Q
Amygdala function
A
Emotions
9
Q
Thalamus function
A
Relay signals from lower brain to cortex
10
Q
Basal ganglia function
A
Sorting, evaluating and executing motor functions, filtering out unwanted movement
11
Q
parts of basal ganglia
A
caudate nucleus
putamen
globus pallidus
subthalamic nucleus
substantia nigra.
12
Q
grey matter content
A
neuronal cell bodies
13
Q
white matter content
A
neuronal axons forming tracts
14
Q
what does the sympathetic nervous system do to the eye?
A
Causes pupillary dilation - Mydriasis by the pupillary dilator muscle
15
Q
Innervation of the pupillary dilator muscle
A
Postganglionic sympathetic fibers project from the superior cervical ganglion.
Fiber travel with the ophthalmic artery, forming a number of long ciliary nerves that supply the dilator pupillae muscle
16
Q
what does parasympathetic nervous system do to the eye
A
Cause pupillary constriction by sphincter pupillae muscle - Miosis
17
Q
innervation of pupillary sphincter muscle
A
Receives parasympathetic innervation via the short ciliary nerves.
Fibers originate from the Edinger-Westphal nucleus of occulumotor cranial nerve III.
18
Q
Pupillary accommodation
A
Contraction of ciliary muscle: close/near vision
Relaxation of ciliary muscle: far vision
19
Q
innervation of cilliary muscle
A
These parasympathetic fibers arise from cranial nerve V, also known as the nasociliary nerve of the trigeminal.
20
Q
Areas of lesion causing effect on pupil
A
Hypothalamus controls - PSNS - constriction
Paravertebral SNS - dilation
Neck - fibers run with ICA
Trauma to orbit
21
Q
name of pupillary constriction and dilation
A
dilation: Mydriasis
constriction: miosis
22
Q
name of unequal size of pupils
A
Anisocoria
23
Q
part of pupil is missing name
A
coloboma
24
Q
describe the light pupil reflex
A
- light in right eye
- AP in pretectal nuclei
- signal from pretectal to EW nuclei - AP
- EW generate AP through the CN III
- Oculomotors nerve causes miosis
25
causes of mydriasis
Adie's pupil
CN 3 lesion
Drugs
Migraine
26
seen in complete CN 3 lesion
mydriasis
ptosis
impaired eye movement
loss pupil reflex
27
drugs causing mydriases
Anticholinergics
Antihistamins
oral contraceptives
TCA
NSAIDS
28
causes of miosis
Horners syndrom
Argyll Robertson pupil
Drugs
29
content of facial nerve?
Motor fibers
Taste fibers - visceral afferent
Parasympathetic - visceral efferent
30
path of facial nerve motor nerve
from lateral brain
cerebellopontine angle
internal auditory meatus
facial canal
exit through stylomastoid foramen
31
path of parasympathetic fibers of facial nerve
With facial nerve into internal auditory meatus
32
what part of tongue is innervated by the facial nerve?
anterior 2/3
33
what is bells palsy
acute paralysis of the face related to a inflammation or swelling. usually unilateral.
34
etiology of bells palsy
idiopathic
Herpes zoster (Ramsay Hunt syndrome)
HSV reactivation
Borreliosis (Lyme disease)
Diabetes mellitus
35
what is bells phenomenon
attempt to close eyes and show teeth one eye can't close and eyeball roles back
36
treat bells palsy
protect eye when sleeping + eyedrops
high dose prednisolone 10 days
antiviral therapy if known virus
37
Ramsay hunt syndrom
herpes zoster infection affecting the geniculate ganglion sudden severe pain, eruption of vesicle in external ear
deafness may be an outcome if CN VIII involvement
38
hemifacial spasm
- unilateral clonic spasms from orbicularis occuli then to rest of face
- contractions are irregular and increases with emotional stress and fatigue
- cause: vascular comprssion of facial nerve at root entry zone like tumor
39
vertigo dizziness
loss of orientation of body in space
leads
to feeling that Room is spinning
40
causes of Central vertigo
■ Tumor (astrocytoma)
■ Cerebrovascular disorders
● Stroke
● Vertebrobasilar insufficiency
● TIA
■ Migrainous vertigo
■ Drugs/toxins
■ Multiple sclerosis (demyelination)
■ Inflammation (meningitis, cerebellar abscess)
■ Trauma
41
etiology of peripheral vertigo (85%)
■ Idiopathic
■ Menière’s
■ BPPV (benign paroxysmal positional vertigo)
■ Trauma
■ Drugs: streptomycin, quinine, salicylates
■ Labyrinthitis
■ Vestibular neuronitis
■ Cerebellopontine angle tumors
● Acoustic neuroma
● Meningioma
42
non-vertigo dizziness
Psychogenic (diagnosis of exclusion)
■ Depression
■ Anxiety etc
Vascular
■ Orthostatic hypotension
■ Arrhythmia
■ CHF
■ Aortic stenosis
■ Vagovagal episodes
Ocular
■ Decreased visual acuity
43
nystagmus in central vs peripheral nystagmus
central: Bidirectional horizontal or vertical
Peripheral: Unidirectional horizontal or rotatory
44
6 things you compare central vs peripheral
| vertigo
imbalance
nausea vomiting
auditory symp
neurological sympt (diplopia, headache, dysphagia)
compensation (rapid in peripheral)
nystagmus
45
Ménière disease is a
Ménière disease is a disorder
caused by build of fluid in the chambers in the inner ear.
It causes symptoms such as
* vertigo,
* nausea, vomiting,
* loss of hearing,
* ringing in the ears,
* headache,
* loss of balance
* , and sweating.
46
to differentiate vertigo from dizziness
vertigo: WORLD IS SPINNING
dizziness: unsteady or lightheaded
47
Plegia vs paresis
plegi: complete loss
paresis: loss of power
48
Different pareses/plegias
Hemiplegia/hemiparesis: unilateral weakness of the limbs
Monoplegia/monoparesis: weakness of one limb,
Paraplegia/paraparesis: weakness of both lower limbs,
Diplegia/diparesis: weakness of both upper limbs,
Quadriplegia/tetraplegia or quadriparesis/tetraparesis: weakness of all four limbs,
49
Lesion of the corona radiata and the internal capsule causes
contralateral severe spastic hemiparesis
with involvement of the lower part of the face and the tongue
50
Isolated lesion of the corticospinal tract in the cerebral peduncle and lesion of the pyramid in the medulla cause
flaccid weakness,
however the joint lesion of all descending tracts leads to spastic hemiparesis.
51
Unilateral lesion of the base of the pons causes
contralateral hemiplegia/paresis, often sparing the face
52
Bilateral lesion of the base of the pons causes
quadriparesis/plegia.
53
Unilateral lesion of the cervical spinal cord at the level of C1-4 segments causes
ipsilateral spastic hemiparesis.
54
Bilateral lesion of the cervical spinal cord at the level of C1-4 segments causes
spastic quadriparesis
55
Lesion of the cervical spinal cord at the level of C5-Th1 segments causes
flaccid weakness of the upper limbs
spastic weakness of the lower limbs
56
Lesion of the thoracic spinal cord causes
spastic paraparesis
57
Lesion of the lumbosacral spinal cord causes
flaccid paraparesis.
58
Lesion of spinal motor neurons and anterior roots causes
flaccid weakness of segmental
distribution (in the corresponding myotomes)
59
Polyneuropathy typically causes
distal symmetrical flaccid weakness of the limbs, first on the lower limbs.
60
most important symptom of UMNL and LMNL
UMNL: BABINSKY - positive pyramidal sign
LMNL: visible fasciculation's
61
lateral corticospinal tract is for
distal limbs and fine manipulation
62
anterior/ventral corticospinal tract is for
trunk and upper leg muscles (posture locomotor)
63
UMNL paralysis:
LMNL paralysis:
UMNL paralysis: spastic
LMNL paralysis: flaccid
64
spasmicity vs rigidity
Spasticity: resistance in one direction. Velocity-dependent.
Rigidity: resistance in all directions. Not velocity-dependent
65
fasciculation vs fibrillations
○ Fasciculations: Visible twitching of motor unit of muscle due to spontaneous firing of
action potentials from damaged nerve
○ Fibrillations: Non-visible twitching of individual muscle fibers when nerve is even more
damaged - only seen on electromyogram
66
main cause of UMNL
stroke
demyelination
ALS(Amyotrophic lateral sclerosis )
67
main cause of LMNL
polio
west nail virus
spinomuscular athropy (SMA)
cauda eq. syndrom
DM neuropathy
botulism
ALS
68
what are the two fibers innervated in a muscle
Intrafusal Gamma fibers: reflex
Extrafusal alpha fibers contraction
69
where does the corticospinal tract deccusate
in the pyramids (brainstem)
70
what is the cerebrobulbar tract?
goes from cortex to pons/medulla and innervate CN
71
destination of corticobulbar tract
CN 5
CN 7
Nucleus ambiguous: CN 9, 10, 11
CN 12
72
why is there atrophy in UMNL
loss of usage
loss of Ach release causing no AP or protein synthesis,
80% muscle loss
73
pathophysiology of fasciculations?
no Ach causes increase in R formation and increased mechanical sensitivity - tapping causes activation of receptors opening Na channels and AP
74
why is there spasmic paralysis in UMNL
because of less inhibitory signals from medullary reticulospinal tract causing hyperflexia and hypertonia
75
which side is the speech scenter on?
LEFT hemisphere is dominant in most people
76
what is aphasia
Aphasia is a speech disorder with an inability to comprehend and/or formulate language. It is caused by
lesions of cortical speech centers and their connections
77
Broca's and Werncks aphasia is?
Broca: Expressive
Wernicks: receptive
78
Areas in Brocas
BA 44
BA 45
79
areas in wernicks
WA 22
WA 39
WA 40
80
BA aphasia present?
know what do say but cant say it
NON FLUENT
(understand but can't find words)
skjønner men finner ikke ord
81
WA aphasia present?
difficulty understanding and finding right words, mix up words
snakker om helt rare ting
FLEUNCY INTACT
problem with comprehension and repetion
82
causes of aphasi
stroke
trauma
brain mass
neurodegenerativ disorders
encephalitis
83
conduction aphasia
understands and speaks but cant repeat
84
what supplies speech area?
middle cerebral artery
85
which artery infarct causes wernicks aphasia?
BRANCH OF MIDDLE CEREBRAL ARTERY: posterior temporal artery
86
etiology of conduction aphasia
Lesion of the arcuate fasciculus.
Characterized by normal speech,
but impaired repetition.
Patient is aware and frustrated by this.
87
alexia without agraphia
Lesion in **left occipital lobe** usually due to **infarct of the posterior cerebral artery**.
The visual information cannot reach the language areas, and the patient is unable to read, but are able to
write (pure word blindness)
alexia=inability to read or comprehend written language
agraphia= loss of a previous ability to write
88
how can you find out which side language center is on?
dominant hand: right hand - left area
left hand: mix of both 70% left 15% right 15% bilateral
89
what connect BA and WE
arcuate nucleus
90
The anatomical basis of arousal is the
Ascending reticular activating system (ARAS),
composed of the pontomesencephalic reticular formation, monoaminergic networks of the diencephalon and the intralaminar and medial nuclei of the thalamus
91
Disorder of arousal
● Somnolence - Mildest form. Awakens with
verbal stimuli, but is asleep without stimuli.
● Stupor - Patient may open eyes to painful stimuli, verbal is not enough. Slow and inappropriate reaction to stimuli.
● Coma - Cannot be awakened. Abnormal posture (decorticate or decerebrate).
i. Coma I - Preserved brainstem reflexes
ii. Coma II - Lost brainstem reflexes
92
disorders of awakefullness
akinetic mutism (decorticate state)
confusion
delirium
Persistent vegetativ state
locked in syndrom (not disorder of conciousness but often misdiagnosed as this)
93
define persistant vegetativ state
Awake but no awareness of their surroundings.
Rostral brainstem remains intact → Thermoregulation (hypothalamus), sleep-wake cycle, endocrine system, cardiorespiratory and other visceral functions are intact
94
when is a vegetativ state permanent?
> 30 days
95
decorticated position
stiff with bent arms, clenched fists, and legs held out straight. Sign of severe damage in the brain
lesion above RN in proximal brainstem; loss of the inhibition of RN from higher brain centers > RST causes flexion of UE
also loss of inhibition of VN causes extension of LE
| betterprognosis than decerebrate
96
decerebrated position
all limbs are stiff en extended.
Results from injury at **distal brain stem** or **pons** lesion ( at level or below RN; flexors not working)
leads to extension of all four limbs and opisthotonos (spasm of the muscles causing backward arching of the head, neck, and spine, as in severe tetanus)
overactive VST
97
causes of vegetativ state
○ Extensive white matter damage
○ Bilateral damage to the thalamus
○ Extensive functional or structural impairment of cerebral cortex
- global cerebral ischemia
- hypoglycemia
- renal/hepatic failure
- post-convulsive state
- Wernicke’s encephalopathy
- final stages of cortical dementias)
98
define akinetic mutism
- Awake, but mute and does not move.
99
Etiology of akinetic mutism
Caused by **bilateral interruption** of connections between the **supplementary motor area,
cingular region** and
**midline nuclei of the thalamus**
100
are you paralised in akinetic mutism
NO, because of withdrawal reflex to pain, suckling reflex, and grasp reflex
101
what can cause akinetic mutism
○ Jet bleeding (ruptured Ant. communicating a. aneurism)
○ Bilateral ischemia in the anterior cerebral artery
○ Subfalcial/cingulate herniation
○ Occlusive hydrocephalus
○ Butterfly tumors growing across the corpus callosum
○ Tumors of third ventricle
102
define delirium
Cannot focus, change or fix attention.
Disoriented and incoherent thinking.
evolves over 24h.
inversion of sleep wake cycle. sweating, tachycardia unstable BP
103
what do you have to have to be counscious`
awareness
arousal
alertness
memory
AAAM
104
function of ascending reticuloactivating system (ARAS)
reflexes
sensory information
respiration
HR/BP
sleep cycle
consciousness
posture
105
composition of ARAS system?
medial column
lateral column
median column
composed of A LOT of nuclei
106
4 things to look for first in an unconscious patient
looks like sleeping?
spontaneous movement?
respons to voice/stimuli?
respiration stable?
107
how do you examine a uncounscious patient?
1. observe
2. stabile ABC
3. GCS
4. signs of external Trauma
5. eye position and reflexes
6. limb position and reflexes
7. deep tendon reflex
108
GCS values
< 3 high rate of death
< 8 coma --> intubate
15 is normal
109
deep tendon reflexes?
There are five primary deep tendon reflexes:
biceps, brachioradialis,
triceps, patellar, and ankle.
110
brain stem reflexes
Pupillary Light Reflex.
Corneal Reflex
Oculo-vestibular Reflex
Pain Stimulus.
Gag Reflex.
Cough Reflex.
111
findings in an unconscious patient and their cause?
ocular symptoms:
1. Pupillary constriction (light reactions are preserved!)
2. Pupillary dilation (NO pupillary light reactions)
3. Fixed, moderately dilated pupils
Gaze disorders
4. Skew deviation (eyes diverge in vertical direction, one down, the other up)
5. Persistent upward deviation
6. Persistent downward deviation
1. Bilateral → hypothalamus and diencephalon damage. Unilateral: Horner's
2. tectum lesions
3. lesion below tectum
4. caudal part of brainstem and meso-diencephalon damage
5. global cerebral ischemia
6. hepatic coma
112
what are the two vestibular reflexes
1. Vestibulo–ocular reflex: move head side to side, pupils should move in opposite direction.
2. caloric reflex: cold/warm water stimulus COWS, a test of vestibuloocular reflex
113
what type of unconsciousness is normally related to metabolic origin?
persistent vegetativ state also called unresponsive awakefullness syndrom
114
metabolic causes of unconsciousness
● Deficiency of essential substrates (glucose, oxygen, vitamin B12)
● Exogenous toxins (eg. drugs, heavy metals, solvents)
● Endogenous toxins/systemic metabolic diseases (eg. uremia, hepatic encephalopathy, electrolyte
imbalances, thyroid storm
115
how can you diff between structural defect or coma due to hypoglycemia?
pupillary reflex normally intact if hypoglycemia
116
what glucose level causes coma?
< 0.6 mmol/L
117
what is seen in hyperglycemia
hyperosmolar effect can cause coma. Can cause involuntary movements, seizures and hemiparesis
118
symptomes in ketoacidosis
Symptoms: dehydration (due to osmotic diuresis), fatigue, weakness, headache, abdominal pain,
Kussmal breathing, confusion, coma
119
when is consciousness lost if pO2 drops
< 40 mmHg
120
pupils in hypoxia?
large and reactive
121
# 9. Unconsciousness due to metabolic origin
what happens in renal/uremic encephalopathy`
false neurotransmitters in brain like Octopamine.
imbalance between excitatory and inhibitory
EEG shows slow waves
A sign of acute renal failure
● Brain amino acid metabolism is also impaired, and causes an imbalance between excitatory and
inhibitory neurotransmitters or accumulation of false neurotransmitters
122
symptoms hepatic encephalopathy
pupils small-reactive
Asterixis,
myoclonus, dysarthria, ataxia,
hyperreflexia,
hemiparesis
123
define
Myoclonus
Dysarthria
ataxia
Myoclonus: uncontrolled jerking
Dysarthria: speech muscle weakness
ataxia: loss of limb muscle control
124
glucose level in hyperosmolar hyperglycemia`
VERY VERY high - monitor cant read > 600 mg/d
(>33.3 mmol/L)
125
treatment of HHS
0.9% saline then 0.45%
change to dextrose when glucose is 250 mg/d (13.9 mmol/L)
give regular insulin IV OBS K+ must be > 3.3
126
what electrolyte disturbance causes coma
Hyper Na / Hypo Na
Hyper Cl /hypo Cl
Hyper Mg
Hypo K+ / Hyper K+
127
drug intoxication causing coma
overdose in suicidal intent of sleeping pills and sedativs
128
areas most sensitiv to hypoxia
basal ganglia and cortex, hippocampus, brainstem
- grey matter more vulnerable than white
129
neurological states causing resp failure
The CNS → Raised ICP - hernia
The PNS → Myasthenia, Guillan-Barré syndrom
130
3 causes of an neurological emergency
1. stroke
2. seizure
3. unconsciousness
131
what are the 3 main components of intracranial volume?
CSF
brain tissue
Blood
if one increase the other must decrease
132
CSF volume
approximately 125 mL to 150 mL
133
normal intracranial pressure
7–15 mm Hg
134
monroe Kellie principle
cerebral bloodflow is the ratio of CAP and CVP (AP/VP) = cerebral perfusion pressure
IC pressure = CVP
so increase in ICP always increase CVP thus decrease perfusion pe
CPP= MAP - ICP (or CVP)
135
what are the two principle ways of decreasing ICP
by decreasing CSF (physiological)
by decreasing blood (not physiological)
136
how can we decrease BF to brain?
Hyperventilation dec. CO2 and causes capillary restriction and decreased CVP
Barbiturate narcosis decreases metabolism need
Hypothermia decreases metabolism need
137
causes of increased ICP
1. Space-occupying lesions (eg. tumor, abscess, hemorrhage) - treat with surgery
2. CSF disorders (occlusive hydrocephalus) - treat with CSF drainage
3. Cerebral edema (vasogenic, cytotoxic, interstitial)
138
clinical presentation of increased ICP
● Progressive headache (one of leading complaints)
● Vomiting
● Papilloedema + blurred vision
● Always global cerebral dysfunction due to global cerebral ischemia
139
treatment of high ICP
● Treat underlying etiology
● Osmodiuretics (mannitol, glycerol)
● Loop diuretics decrease CSF production
● ICU:
○ Controlled hyperventilation
○ Barbiturate narcosis
● Decompressive craniectomy
140
how can you pres CSF out of head yourself?
valsava manuver
141
what are the 4 brain herniations?
1. Cingular/subfalcial
2. central
3. transtentorial
4. transforaminal/tonsillar
142
what happens in Cingular/subfalcial herniation
Cingular gyrus in pressed under the falx.
May compress circumferential branches of anterior
cerebral artery (pericallosal, collosomarginal) if mass
effect is considerable.
143
what happens in central hernia
Compression of diencephalon.
Severity is proportional to lateral or axial shift of diencephalon.
144
what happens in transtentorial hernia
Compression of mesencephalon "Midbrain" in tentorial incisure -
* damaged blood supply of mesencephalon
and
* secondary intraparenchymal bleedings.
145
which artery bleeds in
epidural
subdural
subarachnoid
1. MCA
2. bridging veins
3. intracranial aneurism rupture
146
what to asses on a intracranial injury
○ Period of loss of consciousness: relates to severity of diffuse brain damage
○ Period of post-traumatic amnesia: reflects severity of damage
○ Period of retrograde amnesia
○ Cause and circumstances of the injury: e.g. epilepsy
○ Presence of headache and vomiting: if they persists, IC hematoma must be considered
147
imaging in IC hematoma suspicion
stat CT !!
as soon as possible
148
symptomes indicating specific locations of basal skull fractures
**○ Anterior fossa fracture:**
* CSF rhinorrhea (contains glucose),
* bilateral periorbital hematoma,
* subconjunctival hemorrhage
* Raccoon eyes: subcutaneous hematoma around the eyes
**○ Petrous fracture:**
* bleeding from external auditory meatus or CSF otorrhea,
* subcutaneous hematoma/ bruising over the mastoid (Battle’s sign)
149
management of intracranial hematoma
**Extradural**: horseshoe craniotomy flap
w/ complete evacuation of the hematoma
**Subdural/intracerebral**: question mark flap over temporal and/or frontal areas
w/
subdural/intracerebral evacuation of hematoma and necrotic brain
150
IC bleeding pattern on imaging
Epidural is lens shaped
subdural cresent shaped
151
outcome of acute transvers spinal cord injury
quadriplegia
anesthesia
loss of all function below injury site
152
Brown-Sequard syndrom
* Rare, mainly due to trauma (e.g. gunshot, stabwound)
* Ipsilateral proprioceptive sensory loss
(proprioceptive tract) and weakness (corticospinal tract)
* with contralateral loss of pain and temperature (spinothalamic tract)
153
central spinal cord syndrom presentation
Hyperextension injury
-arm weakness
-sensory deficit (pain,temp)
-motor deficit
Lateral CS stract: cervical+thoracic
White commissure: pain, T, crude touch
anterior grey horn LMNL
154
posterior spinal cord lesion
Dorsal column: gracilis, cuneate: proprioception, vibration, disc. touch
155
anterior spinal cord lesion
anterior 2/3 are involved
autonomic fibers: incontinence
Corticospinal tract: UMNL
Anterior grey horn LMNL
spinothalamic tract: pain, temp, crude touch
only preserved is the dorsal column
complete motor paralysis
loss of pain temp feeling below injury
ASA
156
conus medullaris syndrom
between T12-L1 (BBI)
back pain
Bilat sensory loss
Incontinence
Sensory loss (numbness) in the perianal region and inner thighs (saddle anesthesia) and
loss of bladder control (retention with overflow incontinence)
without leg weakness or diminished stretch reflexes
157
Cauda equina syndrome
belowL2
Lumbosarcal Radicular pain in several dermatomes,
flaccid paralysis of lower limbs with loss of deep tendon reflexes and
overflow incontinence
saddle anesthesia: loss of sensitivity at dermatomes s3-s5
158
jefferson vertebrate fracture
fracture of both arches of C1
stiff neck, pain when moving, no neuro signs
159
Dens vertebrate fracture
C2 fracture
Neck pain radiating to occipital region (worse with neck movements), typically the patient
will hold their head
160
type A C B vertebrate fractures
A: compression = shortening
B: distraction = lengthening
C: Torsonal = rotation (shearing force)
161
symptoms of impaired circulation of ICA
○ Deterioration of consciousness
○ Homonymous hemianopia of the c/l side
○ Contralat hemiplegia
○ Contralat hemisensory disturbance
○ Gaze palsy to opposite side, eyes deviated to the side of lesion
● A partial Horner’s sy. may develop (SY fibers on the ICA wall)
● Occlusion of the dominant hemisphere → global aphasia
162
Occlusion of anterior cerebral artery
supplies medial part of hemisphere: lower limb
primary motor cortex:
* contralateral weakness
somatosensory cortex:
* contralateral sensory loss
163
occlusion of middle cerebral artery
supply lateral part of hemisphere: upper limb
sensory and motor loss + Broca's area
164
occlusion of posterior cerebral artery
Occipital lobe: vision
homonymous hemianopia
165
what does insula do
decision-making
166
supplied by the vertebrate arteries
medulla and inferior surface of cerebellum
167
supplied by the basilar artery
brain stem from medulla and up and gives off PICA and AICA
168
what is supplied by the vertebrobasilar system
brainstem, midbrain, pons , medulla
, cerebellum, occipital lobe, , thalamus
169
symptoms of vertebrobasilar insufficiency
○ Drop attacks (weakness of quads→ fall to the ground)
○ Diplopia
○ Dysarthria
○ Dizziness
○ Vertigo
○ Dysphagia
resolves within 24h
170
cause of vertebrobasilar insufficiency
VBI is usually caused by
atherosclerosis, hypertension,
diabetes, smoking, dyslipidemias.
171
how to treat VBI
Treatment often includes lifestyle changes and treatment of underlying conditions.
Patients can also get started on antiplatelet or anticoagulation.
Angioplasty is a possibility
172
posterior cerebral artery stroke
- Contralateral hemianopia or quadrantanopia
- Midbrain findings:
-ipsilateral CN3 III and CN4 IV palsy/pupillary changes,
-contralateral hemiparesis/hemiplegia (= Weber’s syndrome)
- Posterior cortical infarction in dominant hemisphere: problems in naming colors and
objects
173
basilar artery stroke
locked in syndrom
- bilateral loss of corticospinal tract - **complete paralysis**
- Preserved vertical eyemovement + blinking
- patient is conscious alert and aware
174
AICA stroke
lateral pontine syndrome
- I/L CN VII facial n palsy
- I/L vestibular nuclei damage: vertigo and nystagmus
- I/L Ataxia: poor coordination
- C/L loss of pain and temp (ST tract)
175
anterior spinal artery stroke
Causes medial medullary infarct
○ Contralateral hemiparesis (facial sparing),
contralateral impaired proprioception and vibration,
* ipsilateral tongue weakness
176
PICA stroke
loss of coordination, balance and muscle tone
CN X - dysphagia and loss of gag reflex
horners syndrom
177
what is horners syndrom
ptosis
miosis
anhydrosis
178
occurrence of ischemic vs hemorrhagic stroke?
ischemic 85%
hemorrhagic 15%
179
what is a ischemic stroke tat resolves within 24h called?
TIA
180
causes of ischemic stroke?
endothelial; clotting
embolism/thrombosis
-atherosclerosis: stenosis, occlusion
-dissection
-vasculitis
- emboli
- lacunar occlusion of small vessels
can transform to hemmorhagic after few days (common in cardioembolic stroke)
181
first imaging in a ischemic attach
stat CT no contrast to rule our SAH or ICH
182
if no SAH or ICH and you cant see lesion on CT you...
take a CT angio and look for filling defect
183
MRI on a ischemic stroke you do
DWI and PWI are compared, if hypoperfusion seen > thrombolysis
DWI and a ADC and compare them
DWI show bright hyperintens
ADC show dark hypointens
Apparent diffusion coefficient
184
treatment of ischemic stroke
1. last known well < 4.5h ago give TPA
2. if last known well is more then 4.5-6h ago evaluate:
- no SAH/ICD
- no hypoglycemia or previous stroke
- NIHSS < 25
- infarct is < 1/3 of MCA territory
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contraindication of TPA
If the ischemic stroke is affecting >⅓ of the MCA territory - reperfusion can lead to hemorrhagic transformation
○ Mild symptoms/symptoms improving
○ Cerebral trauma or AMI in the last 3 months
○ Surgery the last 2 weeks
○ Present or previous intracranial hemorrhage/SAH
○ BP >185/110 mmHg
○ Peptic ulcer, epilepsy, hypoglycemia, fracture
○ INR>1.7, abnormal APTT, PLT<100.000/mm3
186
what to do if no tpa treatment in stroke
mechanical thrombectomy
if large vessel occlusion MCA, ICA, Basilar
-endovascular thrombectomy within 6hrs of onset of symptoms
-intraarterial thrombolysis : within 6 hrs
187
BP goals in stroke
if tPA < 185
if no tPA < 220
188
BP medication in stroke
NIcardipin IV
Labetolol IV
Hydralazine
189
DOAC's
Direct oral anticoagulants
dabigatran (direct thrombin (factor IIa) inhibitor)
rivaroxaban ( factor Xa inhibitors.)
apixaban (Xa inhibitors)
190
most common cause if hemorrhagicc stroke
sustained HT causing rupture
trauma
cerebral amyloid angiopathy (b-amyloid plaque)
coagulopathy
hemorrhagic transformation from a ischemic stroke
malignancy
191
most common location of a hemorrhagic stroke
pons
cerebellum
basal ganglia
thalamus
cortex (least common)
192
what to look for on imaging in hemorrhagic stroke ICH
midline shift
hydrocephalus/IVH
Expansion of the hematoma displaces the cortex outward, causing edema in the surrounding area within hours of the initial hemorrhage. This leads to mass effect on other structures, such as the ventricles, which may result in hydrocephalus. Sulci may appear compressed, with the gyri expanded and flattened.
193
blood pressure goal im hemorrhagic stroke
140/90 mm Hg
and if possible and tolerable to 130/80 mm Hg or even lower
194
reversal of anticoagulants
warfarin - vit K
Heparin - protamin
Dabigatran -Idarucizumab (monoclonal Ab)
Apixaban or Rivaroxaban- Andexanet alfa = recombinant modified human factor Xa
195
when to intubate in cerebrovascular disorders
intubation is necessary if bulbar reflexes are absent/patient is in coma
196
when to mechanical ventilate in cerebrovascular disorders
saturation < 90% or pCO2 > 50 mmHg → mechanical ventilation
197
give glucose in cerebral ischemia?
Glucose is contraindicated in cerebral ischemia (except in hypoglycemia).
198
when to give insulin?
Insulin should be given if glucose levels > 15 mmol/L (target: 7,8-10 mmol/L
199
nucleus related to dysphagia
nucleus ambignous
200
dose of tPA
0.9 mg/kg (max 90mg), and 10% is given as i.v. bolus followed by 1h infusion of remaining 90%. Check BP and neurologic symptoms every 15 min during infusion.
201
when is follow up CT indicated in stroke
24h
202
when should antiplatelet therapy start in stroke
NOT before 24h
203
define cerebrovascular disorder
Cerebrovascular disorders include all disorders in which an area of the brain is temporarily or
permanently affected by ischemia or hemorrhage, and one or more of the cerebral blood vessels are
involved in the pathological process.
204
stroke Protocall
1. ABCDE (remember glucose)
2. Blood samples (Glucose, INR) and blood pressure
3. GCS, NIHSS: neurological screening assessment
4. ECG (should not delay CT/MRI)
5. CT or MRI (should be interpreted within 45 minutes)
○ Hemorrhage: consult neurosurgeon
○ Ischemia: candidate for thrombolysis
6. Thrombolysis (rtPA) if within therapeutic window
205
how can imaging tell us if we are still in the therapeutic window?
DWI shows ischemia while FLAIR shows no ischemia
206
how can imaging show if there is pneumbra?
DWI-PWI mismatch: DWI shows infarct core, while PWI shows hypoperfused tissue,
207
INR value allowing thrombolysis ?
< 1.7
208
mandatory to check before thrombolysis
● Hypoglycemia (glucose): can mimic acute stroke
● Coagulopathies (INR): CI of thrombolysis (should be under 1,7 to perform)
● Blood pressure (BP): hypertension increase hemorrhagic risk of thrombolysis
209
diagnostic considerations in cerebrovascular disease
Presence of clinical signs (WHO)
● Hemorrhagic vs. ischemic (CT, MRI)
● Pathomechanism (thrombotic, embolic, hemodynamic, small vessel disease)
● Duration of signs
● Brain region (hemisphere, cerebellum, brainstem; territorial-borderzone)
● Anterior-Posterior
● Supplying vessel (carotid artery, vertebrobasilar, lacunar, ACA, MCA, PCA)
● Prognostic by signs (Bamford – OCSP)
○ TACI, PACI, POCI, LAC
Total anterior circulation infarct (TACI
partial anterior
Posterior circulation infarct
Lacunar stroke
210
○ TACI, PACI, POCI, LAC
TACI: total anterior circulatory stroke
PACI: partial anterior circulatory stroke
POCI: posterior circulatory syndrom
LACI: Lacunar syndrom
211
cranial nerves run in the cavernous sinus what can happen
oculomotor
Opthalmic
maxillary
abducent
can be compressed if infection causing CN palsy
212
eye innervation muscle
superior, medial and inferior rectus + inferior bliq by CN III
lateral rectus by CN VI
superior oblique by CN IV
213
2 venous systems of brain
superficial and deep + sinuses
214
Thrombotic occlusion of the venous system occurs with:
● Infection (especially ear or sinus infection)
● Dehydration
● Pregnancy and puerperium
● Coagulation disorders
● Malignant meningitis
● Miscellaneous disorders (e.g. sarcoid, Behçets)
215
most frequent place of venous thrombosis in brain
Superior sagittal and lateral sinus thrombosis (85% of cases)
216
symptoms of cerebral venous thrombosis
○ Impaired CSF drainage results in headache, papilloedema and impaired consciousness.
○ Venous infarction produces seizures and focal deficits (e.g. hemiplegia).
217
CT sign of venous thrombosis
(Δ) ‘Empty delta’ sign
Consists of a
* central hypodensity (representing the thrombus)
* with a triangular outline of contrast enhancement
May be seen in cerebral venous thrombosis of the superior sagittal sinus.
(contrast filling defect: following contrast the wall of the sinus enhances but not the central thrombus on CT)
218
treatment of cerebral venous thrombosis
Treatment: Correct causative factors (dehydration/infection etc.) + anticoagulation with heparin or alternative
219
cavernous sinus thrombosis signs
● Commonly results from infection from the jaw
● Painful ophthalmoplegia, proptosis and oedema of periorbital structures are associated with facial numbness and fever.
● The disorder may be bilateral.
● Treatment with antibiotics and if indicated, sinus drainage.
ophthalmoplegia, paralysis of the extraocular muscles that control the movements of the eye.
220
prognosis of intracranial bleedings?
approximately half of patients
die within 30 days
221
most common site of intracranial bleeding
In hypertensive patients 70% occur in the basal ganglia/thalamic region. In normotensive patients, 37% occur in this area
222
signs of intracerebral hemorrhage
1. **Basal ganglia** - Hemiparesis, sensory loss, eye deviation
2. **Thalamus** - Sensory loss, later hemiparesis, gaze disturbance
3. Lobar - Better prognosis, intraventricular bleeding is rare
4. Cerebellar - Nausea, ataxia, dizziness, signs of brainstem compression (30% mortality)
5. Pons - Fast progressing hemi- or tetraparesis, disturbed eye movements, decerebration, small
pupils, disturbance of breathing, coma, death (high mortality
223
pathological effect of intracerebral hemorrhage
● Space-occupying effect → Brain shift
● Continued bleeding → Expanding may continue beyond the first few hours.
● Within 48 hours: disruption of BBB, vasogenic and cytotoxic edema, neuronal damage and necrosis
● Resolution in 4-8 weeks → Cystic cavity
224
complication of intracerebral hemorrhage
● Repeat hemorrhage
● Vasospasms
● Deep vein thrombosis
● Dysphagia (can lead to aspiration pneumonia)
● Elevated ICP → Brain herniation
● Seizures
● Hydrocephalus
● SIADH
225
caus of SAH
Traumatic brain injury can cause aneurism
Non-traumatic:
● Aneurysms (75%) rupture
● perimesencephalic hemorrhage (10%),
● Ruptured arteriovenous malformations (AVM) (5%)
● Others: cortical thrombosis, angioma, neoplasm, infection
● 20% of investigations fail to reveal the source
* triggered by acute increase in BP
226
signs and symptomes of SAH
Signs and symptoms
● Severe headache w/ instantaneous onset
● Loss of consciousness, coma
● **Epileptic seizure**
● Nausea, vomiting
● **Neck stiffness** present in most pts
● Focal signs (e.g. limb weakness, dysphasia)
● Reactive hypertension
● **fever**
227
diagnosis of SAH
● Urgent CT without contrast: detects 95% of SAH within 24h
● Lumbar puncture: if CT is neg. but history is very suggestive of SAH,
needs to be done >12h after headache onset to allow breakdown of RBCs → a pos. sample is xanthrochromic (yellow due to bilirubin)
● MRI: may be used in pts w/ multiple aneurysms
● CT/MR angiography, digital angiography: more detailed info
228
treatment of SAH
● Re-examine CNS often (BP, pupils, GCS)
● Maintain cerebral perfusion by keeping well hydrated (aim for SBP<160 mmHg)
● Nimodipine to prevent vasospasms
● Surgery: within 48h, if not a delayed intervention is recommended (after 14 days)
- surgical clipping, endovascular coiling.
- balloon remodeling and flow diversion (newer technique) to close aneurism
229
aneurism surgery?
Endovascular coiling vs. surgical clipping, depending on accessibility and size of aneurysm (coiling is preferred)
Balloon remodelling and flow diversion are newer techniques used for anatomically challenging aneurysms
230
define status epilepticus
A state of continuous seizure lasting ≥ 5 min, or ≥ 2 repetitive, separate seizures with consciousness not fully regained in the interictal period.
231
what is epilepsy
Epilepsy: a chronic neurologic disorder characterized by a predisposition to seizures
232
Generalized tonic–clonic seizure
A generalized tonic–clonic seizure, commonly known as a grand mal seizure, produces bilateral, convulsive tonic and clonic muscle contractions.
233
Focal seizures
are seizures which affect initially only one hemisphere of the brain.
234
Pediatric absence seizures
(also called petit mal seizures) are characterized by a brief altered state of consciousness and staring episodes. And do not cause your child to fall or have significant shaking movements.
235
4 stages of a seizure
Prodromal.
Early ictal (the “aura”)
Ictal.
Postictal.
236
how is the postictal stage after a seizure
Period that begins when a seizure subsides and ends when the patient returns to baseline.
It typically lasts between 5 and 30 minutes and is
characterized by disorienting symptoms such as
confusion, drowsiness, hypertension, headache, nausea, etc
237
causes of seizure in adults
○ Antiepileptic drug withdrawal
○ Acute cerebral embolization
○ Metabolic disorder
○ Alcohol intoxication
○ Tumor
○ Neuroinfection
238
causes of seizure in children
fever
infection
239
what can confirm the end of status epilepticus
The termination of status epilepticus must be confirmed by EEG (due to non-convulsive or
electrographic status epilepticus)
240
why must treatment in status epilepticus happen fast
The risk of a focal status epilepticus turning in to a generalized one is relatively high, therefore
treatment is similar in both forms
241
treatment in status epilepticus
0-5 min usually self limeting do ABCDE, IV accsess, glucose
5-20 min give midazolam IM 10mg or lorazepam IV 0.1mg/kg or diazepam
20-40 min fosphenytoin, Valproic acid IV , levetiracetam
40-60 min repeat 2nd line therapy Valproic
- anesthetic doses of midazolam or thiopental (with continuous EEG)
242
etiology of viral meningitis
○ 80% is caused by enteroviruses (coxsackie, echo, nonparalytic polio).
○ Less commonly caused by VZV, HIV, mumps.
○ Outbreaks are especially common in early spring and late autum
243
etiology of bacterial meningitis
○ Most common pathogens: pneumococcus pneumoniae, N. meningitidis, Listeria (mostly
in alcoholics or when using systemic immunosuppressants)
○ In childhood Staphylococci are the most common pathogens.
244
why can pathogens proliferate easy in the brain
The primary immune response in the CNS is weak, as there is no MHC I and II antigens on
neurons or astrocytes; thus they are not able to do phagocytosis
245
most common rout of infection to meninges in meningitis
Most common route of infection: colonization in nasopharynx → subdural space → subarachnoid
space → meninges and basal cisterns (meningeal signs
246
Clinical signs and complications of meningitis
● Nuchal rigidity, meningeal signs and altered consciousness
● In case of N. meningitidis infection a petechiae can develop
● Serous or purulent exudate block absorption or circulation of CSF → hydrocephalus, cranial nerve abnormalities and altered consciousness
● Increased ICP due to vasogenic edema because of increased permeability of BBB
● Exudates in venous sinuses → thrombosis, reactive vasculitis and brain ischemia
● Ventriculitis when infection reaches the sinuses
● Bacterial: no AB treatment can lead to death in less than 24h. Prophylaxis is given to close contacts
247
diagnosis of bacterial meningitis
Fluid: cloudy, unclear
Protein: high >1g/l
Lactate: high
Glucose: low >0.4 csf/sera
Cell count: High - leukocyte count > 1000/mm3
↑ Granulocytes (> 80%)
Do ELISA (or PCR for listeria)
248
diagnosis of viral meningitis
Fluid: clear
Protein: norm
Lactate: norm
Glucose: norm
Cell count: Variable cell count (leukocyte 100–500/mm3)
↑ Lymphocytes
Do PCR
249
meningitis treatment
● Empirical treatment with 3rd generation cephalosporin + amoxicillin + vancomycin i.v. should
be administered before culture results are ready
● Confirmed N. meningitidis: ciprofloxacin given to close contacts
● Viral: symptomatic treatment
250
define encephalitis
Encephalitis: inflammation of the brain parenchyma
251
etiology of infectious encephalitis
Arboviruses are the most common
Most common causative agents:
* HSV-1,
* VZV,
* tick-born encephalitis viruses
* Enterovirus spp.
In immunocompromised patients:
* CMV, JC viruses,
* Listeria, Mycobacterium, Mycoplasma spp
252
what is limbic encephalitis
Limbic encephalitis: Antibodies against cell surface antigens such as receptors, e.g. anti-NMDA
receptor Ab mediated encephalitis (ovarian teratoma)
253
define multiple sclerosis
A chronic progressive degenerative disease of the CNS characterized by demyelination and axonal degeneration in the brain and spinal cord, which are caused by an immune-mediated inflammatory processes
254
type of reaction in MS
Type IV HS reaction - cell mediated
255
pathophysiology of MS
Tcells are activated in periphery, then
cross react with parts of myelin in CNS leading to:
* **demyelination**
* **secondary axon degeneration**
attacks myelinproteins on nerve cells causing oligodendrocyte (CNS myelin) destruction and plaques called sclera - hence name multiple sclerosis - result is brain athropy
**Inflammation** and immune cell infiltration cause damage to the myelin, affecting the electrical signals moving along the neurons.
256
types of MS
Plot neurological destruction up and time.
Relapsing-remitting (90%)
Secondary progressive
Primary progressive
Progressive relapsing
257
in the CNS the only nerve with oligodendrocyte myelination
is the optic nerve causing optic neuritis
258
symptomes on optic nerve because of MS
Optic neuritis:
loss of visual acuity : blurry vision
loss of color vision
afferent pupil defect - marcus gunn pupil - mydriasis
259
what is seen in MS with eye movement
* bilateral internuclear opthalmoplegia (brainstem symtpoms)
loss of connection between CN 6 and 3
when you look to the side only lateral movement works not medial by CN 3.
But if you ask the patient to focus on a pen it works
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diagnosis of MS
MRI:
periventricular lesions
brainstem lesions
SC lesions
Cerebellum lesions
CSF: oligoclonal gammopathy/bands = IgG Ab from plasma cells
VEP: decreased AP due to reduced conduction velocity
MEP, SEP - to detect subclinical lesions and predict course of disease
Serology (differential: borreliosis, treponema)
261
treatmeant of MS
1. high dose CS (methypredinisolone) OR
2. Plasmapheresis
3. Supportive:
Spasmicity: Baclofen, dandrolone
decrease detrusor hyperactivity: anticholinergic
fatigue: amantidine
paroxysmal symptoms: gabapentic, carbamazepine
4. Disease modifying therapy to prevent relapses: (long term stability)
Glatiramer acetate
interferons
Alemtuzumab (anti-cd52)
Daclizumab (alpha intergrin; inhibits migration through BBB)
Natalizumab (decrease relapse rate)
Autologous stem cell transplantation
262
define brain edema
an excess of brain water, there’s three types of cerebral edema
a) Vasogenic
b) Cytotoxic
c) Interstitial
263
define vasogenic edema
● Damage to the BBB → high protein content extracellular edema, spreading in white
matter along the nerve fibers
● Perifocal in tumors, central abscesses, parenchymal hemorrhage
generalized in meningoencephalitis
● Dx: T2-weighted MRI is most sensitive
● Tx: corticosteroids
264
define cytotoxic brain edema
● Ion gradient btw the intra- and extracellular space decreases → Na+, Cl-, water moves into the neurons
→ damage to voltage-gated Ca++ ch.
→ Ca++ influx → fluid accumulates within cells
* ● Typical in **cerebral ischemia** in the area of **cerebral cortex** and **basal ganglia**, followed
by vasogenic edema as the capillaries are damaged
● Dx: diffusion-weighted MRI
265
define interstitial edema
● Develops in **occlusive hydrocephalus**
● Increased CSF pressure → CSF pressed across ependymal layer of ventricles into the brain parenchyma
● Dx: hypodense halo surrounding the ventricles on CT,
high intensity signal on T2-weighted MRI
266
treatment of brain edema
1) Causative treatment
2) Osmodiuretics (mannitol, glycerol)
3) Loop diuretics: decreases CSF production
4) Controlled hyperventilation: reduction of pCO2 by 10 mmHg decreases the ICP by 30% because of cerebral vasoconstriction
5) Barbiturate narcosis w/ EEG or plasma level monitoring
6) If ICP is so high that herniation is unavoidable → decompressive craniectomy
7) If occlusive hydrocephalus → temporary CSF drainage
raised ICP must be reduced!
267
define wernick-korsakoff syndrom
Wernicke encephalopathy is an acute, reversible condition caused by severe thiamine (vitamin B1) deficiency, often due to chronic heavy alcohol use. Wernicke-Korsakoff syndrome is by definition when symptoms of two different conditions are seen
together; Wernicke´s Encephalopathy (WE) and Korsakoff Syndrome (KS).
268
site of lesion in wernick-korsakoff syndrom
Lesions are bilateral around 3rd and 4th ventricle and the cerebral aqueduct.
269
define Korsakoff syndrome
(chronic and only 20 % reversible with treatment): memory symptoms
- Anterograde amnesia (problems learning new information) and short-term memory loss
- Confabulations (make up information to fill memory gaps)
- Disorientation in time and space
270
etiology of wenicks-korsakoff syndrome
- Chronic alcoholism (most common)
- Malnutrition (starvation, eating disorders, gastric surgery, cancer, prolonged vomiting etc) → decreased absorption of thiamine
271
diagnosis of wernicks korsakoff syndrom
↓ Serum thiamine levels
↓ Erythrocyte transketolase activity (thiamine dependent)
↑ Serum lactate and pyruvate
Evidence of alcohol-related liver dysfunction
Brain MRI: T2-weighted hyperintense lesions
in the mammillary bodies,
midbrain tectal plate,
dorsomedial nuclei of the thalamus,
cerebellum,
and around the aqueduct and the third ventricle
272
treatment of wernick korsakoff
Thiamine replacement iV
treat underlying condition
273
Define Guillian-Barre
acute immune-mediated polyneuropathy that typically manifests with bilateral ascending flaccid paralysis and sensory involvement.
274
coars of Gullian barre (GBS)
About two-thirds of GBS patients experience symptoms of an upper respiratory or gastrointestinal tract infection up to 6 weeks prior to onset of GBS.
275
pathogens related to Gullian Bare Syndrome
Campylobacter jejuni: Campylobacter enteritis is the most common
Cytomegalovirus (CMV)
HIV
Influenza
Zika virus
Epstein-Barr virus
SARS-CoV-2
Mycoplasma pneumoniae
276
pathophysiology of GBS
Postinfectious autoimmune reaction that generates cross-reactive antibodies (molecular mimicry)
Infection triggers humoral response → formation of autoantibodies against gangliosides
277
diagnosis of GBS
CSF analysis:
May be normal in the first 1–2 weeks of the disease.
Typical: albuminocytologic dissociation (i.e., increased protein levels with normal leukocyte count < 10 cells/mcL in CSF)
Electromyography
Pathological spontaneous activity is a sign of an unfavorable prognosis.
278
symptomes of GBS
Limb involvement:
Bilateral and ascending from the lower limbs
Progressive flaccid paresis or paralysis
Paresthesia (prikking): stocking‑glove distribution
Hyporeflexia typically begins in the lower limbs.
Back and limb pain (often an early symptom)
Involves nociceptive and neuropathic pain
Autonomic dysfunction
Cardiac arrhythmias, blood pressure fluctuations
Urinary retention and/or intestinal dysfunction
Respiratory muscle involvement may lead to respiratory failure.
Cranial nerve involvement
Facial palsy: due to bilateral facial nerve involvement (most frequently affected cranial nerve in GBS)
279
treatment of GBS
IVIG or plasmapheresis
280
define myasthenia gravis (MG)
Autoimmune disease of the neuromuscular junction (NMJ) characterized by muscle weakness that worsens with activity and improves with rest
281
type of reaction in MG
Type 2 HS reaction - cytotoxic
282
age distribution of MG
young women 20-30
Old men 60-70
283
pathophysiology of MG
Responsible for inhibition of signal transduction at the neuromuscular junction (NMJ)
Antibodies target postsynaptic AChRs of normal muscle cells → competitive inhibition of acetylcholine (ACh) → AChR decay through receptor internalization (↓ receptor density at the postsynaptic membrane) and activation of complement (→ muscle cell lysis) → impaired signal transduction at the NMJ → skeletal muscle weakness and fatigue
284
classification of MG
**Ocular myasthenia**: only the extraocular and/or eyelid muscles
**Generalized myasthenia**
All skeletal muscles may be involved.
Especially ocular, bulbar, limb, and respiratory muscles
- proximal limb weakness, hyperreactive reflexes.
**bulbar form**: dysarthia, dysphagia, myesthenic snarl (buccinator weakness produces characteristic smile)
285
serotypes of MG
Seropositive MG (80–90% of cases): positive assays for antibodies (in blood) against the acetylcholine receptor (AChR-Ab)
Seronegative MG (10–20% of cases): negative for AChR positive for MuSK Ab (muscle specific kinase) , anti-striated muscle antibodies
286
MG symptomes
Eye muscles: diplopia, blurred vision,ptosis
Bulbar muscles: dysarthria, difficulty chewing and/or swallowing
Proximal muscles: difficulty standing from a chair , climbing stairs, brushing hair
Respiratory muscles: dyspnea, respiratory failure
287
MG diagnosis
● Clinical picture
● IV edrophonium = Cholinesterase Inhibitor (Tensilon) test: assess for improvement over 2 min, positive if clear
improvement in weakness
● Electromyography: Repetitive stimulation → decremental response (reduced amplitude)
● Serology: anti-AChR Ab (present in 70-90%), MuSK antibodies (30%)
● Chest CT/XR to screen for thymic hyperplasia (thymoma (10%) , persistent thymus (70%)
288
MG treatment
1. Cholinesterase inhibitor: first-line agent is pyridostigmine
2. Immunosuppressants:CS, azathioprin
Indications: inadequate symptom control with (or intolerance to) pyridostigmine
-suppresses the production of antibodies
3. Thymectomy if thymoma or severe generalised myesthenia
4. Myesthenic crisis: IVIG / plasmapheresis
289
diseases of micturition
1. Spastic, neurogenic bladder
2. Flaccid neurogenic bladder
3. Detrusor-sphincter dyssynergia
4. Frontal lobe incontinence
290
Spastic, neurogenic bladder
CNS lesions **above sacral** spinal cord
→ Disinhibition and increased sensitivity (hyperreflexia) of detrusor muscle
→ Urge incontinence and decreased bladder capacity
291
Detrusor-sphincter dyssynergia
● Uncoordinated function of detrusor muscle and external sphincter.
● Urge to urinate, but cannot due to sphincter spasm → Retention.
● From CNS lesions **above sacral** spinal cord. May have together with spastic bladder
292
Flaccid neurogenic bladder
● Hypotonic bladder wall → Increased capacity.
● From lesion in sacral spinal micturition center (S2-4) or distally (conus, cauda equina, peripheral nerves).
● Bladder wall is insensitive and paralyzed → Overfilling → Overflow incontinence,
constant urine dripping
293
Frontal lobe incontinence
● Disorders that affect the frontal lobes can cause
1. detrusor hyperactivity
2. or altered social behavior regarding micturition
no inhibition on pontine micturition center when bladder is filled
294
Disturbance of Defecation
* Fecal retension - caused by transverse lesion of Spinal cord above L1-2 (where hypogastric nerve originates)
* fecal incontinence: lesion of sacral segments
295
most vulnerabel spots for spinal cord injury
border between rigid and flexible regions (
1. craniocervical, CC
2. cervicothoracal, CT
3. thoracolumbar zones
296
types of spinal cord injury
Acute transverse
Brown-Sequard syndrom
Anterior cord
Central cord
Conus medullaris
Spinal cord concussion
Cauda equina syndrom
297
Genetic background of alzheimers disease
AD
genetic risk factor: ApoE epsilon4 allele ( 1 increases risk by 2-3, 2 alleles increase risk by 10x)
* monogenic form:
*beta-amyloid precursor protein (APP)
*Presenilin-1 mutation
*Presenilin-2 mutation
* Polygenic: multiple genes, and environmental
298
Genetic bckground of frontotemporal dementia
* Polygenic, complex inheritance
* Autosomal Dominant (AD) in 20-50% of cases
* mutations of 2 genes:
*Microtubule ass. protein tau gene MAPT -> tau protein
*GRN encodes progranulin
due to mutations pathologic proteins are expressed which aggregate in neurons
299
Huntington's disease definition
Neurodegenerative disorder causing progressive, selective (localized) neural cell death
associated with choreic movement and dementia
300
what is huntington disease associated with
associated with
* choreic movement
* dementia
301
Etiology of Huntingtons disease
increased length of a CAG tripplet repeat present in the huntington gene on chr 4p16.3
CAG repeats is normally < 36 (average 19)
in pts with mutated HD gene the repeat size is between 36-121
the higher the no. the worse the course with earlier onset, more severe, quicker progression
302
prevelance of HD
4-8/100,000
no difference in gender
303
when do symptoms start in HD
between 3rd-5th decade
lasting on average 10-15 years
10% had juvenile onset
20% late onset mild HD
more severe with earlier onset in each succeeding generation (anticipation)
304
cause of death in HD
deterioration of somatic condition
associated with secondary diseases
305
pathomechanism of HD-which areas affected in nervous system?
most prominent changes in
* caudate
* putamen
* substantial nigra
* cerebral cortex
* hippocampus
* purkinje cells in cerebellum
306
which neurons are most vulnerable in HD
striatal spiny neurons
307
what does CAG code for
glutamine -> polyglutamine tract
> production of altered 3D forms of protein that aggregates, forms neuronal inclusion bodies, toxic protein fragments > metabolic stress > altered DNA expression and protein-protein interaction
308
signs and symptoms of HD
* **Motor symptoms**: chorea, hyperkinesia, rigidity, dysarthia
* **Psychopathological**: irritation, impulsivity, aggression, depression
* **cognitive decline, dementia**:
*memory loss, slow data processing
*already present from the beginning
309
first motor sign in HD
often CN3 occulomotor function disturbance
then orofacial dyskinesia (continuous grinning)
later dyskinesia appears in other muscle groups (generalized chorea)
even later stages: bradykinesia, rigidity, dystonia may dominate
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what is chorea
brief, quasipurposeful, irregular muslce contractions that are not repetitive or rhythmic
but flow from one muscle group to the next
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diagnosis of HD
* clinical sign
* postive family history
* genetic testing (if negative, do MRI to rule out inflammatory, autoimmune or neurodegenrative CNS disorders)
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TX of HD
symptomatic
* SSRI, mirtazapine, sulpiride for depression
* neuroleptic drugs : clozapine, olanzapine, tiapride, tetrabenazine, amantidine) for hyperkinesia
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fragile X definition
x-linked dominant disorder
causes range of developmental problems :
* learning disability
* cognitive impairment
males more severely affected
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fragile x ETIOLOGY
>200 CGG repeats in FMR1 (Fragile x messenger ribonucleoprotein 1) (normally 5-40 repeats
> causes FMRP protein defiency (regulates production of other proteins and plays roles in development of synapses)
> distruption of nervous system functions
premutation (55-200 repeats) milder version of disease
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fragile x signs and symptoms
delayed development of speech and language by age 2
intelectual disability (mild/moderate)
children may have anxiety, hyperactive behaviour, attention deficit disorder or autism
seizures
characteristic physical features:
* long
* narrow face, large ears
* prominent jaw, forehead
* flexible fingers
* flat feet
* enlarged testicles after puberty
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fragile x diagnosis
molecular testing
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fragile x tx
speech and language therapy
special education in children
