CASE STUDIES (in order of

their presentations)
GROUP 11
Guillain-Barre Syndrome
Acute Inflammatory demyelinating polyradiculoneuropathy (AIDP) most
common form in US
Autoimmune attack of PNS destroying myelin and in severe cases axons
Commonly triggered by respiratory or GI infection (bacterial or viral)
Campylobacter jejuni is most common precipitating infection
Reduced nerve conduction
Progressive limb weakness, reduced sensation, and areflexia in
extremities and progressing proximally (stocking-glove)
Can impact cranial nerves (unable to bury eyes)
Can impact autonomics (tachycardia, constipation,
diaphoresis=sweating)
Elevated CSF protein without increase in CSF WBC (albuminocytologic
dissociation)
Treatments
Plasmapheresis
Remove plasma from whole blood and return WBC and RBC to
body
Thought to remove immune cell factors that are attacking PNS
High dose immunoglobulin therapy
High IV dose of Ig from healthy donors
Thought to reduce host immune cell attack
Guillain-Barre Syndrome
Prognosis
Weakest point 2-3 wks from onset of symptoms
30% of recoveries have residual weakness for at least 3 years
3% of recoveries may experience relapse of symptoms

Guillain Barre Multiple Sclerosis

Demyelination of PNS Demyelination of CNS
LMN deficits UMN deficits
Flaccid paralysis Increased muscular tone
Hyporeflexia Hyperreflexia
Rapid onset More gradual onset
Stocking Glove Often presents with
Numbness visual changes
Practice
Questions Guillain-Barre syndrome
Which disease manifests as self-limiting
ascending paralysis associated with prior
infection or vaccination?
Campylobacter jejuni
Infection with what bacteria is associated
with about 25% of cause of Guillain-Barre
syndrome?
Respiratory muscle paralysis

What is the biggest risk for mortality in

Guillain-Barre syndrome? Plasmapheresis or IV immunoglobulin

What is the treatment for Guillain-Barre Increased CSF protein without an

syndrome? increase in cells (albuminocytologic
dissociation)
What CSF abnormalities are associated
with Guillain-Barre syndrome?
GROUP 15
Summary Acute, Right Cerebellar
Hemorrhagic Stroke
Patient Presentation
Treatment
Hypertension
Monitor patient for
Nausea, dizziness, vertigo
Vestibular nuclei posterior fossa
Bi-frontal headache edema
Lethargic If necessary,
Flat optic disc w/ copper wiring posterior fossa
decompression
Neuro exam
Nystagmus on right gaze
Decreased muscle tone R arm & leg; normal Key Terms
strength Vertigo
Ipsilateral loss = Vermis, medial hemisphere
Hypertension
Intention tremor
Finger-to-nose = Intermediate & lateral Intension tremor
hemispheres Lipohyalinosis
Heel-to-shin = Lateral hemisphere
Lumbar puncture
L Babinski sign
Broad-based gait; list to R
Drunken sailor = Anterior hemisphere
GROUP 13
 Summary: Huntingtons Disease

Cause: Autosomal dominant mutation in

huntingtin gene on chromosome 4p; CAG
repeats
Symptoms:
Atrophy of head of caudate
Huntingtons Triad: Chorea, dementia,
psychosis
Treatment: Dopamine antagonists
(phenothiazines) or dopamine-depleting
agents. No cure.
Practice
Questions
What basal ganglia structures are Striatum, particularly caudate nucleus
affected by Huntington disease?

What is the genetic alteration Triplet repeat (CAG) on chromosome 4

responsible for HD?
GROUP 18
 High Yield Summary-Acoustic
Neuroma
CN V, VII, VIII (IX and X) deficits
Sensorineural hearing loss and
tinnitus
Weber: lateralizes to
unaffected ear
Rinne: AC > BC bilaterally
Vestibular signs
Hyperintense mass in internal
auditory canal
Corneal (blink) reflex
Afferent: CN V
Efferent: CN VII
Scwhannoma = S100+
Bilateral acoustic neuroma =
Neurofibromatosis type 2
Treat with surgery or radiosurgery
Cat burglar=neither a cat that
Vertigo, tinnitus,
steals things nor a person who
hearing loss
steals cats, but a person who
climbs up the sides of buildings in
order to perform their burglary
GROUP 16
Summary: VPL/VPM LESION
54 year old woman with left sided sensory loss/numbness

Began in left hand and spread to entire left side of body

Ataxic left finger-to-nose and heel-to-shin

CT/MRI: lesion to Right thalamus near VPL/VPM nuclei (contralateral)

Diagnosis: Right Thalamic Lesion of the VPL/VPM Nuclei that contributed to

the development of Dejerine-Roussy Syndrome (Thalamic Pain Syndrome)
Post Thalamic stroke

Progressive one sided weakness beginning with numbness/tingling in

opposite side of body

Dysaesthesia
GROUP 21
 Summary: The Bruised
Baker (Betty)
Presentation
Difficulty visualizing right visual
field
Bumping into things on her Optic Tract vs. Optic
right, fell twice Radiation vs. Visual
5-10 min episodic diplopia, no Cortex
No RAPD, CT with
recurrence
lesion in area of PCA
PMH
visual cortex
Hypertension (13 yrs), Tobacco
(>35 pack yrs)
Physical Exam Right
BP = 158/91 Homonymus
Faint left carotid bruit
Mild narrowing of retinal Hemianopia -
arterioles Left PCA
No reflex afferent pupillary
defect (RAPD) Stroke
No loss of visual acuity Left visual cortex
Contralateral cortical blindness V1 lesion
with macular sparing Macular
sparing due to collateral supply
GROUP 7
Summary: Left MCA Stroke
Patient Presentation:
60 y.o. right-handed secretary
sudden onset of right- hand
clumsiness and halting and limited
speech
intermittent palpitations Significant PMH:
Significant findings from history of essential hypertension
physical/neurological exam and
labs
atrial fibrillation/ Hx of
hypertension embolism/infarct
present Babinski Sign, pronator drift
and dysdiadochokinesia UMN
issue
acute hyporeflexia/ hypotonia of
right arm
Right upper extremity weakness,
left lower facial droop and Brocas
aphasia infarct to region
covered by MCA
Practice Questions

What artery is occluded if a patient presents with Dominant MCA

contralateral hemiplegia especially of upper
extremities, contralateral hemisensory loss
especially of upper extremities, homonumous
hemianopia, and aphasia
Broca aphasia
What expressive/nonfluent aphasia results from
diminished MCA supply to the inferior gyrus of the
frontal lobe in the dominant hemisphere?
The patient speaks slowly and with difficulty, but h
What are the symptoms of Broca aphasia? good comprehension of speech

Wernicke aphasia
What receptive/fluent aphasia results from
occlusion of the MCA supply to the posterior
aspect of the dominant superior temporal gyrus?
The patient speaks quickly and incoherently with p
What are the symptoms of Wernicke aphasia? comprehension of the spoken language

Nondominant MCA
What artery is occluded if a patient presents with
contralateral hemiplegia, homonymous
hemianopsia, contralateral hemisensory deficits,
sensory neglect, and apraxia?

Left (dominant) MCA stroke

GROUP 9
Summary Slide-
Lacunar Stroke Peripheral Neuropathy

Posterior Internal -LMN deficits: absent

reflex in ankle
Capsule/Basilar
Pons -Foot ulcer indicates
peripheral sensory loss
-Hemiparesis of CL
face, arm, leg
-UMN deficits:
Babinski,
Hyperreflexia,
Pronator drift
-
GROUP 10
 Group 10
ALS

Summary
Combined UMN and LMN deficits with no sensory or
oculomotor deficits

Can be caused by defect in superoxide dismutase 1

Commonly presents as fasciculations with eventual atrophy

and weakness of hands

Riluzole treatment modestly survival by

presynaptic glutamate release
For LOU Gehrig disease, give riLOUzole (a glutamate
antagonist)

Can present with pseudobulbar palsy due to BL UMN lesions

interrupts signals to the lower motor neurons in the brain stem
Caused our patients difficulty speaking and swallowing
contributes to emotional incontinence - difficulty controlling
emotions leading to crying/laughing at inappropriate times
Practice
Questions
What disease of upper and lower motor neurons presents
ALS (Lou Gehrig disease)
with weakness in distal extremities and atrophy of the
hands and forearms, but no sensory alterations?

Cu/Zn-superoxide dismutase (SOD1)

ALS is associated with a deficiency of which free radical
neutralizing enzyme?
GROUP 17
 Summary: WERNICKES
werNICkes
Mnemonic:
Short-term memory lossN: Nystagmus (Eye Movement
Alcohol-Related Dysfunction)
Dysconjugate Gaze

I: Incoordination (Ataxia)
Nystagmus
Ataxic Heel-to-Shin TestC: Confusion (Short Term Memory
IV - Thiamine Treatment Loss)

Terms to Know:

Heel-to-shin testing - assesses coordination of movements for distal extremities;

patient is supine and is asked to pass the heel of one leg slowly down the shin of
the other leg from knee to ankle

Confabulation - a filling in of gaps in memory through the creation of false

memories by an individual who is affected with a memory disorder and is unaware
that the fabricated memories are inaccurate and false

Dysconjugate gaze - failure of both eyes to turn together in the same direction
Practice Questions

What is the cause of Wernicke encephalopathy? Thiamine (B1) deficiency

What is the main population affected by Wernicke Alcoholics

encephalopathy?

What are the characteristic symptoms of Wernicke Ocular palsy, ataxic gait, confusion, drowsiness
encephalopathy?

What other syndrome is often seen with Wernicke Korsakoff syndrome

encephalopathy?

What are the symptoms of Korsakoff syndrome? Amnesia and confabulation

GROUP 19
SUMMARY: SEIZURES
PMH:
Brain injury at age 8
Secondary generalized seizures
with aura (sensory manifestation
of seizure) post-injury, treated
with medication
Complex partial seizures
(hallucinations, sympathetic
symptoms, and motor response)
Behavioral changes (lethargy,
aggression, irritability)
Mild hirsutism**
Neuro Exam:
Mental status was restless and
inattentive, short term memory
loss, paraphasic errors
Cerebellar deficits including
nystagmus, ataxia, difficulty
with rapid alternating
movements, pronator drift
Hyperreflexia with L biceps,
brachioradialis
Tests:
EEG: focal initiation of seizure in
the temporal lobe
Medications: Phenobarbital and
phenytoin at first; then
switched to carbamazepine
Diagnosis: Epilepsy with origin in
head trauma
Key Info:
Complex Partial seizure-
single focal area of brain,
consciousness altered
(preserved in simple partial
seizure), automatisms
(complex repetitive
behaviors)
Secondary Generalized
seizures- begins as focal
seizure and spreads to both
hemispheres, consciousness
is lost
RED= Key terms
**Hirsutism (female
androgenization) caused by
Practice Questions

What is epilepsy? Chronic condition characterized by recurrent

seizures
What is a partial seizure?
They originate in a focus and do not involve
both hemispheres
What is the major difference between simple
and complex partial seizures? Consciousness is altered in complex partial
seizures, but not in simple partial seizures

What is the term to describe a seizure that is

initiated in both hemispheres of the cerebral Primary generalized
cortex?

What is the term to describe a seizure that Secondary generalized s

starts focally and spreads to involve both
hemispheres of the cerebral cortex?

Temporal lobe epilepsy

GROUP 20
 Summary: MULTIPLE SCLEROSIS
Patient Presentation
Acute blurry vision in right eye
Second episode several
months later:
Internuclear
Opthalmoplegia
weakness & clumsiness of
the left leg
left hemiparesis
left facial weakness
left Babinskis sign
Neuro Exam
Light perception only in right
eye with swollen optic disc
Marcus Gunn pupil
Argyll-Robertson pupil
Diagnosis
Multiple Sclerosis
Testing
Head MRI with gadolinium
Treatment
Short term - high-dose
corticosteroids
Prophylactic treatment
- Interferon B-1b
(Betaseron), Interferon B-
1a (Avonex), Glatiramer
Acetate (Copaxone)
Key Terms
Afferent pupillary defect
(Marcus Gunn pupil)
Hemiparesis
Argyll-Robertson pupil
Practice
Questions
Random and asymmetric white matter Multiple Sclerosis
lesions with mixed sensory and motor
deficits
Multiple sclerosis
What is the most common cause of
internuclear ophthalmoplegia?
GROUP 2
Summary Slide - Alzheimers
Disease
Key findings
Slowly progressive, neurodegenerative
disease
Amyloid plaques and neurofibrillary
tangles
Decreased Ach & Increased Glutamate
NORMAL LABS
CT
Generalized cerebral atrophy, wide cortical
sulci, enlargement of ventricular system,
no evidence of cerebral infarctions
Px
Practice Questions

Loss of neurons in the major acetylcholinergic Alzheimer disease

nuclei is associated with what dementing
illness?
Acetylcholinesterase inhibitors
What class of drugs can be used for the
treatment of Alzheimer disease?
Hydrocephalus ex-vacuo
What is the name of the condition
characterized by ventricular dilation after
substantial neuronal loss, such as in
Alzheimer disease?
GROUP 8
 Diagnosis: Hemorrhagic stroke
Summary: Suspected hemorrhage from MCA
Intracerebral hemorrhage that is affecting right basal
Past Medical History: ganglia
Untreated HTN Chronic HTN is a risk factor for hemorrhagic stroke
Heavy alcohol use Will cause a mass shift that will often compress other
Frequent headaches structures
This is what caused uncal herniation
Physical Exam: affecting R oculomotor nerve
Treatment
Hypertensive - BP 250/120 :
Manage ICP w/ Mannitol, hyperventilation,
Unconscious
surgery
Displaying Cheyne-Stokes
Intubate to protect airway
Respiration Alcohol consult
Neuro exam:
Prognosis:
Right eye deviation due to R frontal eye field
Perihemorrhagic edema peaks 48-72 hours
damage
following insult
Left extensor posturing (decerebrate) due to
If patient survives this period, will likely
damage of R midbrain below level of red
survive
nucleus
CONTROL OF ICP IS CRUCIAL
Anisocoria due to compressive Right CN III paresis
Persistent right hemispheric defects likely if
resulting from uncal herniation
patient survives (left hemiplegia, left visual
Left side
Other important points: field cut, etc.)
Loss of consciousness due to bilateral hemispheric damage and damage to reticular activating system
Decerebrate posturing = red nucleus not intact
Decorticate posturing = red nucleus intact
Hyperventilation decreases ICP by decreasing PaCO2 -> arterial vasoconstriction -> decreased cerebral
blood flow and ICP
GROUP 14
SUMMARY: MS
Key History: Relapsing and remitting quality
of numbness, weakness, and
diplopia

Key Exam: Optic neuritis, INO, UMN signs

including hyperreflexia and
positive Babinksi signs,
sensory loss, ataxia/ positive
Romberg sign, and ataxia

Confirming diagnostic tests MRI showing acute or chronic

demyelinated plaques and/or
Lumbar Puncture positive for
oligoclonal bands of IgG

Treatments Treat the individual,not the

disease!
High dose corticosteroids
available for acute
exacerbations
Immune-modulating and
immunosuppressive
agents for long-term
manifestations of MS