Case Presentation
Kyle Carpenter, DO
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Older yo right handed Caucasian female
Came to ER after she awoke with double vision
She also had noticed a tremor in her left arm
She had no history of ocular disease, no prior history of
anything like this in the past
No history of tremor. Worse with intention
Diplopia goes away when closing one eye
She did not notice any weakness, numbness or tingling in
any extremity
She went to bed the night before in good at 10pm, did not
awake at all during the night and awoke at 6am she arrived
to the ER at 7:30am
History
• PMH
– Type II diabetes insulin
dependent
– Hypercholesterolemia
– Hypertension
– Coronary artery disease (2
stents about 4 years ago)
• PSH
– Lap chole
– C section
– Appendectomy
• Meds
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Aspirin 81mg qday
Simvastatin 20mg qday
Lisinopril 10mg qday
Metformin 1000mg bid
Lantus 10 Units qhs
Aspart insulin 4 units with
each meal
– Multivitamins
• Social
– Non smoker, non drinker, no
drugs
– Married, lives with husband
Exam
• Vitals
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BP 173/87
HR 77
RR 16
99% on RA
General Exam
- unremarkable
Neurologic Exam
• Mental status
– Fully awake, alert and
oriented
• Speech
– Fluent, clear,
comprehension, naming
repetition are intact
• Cranial Nerves
– Right pupil was dilated at
7mm and fixed (no
response to direct or
consensual light), left pupil
was 5mm and reactive
– Visual fields were full to
confrontation
– Right eye showed lateral
strabismus and could not
cross midline when
attempting to look to the
left
– Left eye position was
normal
• Motor exam
– Abnormal involuntary
movements on left upper
extremity (choreiform)
– Drift on left leg and arm
– Left upper and lower
extremities had 4/5
strength
– Right extremities were 5/5
• Sensation
– Intact to light touch,
pinprick
• Reflexes
– 1+ throughout
• Coordination
– Tremor on left
– Normal on right
• Where?
• What?
• Who?
Benedikt Syndrome
• Stroke of the midbrain
tegmentum
• Affects the red nucleus
and substania nigra and
fasiscular portion of CNIII
• Occlusion of PCA
perforators
• Ipsilateral CN III palsy and
contralateral involuntary
movements and
hemiplegia (if it affects
the corticospinal tracts)
Mortiz Benedikt
EPONYMOUS BRAINSTEM STROKE
SYNDROMES
Weber Syndrome
• Similar to Benedikt’s
but more severe
contralateral weakness
• Also associated with
third nerve palsy with
dilated pupil
• Can also affect the
corticobulbar tracts
• PCA perforators
Sir Hermann David Weber
Claude’s Syndrome
• More dorsal than
Benedikt
• Red Nucleus
• Dentothalamic nuclei
within superior cerebellar
peduncle
• CN III fasiscles
• Ipsilateral CNIII palsy
• Contralateral hemiataxia
and dysmetria tremor
Henri Charles Jules Claude
Nothnagels Syndrome
• Superior Cerebellar
Peduncle
• Contralateral cerebellar
ataxia
• Ipsilateral third nerve
paresis (can also have
bilateral)
• More often associated
with mass occupying
lesions of midbrain
Parinaud Syndrome
• Dorsal midbrain syndrome
• Superior colliculus and mibrain
tectum are damaged
• Most often caused by tumors
(esp Pineal gland), also by
hydrocephalusd, thalamic or
midbrain hemorrohage or
infarction, paraneoplastic
encephalitis (anti MA2 abs),
Wilson disease, Whipple disease,
tuberculosus, drugs (Barbituates,
carbamazepine and neuroleptics)
• Ophthalmic findings
– Vertical gaze abnormalities (esp
upgaze)
– Setting sun sign
– Primary position downbeat
nystagmus
– Impaired convergence and
divergence
– Convergence-retraction nystagmus
– Pretectal pseudobobbing
– Bilateral superior oblique palises
– Fixation instability with square
wave jerks
– Bilateral upper eyelid retraction
(tucked lid sign)
Foville Syndrome
• Dorsal pontine
tegmentum in caudal
third
• Basilar artery perforators
• Facial N (VII) fascicle,
PPRF, corticospinal tract
• Ipsilateral peripheral VII
palsy, gaze paralysis,
contralateral hemiparesis
Raymond Cestan Syndrome
• Rostral lesion of dorsal
pons
• Affects
– Medial leminscus and
spinothalamic tract
– cerebellar peduncles
– MLF
– Ventral extension can
affect corticospinal tracts
• Signs
– INO, CL hypesthesia to face
and extremities, cerebellar
sings with “rubral tremor”
Millard-Gubler Syndrome
• More anterior than
Foville- spares the
abducens nucleus but
affects the fascicles
• Ipsilateral peripheral VII
• Ipsilateral lateral rectus
• Contralateraal
hemiplegia
Millard
Gubler
Marie-Foix Syndrome
• Lateral pontine lesions
especially brachium
pontis
• Ipsilateral cerebellar
ataxia
• Contralateral hemiparesis
• Variable contralatateral
hemihypesthesia for pain
and temp
• (different from FoixChavany-Marie
syndrome)
Wallenberg Syndrome
• AKA lateral medullary syndrome
• Intracranial vertebral artery or
PICA
• Spontaneous dissection of vert a.
are most common cause
• Also with cocaine, medullary
neoplasm, abscess, demylinating,
radionecrosis, hematoma, neck
manipulation, bullet injury
• Affects
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Trigeminal spinal nucleus and tract,
spinothalamic tract
Nucleus ambiguus
Descending sympathetic fibers
Vestibular nuclei
Inferior cerebellar peduncle
• It has a variety of presentations
depending on size of infarct
• Ipsilateral facial hypalgesia and
thermoanesthesia
• Contralateral trunk and extremity
hypalgesia and thermoanesthesia
• Ipsilateral palatal, pharnygeal and
vocal cord paralysis
• Ipsilateral Horner syndrome
• Vertigo, nausea and vomiting
• Ipsilateral cerebellar signs
• Hiccups, diplopia
First described by
Gaspard Vieussex in
1808 but Adolf
Wallenberg described
clinical
manifestations and
autopsy in 1901
Dejerine’s Syndrome
• Medial medullary syndrome,
inferior alternating syndrome
• Vetrebral artery, anterior spinal
artery or lower segment of basilar
• Pyramid, medial lemniscus,
hypoglossal nerve and nucleus
• Ipsilateral paresis, atrophy
Joseph Dejerine
fibrillation of tongue,
• Contralateral hemiplegia (spares Also to his Name
face)
Dejerine’s Onion Peel Sensory Loss
• Contralateral loss of
Dejerine cortical sensory syndrome
propioception and vibration
Dejerine- Mouzon Syndrome
• Can affect the MLF and cause
Dejerine Klumpke paralysis
upbeat nystagmus
Dejerine Roussy syndrome
• Can also occur bilaterally
Dejerine Sottas disease
Dejerine Thomas olivopontocerebllar atrophy
Landouzy Dejerine sydrome
Midbrain
Weber
Oculomotor palsy with contralateral hemiplegia/paralysis
Claude
Oculomotor palsy with contralateral tremor, ataxia
Benedikt
Oculomotor palsy with contralateral involuntary movements and hemiplegia
Nothnagel
Oculomotor palsy with contralateral ataxia
Parinaud
Upward gaze paralysis, ophthalmic findings
Pons
Foville
peripheral VII, gaze paralysis, contralateral hemiplegia
Raymoond Cestan
INO, sensory findings, cerebellar findings
Millard Gubler
Peripheral VII, CN VI palsy, contralateral hemiplegia
Marie Foix
Ipsilateral cerebellar ataxia, contralateral hemiplegia, variable sensory findings
Medulla
Wallenberg
facial hypalgesia, contral trunk sensroy findings, ipsilateral horner and cerebellar signs
Dejerine Syndrome
Tongue findings, contralateral loss of propioception and vibration, upbeat nystagmus