Case Presentation

Kyle Carpenter, DO

• 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– 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– 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 Foix-Chavany-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– 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

fibrillation of tongue, • Contralateral hemiplegia (spares

face)• Contralateral loss of propioception

and vibration• Can affect the MLF and cause

upbeat nystagmus• Can also occur bilaterally

Joseph Dejerine

Also to his NameDejerine’s Onion Peel Sensory LossDejerine cortical sensory syndromeDejerine- Mouzon SyndromeDejerine Klumpke paralysisDejerine Roussy syndromeDejerine Sottas diseaseDejerine Thomas olivopontocerebllar atrophyLandouzy 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