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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
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