1. Case Presentation
Kyle Carpenter, DO

2. 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

3. History PMH PSH Meds Social 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

4. Exam Vitals General Exam - unremarkable BP 173/87 HR 77 RR 16
99% on RA
General Exam - unremarkable

5. Neurologic Exam Mental status Speech Cranial Nerves
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

6. Motor exam Sensation Reflexes Coordination
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

7. Where?
What?
Who?

9. 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

10. Eponymous Brainstem stroke Syndromes

11. 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

12. 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

13. 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

14. 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)

16. 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

17. 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”

18. 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

19. 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)

20. 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

21. First described by Gaspard Vieussex in 1808 but Adolf Wallenberg described clinical manifestations and autopsy in 1901

22. 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 Name
Dejerine’s Onion Peel Sensory Loss
Dejerine cortical sensory syndrome
Dejerine- Mouzon Syndrome
Dejerine Klumpke paralysis
Dejerine Roussy syndrome
Dejerine Sottas disease
Dejerine Thomas olivopontocerebllar atrophy
Landouzy Dejerine sydrome

23. 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