Case Presentation

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

5. 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

6. 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

7. Where? • What? • Who?

9. Benedikt Syndrome • Stroke of the midbrain tegmentum • Affects the red nucleus and substanianigra 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) MortizBenedikt

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 mibraintectum are damaged • Most often caused by tumors (esp Pineal gland), also by hydrocephalusd, thalamic or midbrain hemorrohageor infarction, paraneoplastic encephalitis (anti MA2 abs), Wilson disease, Whipple disease, tuberculosus, drugs (Barbituates, carbamazepine and neuroleptics) • Ophthalmic findings • Vertical gaze abnormalities(espupgaze) • Setting sun sign • Primary position downbeat nystagmus • Impaired convergence and divergence • Convergence-retraction nystagmus • Pretectalpseudobobbing • Bilateral superior oblique palises • Fixation instability with square wave jerks • Bilateral upper eyelid retraction (tucked lid sign)

16. Foville Syndrome • Dorsal pontinetegmentum 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 lesionsespecially brachium pontis • Ipsilateral cerebellar ataxia • Contralateral hemiparesis • Variable contralatateralhemihypesthesia 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 • Ipsilateralfacial 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 DejerineKlumpke paralysis DejerineRoussy syndrome DejerineSottas disease Dejerine Thomas olivopontocerebllar atrophy LandouzyDejerinesydrome