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 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

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

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

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

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 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

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