10/25/ weeks 0.5%-5% 90% unilateral, 10% bilateral. LOCATION = OPTIC CANAL; 71% of the time

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Sylvia Davidson
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3 90% unilateral, 10% bilateral 3-6 weeks 0.5%-5% LOCATION = OPTIC CANAL; 71% of the time 3

4 IV steroids <8 hours of injury = greatest chance of motor/sensory improvement in comparison to placebo and naloxone 4

5 57% of observation group 52% of steroid group 32% of decompression group No clear statistical benefit between any of the treatment groups increased risk of death 40%-72% 5

6 1. National Acute Spinal Cord Injury Study 2 (NASCIS 2) (1990) 2. National Acute Spinal Cord Injury Study 3 (NASCIS 3) (1997) High-dose steroids within 8 hours of injury = greatest recovery chance Increased risk of sepsis and pneumonia with high-dose steroids in these patients 3. International Optic Nerve Trauma Study (IONTS) (1999) 4. Corticosteroid Randomization After Significant Head Injury (CRASH) (2004) 1. Observation is best option currently 57% of TON spontaneous improve on there own at least somewhat } Both Tx are NOT yet proven better than observation alone provide a very cogent rationale for with-holding high-dose methylprednisolone in TON There is sufficient evidence to conclude that neither corticosteroids nor optic canal surgery should be considered the standard of care for patients with TON. ---Levin et al. (IONTS study) (1999) 6

7 Promising for sure, not proven yet 7

8 only 1-2% of patients with nonglaucomatousvf defects have abnormalities in the peripheral field beyond 30 degrees in the absence of central field defect 98-99% of neurological VF defects will show up in the central 30 when tested Kedar S, Ghate D, Corbett JJ. Visual fields in neuro-ophthalmology. Indian J Ophthalmol. 2011;59:

9 ipsilateral MLF 9

10 midbrain pons 10

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12 One-And-Half Syndrome 12

13 Lesion = inferior pons! A. Lower motor neuron lesion = half of face involved Nuclear/fascicle/ nerve lesion B. Upper motor neuron lesion = lower 1/4 face involved Supranuclear lesion Forehead is the key! My patient can be localized to lower motor neuron based on total right side of face involvement. 13

14 Decussation of corticospinal tract = upper medulla! Millard-Gübler Syndrome } ventral paramedian pons Ipsilateral Abducens nerve or gaze palsy Ipsilateral CN VII/Facial palsy Horner s Syndrome Analgesia of face (CN V) Peripheral deafness (VIII) Loss of taste (V-mandibular branch) Lesion = dorsolateral pons Only V, VI, VII, VIII are involved 14

15 Case History 15

16 Initial Exam Dilated Fundus Exam Left Pontomedullary Junction = lesion location 16

17 MRI Results arrow yellow MRI Results arrow yellow MRI Results yellow arrow 17

?? Corticospinal fibers are responsible for voluntary limb movement Origin: motor cortex of precentral gyrus

18 Lesion in Left Lower Pons (Transverse Cross Section) Lateral Lemniscus What about the contralateral hemiplegia??? Corticospinal fibers are responsible for voluntary limb movement Origin: motor cortex of precentral gyrus in frontal lobe Decussation fibers = upper medulla! Inferior to lesion in this case! Left pons lesion would lead to rightsided hemiparesis as in this case! 18

19 Quick Re-cap 4 components 1 ½ ½ = 17 Seventeen Syndrome Causes/Lesions of OAAH Syndromes Vascular Demyelinating } Most common causes Stats of One-And-A-Half Syndromes 8% 30% * 10% * 30% * 35% * Wall M, Wray SH. Neurology (Cleveland). 1983;33: * = this patient s clinical findings 19

20 8 ½ Syndrome 9 Syndrome 13 ½ Syndrome 15 ½ Syndrome 17 Syndrome Additional Labwork to Consider if CWS Linger 3 month follow-up exam Nine Syndrome findings slightly improved but still present Cotton wool spots resolved with improved systemic control Therefore, no lab work ordered at this time Follow up x 6 months. 20

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22 Courtesy of Dr. Ellen Nguyen New Orleans, LA 22

23 OS OD 23

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25 Immediate trans-femoral artery aneurysm coil repair 25

26 When in doubt, go back to the case history! 26

27 Drug addicts/iv drug users Immunosuppresive therapy Immunosuppresion Prosthetic heart valves (90-95%) (5-10%) Candida 27

28 1. Blood Cultures 4. Echocardiograms 28

29 80-94% mortality rate DOC = Amphotericin B Flucytosine (second DOC) Stroke Mycotic aneurysms Intracranial (15%) Subarachnoid hemorrhage Intracerebral hemorrhage Seizures 1. Oslerian MA 29

30 most common sites MCA } Most common 30

31 Candida species } Most common fungi 31

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33 82% of cases Coronal high-field T1-weighted 10.6 mm and 17.4 mm Wagner AL, et al. Measurement of the normal optic chiasm on coronal MR images. Am J Neuroradiol. April 1997;18:723-6 Normal T1 + C Coronal Abnormal T1 + C Coronal 33

34 optic chiasmal atrophy Case #11: 34

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36 Weber s Syndrome Two 36

37 One 2 lesions total! ) 37

38 Midbrain Blood Supply Posterior Cerebral Artery & Its Branches Occipital Lobe Blood Supply Posterior Cerebral Artery & Its Branches peduncle tract Weber s Cerebral corticospinal Benedikt s Ipsilateral 3 rd nerve palsy Contralateral ataxia/involuntary movements Red nucleus = lesion location 38

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40 Same day CT scan of orbits Orbital Pseudotumorsecondary to left medial rectus myositis 5-8% 40

41 Most common causes: Orbital cellulitis 2 most commonly confused with IOIS TRO } Less common but possible causes: Crohn s Disease Giant Cell Arteritis Histiocytosis X Idiopathic fibrosclerotic disorders Periarteritis nodosa Scleroderma Sclerosing cholangitis Churg-Strauss Syndrome IgG4-Related Disease Orbital Pseudotumor (myositis with entire muscle involved and scleral insertion) Vs. Thyroid Orbitopathy (belly of muscle only; (-)tendons involved) Signs of Orbital Cellulitis } 41

42 Dacryoadenitis Myositis } My patient had all 5! myotendinous junction surrounding fat, tendon, and Medial Rectus = 29% Siatkowski RM, Capo H, Byrne SF, et al. Clinical and echographic findings in idiopathic orbital myositis. Am J Ophthalmol. 1994;118:

43 Plasma Cells (derived from B cells) Lymphocytes (B and T cells) Macrophages (derived from Monocytes) Granulocytes / PMN s 37% 78% 20% 52% inadequate and outdated woefully 43

44 Infliximab (Remicade) Methotrexate Others Rituximab (Rituxan) Best/Recommended Order of Treatment (Carruth et al. 2012): Corticosteroids Methotrexate Infliximab Co-Management with Rheumatology is important for possible toxicities to meds! ANA Anti-dsDNA c-anca, p-anca Lyme titers RF Anti-CCP FTA-ABS RPR Thyroid Panel Serum Protein Electrophoresis IOIS can only can be considered once a thorough investigation for other entities has proved unrevealing. ---Jacobs et al. (2002) 44

Bisphosphonate-associated orbital inflammation a case report and review. Orbit. 2012;31:119-23.

45 Bisphosponate-Associated IOIS concomitant anterior iritis ~33% of patients within 3-6 days Bilateral 29% IOIS Peterson JD, Bedrossian EH. Bisphosphonate-associated orbital inflammation a case report and review. Orbit. 2012;31: IgG4-Related Disease (IgG4-RD): IOIS/myositis = most common New Association #2: IgG4-Related Disease IgG4 = only 4% of total IgG 96% IgG = IgG1, IgG2, IgG3 45

46 1) > 135 mg/dl 2) >30% Carruthers MN, Stone JH, Khosroshahi A. The latest on IgG4-RD: a rapidly emerging disease. Curr Opin Rheumatol. 2012;24:60-9. substantial proportion of cases of presumed idiopathic orbital pseudotumor in fact represent an ocular manifestation of IgG4-RD These patients warrant the separation of orbital manifestation of IgG4 systemic disease from the inflammatory orbital pseudotumor category. of lymphoma development increased risk Berry-Brincat A, Rose GE. Idiopathic orbital inflammation: a new dimension with the discovery of immunoglobulin G4-related disease. Curr Opin Ophthalmol. 2012;23:

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THE BRAINSTEM. Raymond S. Price, MD University of Pennsylvania

THE BRAINSTEM. Raymond S. Price, MD University of Pennsylvania THE BRAINSTEM Raymond S. Price, MD University of Pennsylvania Overview of Brainstem Functions The brainstem serves numerous crucial neurologic functions. The most clinically relevant functions include: