2/13/2015 Disclosures • None RAIN Conference 2015 Case Presentations Maulik Shah, MD UCSF Neurohospitalist Division Case #1, continued Case #1: Rapidly progressive weakness • He was at home with family stable on pain • 18 year old man who developed diarrheal illness medications until October, when he was taken and colitis while traveling in India in July to Emergency Department with worsening • GI illness improved but then he developed headache and high fever persistent and diffuse joint pains, oral ulcers, hair • He was admitted and the next day started to loss, frequent headache, and intermittent fever complain of tingling and weakness in his feet • Upon return to US, had work-up for tropical than quickly progressed to his thighs over few diseases which was unrevealing hours • Labs notable for new normocytic anemia and ESR • Noted to be areflexic > 100 1
2/13/2015 Question 1 Lumbar Puncture Results How would you manage this patient at this time? • The patient had lumbar puncture at that time A. Start antibiotics (Vancomycin and Ceftriaxone) at meningeal dosing and • Normal opening pressure 30% Acyclovir • WBC 21 (61% neutrophils) 26% B. Start antibiotics, Acyclovir, and 21% • Glucose 52 (serum 126) 18% steroids • Protein 388 C. Start high dose steroids 4% D. Start IVIg g s S . . d I . . V C E. Obtain urgent EMG/NCS . v i i I N m o o r t / o l r c e a G c y t n s t M c S a A e E V s t ( , o n s d s c e c i i t h g t o g r o u b i i b i h n i i t t i t n r a n a a b t a t t S O t r r a a t S t S Case #1, continued Initial Neurologic Examination • The patient was started on antibiotics and • Intubated on ventilator in the ICU Acyclovir • MENTAL STATUS awake and alert, able to shake head to answer questions and follows midline commands • Over next 24 hours, his flaccid weakness and • CRANIAL NERVES vfftc, PERRL, full extra-ocular tingling progressed to involve all limbs muscles, full facial strength with eyelid closure, and • He developed respiratory failure requiring able to lift his head out of bed intubation as well as worsening anemia • MOTOR flaccid paralysis of arms and legs requiring transfusion • SENSORY absent over limbs, trunk, buttocks and back but intact over upper neck and face • He was then started on IVIg and was • COORD no tremor or involuntary movements transferred to UCSF Neurology service • REFLEXES absent throughout in limbs with intact jaw jerk 2
2/13/2015 Question #2 Wh at diagnostic testing would you pursue at this time? 60% A. MRI Cervical and Total Spine B. Repeat lumbar puncture C. Urgent EMG/NCS 26% D. CT Cervical Spine 8% 7% S e e C n . r . . u N i p l t / S a c G t n M l o u a T c p E i v d r n a t r n e a b e C l m g a T c u r C U i l v t r e a e C p I e R R M Question #3 Repeat Lumbar Puncture Which diagnosis is LEAST likely at this time given neurologic examination, CSF and imaging • WBC 198 (76% neutrophils) findings? • Glucose 26 A. Neuromyelitis optica (NMO) 36% • Protein 233 28% B. SLE Myelitis C. Acute Disseminated 14% 13% • CSF gram stain negative Encephalomyelitis 10% • Prior CSF and blood cultures negative to date D. West Nile Encephalomyelitis s s E. VZV Myelitis ) i s i O t i t i . t i M l . i l e l e c . e y y N n y M M ( E m a d o V c E L e l Z t i a S t V p a h o n p i e s m i c t e n i l s E e s y e i m D l i e N o r t t u u s e c e A N W 3
2/13/2015 MRI of Spine Additional Serologic Work-up CSF Analysis: Elevated WBC or IgG index or OCB • HIV negative Vascular (Ischemic) • ESR > 100 Dural AV fistula Vitamin B12 deficiency • ANA 1:160 Nitrous Oxide toxicity Multple Sclerosis HSV Lymphoma Vitamin E deficiency • DS-DNA titers > 1:10,000 titer Neuromyelitis optica CMV Intramedullary tumors: Copper deficiency ADEM EBV -Ependymoma Adrenoleukodystrophy / • Low C3 and C4 complement levels SLE VZV -Astrocytoma Adrenomyeloneuropathy Sjogren’s HIV -Hemangioblastoma HIV vacuolar myelopathy • Normal lupus anti-coagulation and anti- Sarcoid WNV (AHC) Paraneoplastic Vasculitis HTLV (not always phospholipid antibody panels inflammatory) Enterovirus 71 Fungal: cocci, histo Tuberculosis • CSF HSV, VZV PCR negative Mycoplasma Lyme Syphilis SLE Myelitis Grey and White Myelitis • Occurs in 1% of patients with neuropsychiatric SLE, most commonly in women • Can be the first presentation of SLE • Longitudinally extensive > 3 segments in 70% of cases, often holo-cord involvement • In one study of 75 patients with neuropsychiatric SLE, only one had +NMO antibody – Patient had longitudinally extensive myelitis – Increased incidence of +NMO antibody in SLE and other rheumatologic disorders in patients with myelitis and/or optic neuritis • Two clinical phenotypes in terms of presentation 4
2/13/2015 SLE Myelitis: Clinical Subtypes • Grey matter myelitis: rapid onset weakness (often within 6 hours) with LMN findings of flaccidity and hypo-reflexia on presentation – First symptom was usually acute urinary retention – LMN signs persist over time • White matter myelitis: UMN findings of spasticity and hyper-reflexia Question #4 SLE Myelitis: Treatment How would treat this patient at this time? • No randomized controlled trials, most data from A. High dose steroids and Cyclophosphamide outcomes of case series for next few months, then Azathioprine • European League Against Rheumatism long-term 37% recommendations B. Plasmapheresis and prolonged steroid taper 27% – Early high-dose steroids and ideally 24% C. High dose steroids and then Rituximab if Cyclophosphamide (although LP will often be NMO returns positive concerning for infection) 13% – Anticoagulation in those with +APLS antibodies D. High dose steroids and Cyclophosphamide – Reasonable to use plasmapheresis in severe cases for next few months, then Rituximab if NMO returns negative • Several case reports suggest efficacy of Rituximab . . . . . . . C . . . C . o . t d r d n p d n n – NMO association and recurrent myelitis a d a a s n s a s d d i d i o s i o i o r s r e e r e e s t r s t e t h s e e s p e s o s o a o d m d d h h s h g a g i l g i H P H H i 5
2/13/2015 Prognosis: Grey vs. White Case #2: An attack of ataxia • Grey matter myelitis: despite getting more immune • 84 year old woman with rheumatic heart disease therapy, mean EDSS 8.0 (range 8-10) and hypertension who experienced abrupt onset – Irreversible paraplegia of difficulty holding playing cards – Wheelchair bound • Her voice then became slurred and slowed and • White matter myelitis mean EDSS 6.0 (range 2-10) over next few hours she was unable to walk – Dependent on ambulatory assistive device to walk without support • Went to Emergency Department and was felt to • Patient was treated with IV steroids and Cyclophosphamide have an acute stroke although MRI Brain showed • Our patient’s arm strength improved and he was extubated no evidence of ischemia • Continued on six months of Cyclophosphamide and then Azathioprine • Discharged home with adjustment of antiplatelet • Remains paraplegic with distal hand weakness and and initiation of statin bowel/bladder dysfunction Case #2, continued • At home, she had progressive symptoms – Double vision – Recurrent falls, unable to ambulate even with walker • Additional review of symptoms – 25 lb weight loss due loss of appetite – URI while visiting Hawaii two months prior Patient provided permission for video to be used for teaching purposes • 6
2/13/2015 Question #5 Lumbar Puncture Given patient’s examination, the imaging shown has most definitively ruled out which of the • Clinical examination was felt to be most following etiologies? consistent with pan-cerebellopathy A. Acute or subacute cerebellar stroke • CSF was collected as next part of diagnostic work- up and revealed B. Auto-immune cerebellitis 82% – WBC 30 (81% lymphs) C. Post-infectious viral cerebellitis – Protein 130 – Glucose 78 D. Creutzfeldt-Jakob Disease E. Miller Fisher variant AIDP • CSF cytology and flow cytometry negative 6% 5% 4% 3% • CSF EBV, HSV, VZV PCR negative s e . . s P . t i . D . i . a e l b e I b l e A e s e b r i t r e D n e c e r b a c e l i a o r e c k a t r e v i a v u n J c - r a u s t e u d h b m o u l s m i e i s t f F c z r - i e t r o o u e f e t n e l l u i i t A - r M u t C s c o A P 7
Recommend
More recommend
