Transcript Presentation

Patient SP
Clincopathologic Conference (CPC)
9/17/2015
Neurology Resident: Deepak Soneji
Pathologist: Dr. Ronald Hamilton
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CC: cognitive decline, personality changes, hallucinations
HPI: 60 yo F with h/o HTN who presented to the ED per
recommendation of her outpt neurologist for ~3 months of
cognitive decline. Per husband, sx may have started 8 months
prior to admission, with episodes of staring when asked a
question or simply not responding when family tried to speak to
her. Then 3 months prior to admission, pt and family took trip to
rural northern PA where pt was noted to be more withdrawn,
exhibited evidence of visual + auditory hallucinations, and made
nonsensical statements. Sx acutely worsened in the 4 weeks
prior to presentation, where pt was essentially sleeping all day.
Her gait was noted to be unsteady, though pt did not have any
falls. In the last 2 weeks, pt had become completely dependent
on her husband to perform all her IADLs (e.g. pt didn’t know
what to do when handed a utensil or a piece of toilet paper).
Husband noted some urinary incontinence in the last week.
Some family members noted “twitching” of L forearm which
lasted ~30s-1 minute each.
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Further history: pt had been “stressed” in recent months due to
her mother being diagnosed with Alzheimer’s approximately 1
year ago; no h/o depression, anxiety, or substance abuse. No
new medications or access to other people’s meds. No recent
bug bites, rashes, fevers, chills, sweats, weight loss, headaches,
CP, SOB, cough, abd pain, n/v/d
Remainder of ROS: per husband, negative
Histories
PMH/PSH: HTN
Fam Hx: Mother with unknown thyroid d/o, Alzheimer’s
dementia (diagnosed in her 80s)
Soc Hx: used to work as telephone operator but currently
unemployed, former 0.5ppd smoker for ~5 years, rare EtOH,
no illicits; lives in Johnstown, PA with husband; no children
Meds: HCTZ 25mg, no OTCs or herbals
Allergies: None
Physical Exam
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T 37.4C, BP 125/82, P 83 and regular, RR 16, O2 97% on RA
GENERAL: NAD, somnolent but easily arousable, oriented to person
only
EYES: no scleral injection, no discharge or icterus
ENT: Oral mucosa dry, pharynx without erythema or exudate
NECK: Supple, non-tender, no LAD, no thyromegaly
LUNGS: Lungs clear to auscultation bilaterally, no crackles or wheezes
appreciated
HEART: normal rate, reg rhythm, no m/g/r, no peripheral edema
ABDOMEN: Non-distended, no stigmata of liver disease, +BS, no
organomegaly, NT, no rebound or guarding
SKIN: warm, dry, without rashes, no jaundice
EXTREMITIES: Without tenderness or deformity, no effusions
PSYCH: flat affect, minimally conversant, poor eye contact
Physical Exam
• MS: Somnolent but easily arousable, orientation as above. Language
fluent but makes nonsensical statements. Attention poor. Follows
commands inconsistently. Naming intact. Cannot spell "WORLD"
backward or perform serial 7s, recalls 0/3 words; +glabellar, +BL grasp
• CN: PERRL. EOMI without nystagmus. +blink to threat. Facial
sensation intact to LT. Facial muscles full and symmetric. Hearing intact
to conversation. SCMs and shoulder shrug normal. Tongue midline.
• MOTOR: Normal bulk, increased tone and spasticity of UE BL. No
pronator drift. Trace low amplitude rhythmic movement of L forearm
(only observed this 1x during hospital course)
• UE strength 5/5 deltoids, biceps, triceps, hand grip bilaterally
• LE strength 5/5 iliopsoas, gluteals, hamstrings, quadriceps, tibialis
anterior, gastrocnemius
• REFLEXES: 3+ at biceps, triceps, brachioradialis, patella, and achilles.
No clonus. Flexor plantar response bilaterally. Hoffman negative.
• SENSORY: Withdraws all 4 extremities to noxious stimulation
• COORDINATION: Did not participate in FTN or HTS
• GAIT: limited exam, required assistance x2 to sit up from bed
Differential Diagnosis
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Infectious vs. autoimmune encephalitis
CJD
Sarcoid
CNS lymphoma
NPH
Neurodegenerative d/o such as DLB
CNS vasculitis
Initial Labs
• CBC: WBC 6.7, Hgb 12.9, Plt 138
• BMP: Na 159, K 3.6, Cl 123, CO2 25, BUN 30, Cr 0.9
• ALT 107, AST 61, AP 62, Tbili 0.7, Albumin 3.4
Imaging
Imaging
Diagnostic Workup
• ESR 72, Anti-TPO 18 (normal<10), TSH WNL but Free T4 low (0.74)
• NH3, copper, ceruloplasmin, Pb, serum ACE, ANA, RF, SSA/SSB,
Lupus ab, B12, anti-thyroglobulin ab, Lyme ab, RPR, HIV ab, Hep
A/B/C screen, UA, UDS, serum autoimmune encephalitis panel
unremarkable
• SPEP showing elevating a2 and gamma globulins but no monoclonal
proteins; UPEP neg
• LP: OP 14 cm H20, 15 WBCs (95% lymphs), glucose 67, protein 105
• Cytology neg
• VZV, HSV, EBV PCRs neg, bacterial and fungal cx NG; West Nile
ab, Crypto ag, India ink stain, AFB cx neg (OSH)
• OCBs and elevated IgG index
• Autoimmune encephalitis panel neg
• EEG: mod generalized slowing, no focal slowing, no seizures or
epileptiform discharges
• CT C/A/P w/ contrast: incidental BL PEs w/o cardiac strain, 1.2cm R
thyroid nodule, no lymphadenopathy, no suspicious masses
• LE venous duplex: BL DVTs
Hospital Course
• Pt started on IVF to correct free water deficit
• Empiric IV solumedrol 500mg q12 for planned 5d course
• no significant improvement after 3d
• Heparin gtt - > therapeutic Lovenox for PE/DVT
• WBC rose to 17 on day 5, but we attributed this to steroids
• CXR neg, UA pos -> started pt on cefuroxime
• By day 5 of admission, transaminases continued to rise
• RUQ u/s w/ dopplers -> no evidence of cirrhosis, no thrombosis
• GI consulted
• On the evening of day 5, pt became tachypneic and hypotensive
• Condition C called; by the time CCM arrived, pt was apneic and in
pulseless arrest
• CPR, urgent intubation, CCM team noted ?coffee ground emesis in
airway
• Stat labs revealed Hgb 4.0, Plt 54, Lactate 26, ABG w/ pH of 6.95
• pt transferred to NICU where she continued to deteriorate despite
multiple pressors, bicarb drip, and transfusions; family contacted
and eventually made pt CMO
• Autopsy report revealed massive spontaneous LLQ retroperitoneal
bleed; no GI bleed; no occult malignancy
Predicted Pathology
 Gross specimen
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-edema in amygdala, temporal lobes, thalami
Microscopic
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Perivascular inflammation
-lymphocitic infiltration
-viral inclusions?
Virtual Microscopy
 2A H&E, CD68, IBA1, NeuN, NF,
GFAP, CD20, CD4, CD8