Transcript Presentation

GM
Clincopathologic Conference (CPC)
11/20/2015
Neurology Resident: Thomas Shoemaker, MD
Pathologist: Julia Kofler, MD
History
• GM is a 70 year old retired salesman with a history of
CAD s/p CABG, OSA, and HTN who presented with a
three year history of cognitive difficulty:
– Initially had difficulty with general memory over several years
• Couldn’t follow conversation, difficulty with new applicances in the
home
– This was followed by word finding difficulty
– Per wife: “Had to practice friend’s names before going to lunch
with them”
– Started on Aricept with significant transient improvement for six
months
– Cognitive ability then plateaued for several months afterward
History (cont)
• One year ago he had quadruple bypass surgery,
followed by a dramatic decline in memory
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He gave up entirely with family investments and tax preparation
Spent less time on the computer
No longer able to drive due to confusion, becoming lost
Forgetful of details of conversation
He and his wife would have to fill a pillbox each week and she would
need to prompt him frequently to take his medications
PMH
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Coronary artery disease.
S/P quadruple bypass
Borderline diabetes. Recent A1C is within normal limits.
Increased cholesterol.
Status post bilateral cataract extraction.
Sleep apnea. Wife reports for his first memory evaluation in
2008 they recommended sleep studies and he was found to
have sleep apnea and is compliant with CPAP.
• Spinal stenosis.
• Probable past silent MI.
• Hypertension.
Family History
• Parents: No cognitive issues
• Sister, deceased, age 86 of Alzheimer’s Disease
• Brother, living, CAD
Social History
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1-2 PPD smoker until age 60
Occasional Wine
No recreational drugs
Retired salesman and teacher
15+ years of education (Masters equivalaent)
Medications
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Lisinopril
Simvastatin
Namenda
Aricept
Vitamin D
ASA
Plavix
Lopressor
20 mg
20 mg
10 mg
10 mg
1000 units
81 mg
75 mg
25 mg
1 q day
daily
b.i.d.
q.h.s.
daily
daily
daily
b.i.d.
Examination
• Mental Status: Listed age correctly as 70 and was
incorrect on year of birth. Had some difficulty dating
date of bypass surgery but was able to state 2-3
years ago. When asked current month, he said ‘the
first one.” For the year he stated as 20_0 but then
corrected himself. He was unable to calculate
quarters in $6.75. Significant word-finding difficulty
was present. He needed cues to name items such as
cuff and collar on a shirt.
Examination
• CN: Normal
• MOTOR: Strength was symmetrical throughout. Motor tone
was unremarkable. There was no cog wheeling. On the left
extremity only, there was action and sustention tremors.
There were no tremors on the right
• SENS: Vibratory sense was decreased to the ankle. Patient
missed one item on Stereognosis and was not able to name
paper clip
• REFLEX: All deep tendon reflexes were symmetrical
throughout. There were no frontal release signs
Examination
• COORD: Cerebellar function was intact including the finger to
nose and heel to shin.
• GAIT: Able to complete tandem walking. Casual gait did show
lack of arm swing with slight shuffling gait. There was also 1-2
steps backwards and postural instability but he recovered
spontaneously
Examination
• MMSE: 17/30
Modified Rey-Osterrieth Figure
• The ROCF consists of
three test conditions:
Copy, Immediate Recall
and Delayed Recall
• Tool for measuring
executive function that is
mediated by the
prefrontal lobe
Trails making test A
• Scoring: reported as the
number of seconds
required to complete the
task; therefore, higher
scores reveal greater
impairment.
• Average <29 sec
• Deficit >79 sec
• Provides information on
visual search, scanning,
speed of processing,
mental flexibility, and
executive function
Imaging
• MRI – 2/6/2008 - Scattered white matter changes, small left
basal ganglia lacune
Differential Diagnosis
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Vascular dementia
Alzheimer’s Dementia
dementia with Lewy bodies
frontotemporal dementias
Creutzfeldt-Jakob
Predicted Pathology
• Predicted Gross pathology:
– Diffuse gyral atrophy and ventricular dilatation, with most severe
involvement of the temporal cortex, amygdala, hippocampus, and
entorhinal cortex with relative sparing of the occipital lobe
• Predicted Micro pathology:
• NFT, most numerous in the hippocampus, amygdala, and entorhinal
cortex, but usually also in the neocortex
– NFTs are intraneuronal accumulations of PHF-tau
• Diffuse plaques and neuritic plaques found throughout the
neocortex
– Neuritic plaque contains Aβ protein, often forming a dense central core of
amyloid, surrounded by distorted neurites usually containing paired helical
filament–tau (PHF-tau) protein
• Hirano bodies
Virtual Micro
• Frontal lobe Slide A
– H&E
– Ab
– Tau
• Hippocampus Slide E
– H&E
– A beta
– Tau
• Cerebellum Slide F
– H&E
– A beta
• Midbrain Slide G
– H&E
– Abeta
– GFAP
– CD68