Forensic Neuropathology

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Consultation Front Page

CLIENT CASE # DECEDENT NAME NAAG CASE # CONSULTANT

NOK Kathrine KOHRMANN 2020-0106 Vivian Snyder

DATE OF BIRTH DATE OF DEATH DATE OF AUTOPSY DATE OF SUBSPECIALTY EXAM

04/15/1949 09/01/2020 09/05/2020 N/A

SEX AGE REQUESTING PATHOLOGIST ORIGINATING AGENCY

F 71 years Sam Andrews MD FRCPC Lubbock County Medical Examiner

REPORT STATUS REPORT FINAL DATE RELATED CASES

***FINAL*** 02/06/2021 2020-0106,

Forensic Neuropathology

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Report of Findings

BRAIN AND DURA MATER:

1. Severe Alzheimer's disease 2. Bilateral hippocampal sclerosis 3. Cerebrovascular arteriolosclerosis

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Summary & Opinion

According to the investigative reports, the decedent was a 71-year-old woman with a medical history including dementia, oral cavity cancer, salivary gland cancer, metastatic lung cancer,

hypothyroidism, chronic kidney disease, and osteoporosis.

Formal neuropathologic evaluation demonstrated features of severe Alzheimer's disease and hippocampal sclerosis. Alzheimer's disease (AD) is the most common cause of dementia. There are five main groups of AD: (1) sporadic late onset, (2) familial late onset, (3) familial early onset, (4) associated with Down syndrome, and (5) associated with other degenerative diseases. Sporadic late onset is the most common form, and is the most likely type in this case. Several genes increase the risk of development of AD. These genes interact with environmental factors that may result in AD. Familial AD is rare and is known to involve three genes (APP, PSEN1, and PSEN2); these patients usually develop early onset AD.

The family provided the following list of questions for my consideration:

(1) What type of Alzheimer's and/or dementia she had with as much information as possible

Mrs. Kohrmann had severe AD and bilateral hippocampal sclerosis. More likely than not, based on the totality of available pathologic information, this is "sporadic late onset"-type AD. Hippocampal sclerosis may occur by itself or with many other conditions including temporal lobe epilepsy, multiple sclerosis, Alzheimer's disease, cardiovascular disease, frontotemporal dementia, and amyotrophic lateral sclerosis. Hippocampal sclerosis also causes memory loss.

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Summary & Opinion

symptoms of the disease.

The disease was very far advanced by the time of death, and the features of the disease were very widespread and severe.

(3) Did she suffer from a particular type of dementia that is genetic?

Brain-only autopsies are unable to test for the genetics of AD. However, it is unlikely that Mrs.

Kohrmann suffered from the familial (genetic) type of AD, given that it is rare and most often presents as early onset (i.e., much earlier in life).

Declaration

Tasks

I declare that I personally performed or directly supervised the tasks described in this report. It is only after careful consideration of all the data available to me at the time this report was finalized that I attest to the diagnoses and opinions stated herein. My opinions and conclusions may change if I am presented with additional information.

Photos

Numerous photographs were obtained along the course of the examination. I have personally reviewed those photographs and attest that they are representative of the findings reported in this document.

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Neuropathology (brain/dura mater)

Date specimen received: 9/5/2020

Date of examination: 10/08/2020

Individual(s) present at examination: Vivian Snyder DO, Lisa Hang

Materials available for examination: Brain and dura mater

Method of dissection: Coronal (cerebrum), axial (brainstem

and cerebellum)

Weight (grams): 1080

Evidence of medical intervention

None

Evidence of injury

None

Postmortem changes

None

General

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Neuropathology (brain/dura mater)

The smooth pachymeninges have no masses. The dural venous sinuses are patent. The brain is covered by smooth, focally fibrotic leptomeninges and is without epidural, subdural, or subarachnoid hemorrhages. The leptomeninges overlying the right hemisphere are congested. The superficial cortical vasculature has no thromboses or vascular malformations.

The gyral convolutions and the sulci are appropriately configured. There is generalized cortical atrophy (frontal and temporal greater than parietal and occipital). The unci and bilateral cerebellar tonsils are without parenchymal softening, hemorrhage or necrosis. The vessels at the base of the brain are dolichoectatic and have no aneurysms or atherosclerotic lesions. The cranial nerve roots are symmetric and normally distributed.

The cortical ribbon is intact, without contusion, and is thinned; some areas have a spongy appearance. The gray-white matter junctions are distinct. The amygdalae and hippocampi are atrophic (right greater than left). The ventricular system is markedly enlarged; the superior angles of the lateral ventricles are blunted. The internal capsules, deep gray nuclei, mammillary bodies, superior cerebellar vermis, cerebellar parenchyma, brainstem structures and the proximal cervical spinal cord are of normal configuration. The substantia nigra and the locus ceruleus are slightly pale. The brain parenchyma is without neoplasm, cyst, abscess, or hemorrhage.

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Microscopy

The dura mater is unremarkable.

The neocortex of the frontal and temporal lobes is thinned and has mild to moderate neuron loss, gliosis, and vacuolization/spongy change. Microglial populations are conspicuous. The neuropil is littered with neuritic plaques. The subcortical white matter is pale and rarefied and has varyingly prominent populations of corpora amylacea. The parietal and occipital lobes have relatively well-maintained neuronal populations, but also have abundant neuritic plaque populations.

The blood vessels of the cortex, subcortical white matter, cerebellar white matter, and basal ganglia are thick and hyalinized; some have "stove-pipe" configuration. Many of the blood vessels within the subcortical white matter, cerebellar white matter, and basal ganglia sit within wide perivascular spaces.

The anterior hippocampal formations have severe neuron loss; the pyramidal cell layer is not identified. Neuronal clusters in layer 2 of the entorhinal cortices are severely depopulated and are replaced with ghost tangles (extracellular neurofibrillary tangles); only a few neurons remain. The amygdalae have moderate to severe neuron loss, gliosis, increased microglial populations,

abundant neuritic plaques, and abundant ghost tangles. The pyramidal cell layer of the posterior hippocampal formation has mild to severe neuron loss. CA4, CA3, and CA2 have mild to moderate neuron loss, scattered neuritic plaques, ghost tangles, and rare intracellular tangles. CA1 of both (bilateral) pyramidal cell layers has severe neuron loss and accompanying reactive gliosis. In some places the neuron loss is near-total and is associated with linear cavitation, extending into the subiculum. Extracellular neurofibrillary tangles are scattered within these areas, but do not appear to account for the severe neuron loss.

The brainstem structures are appropriately configured. The substantia nigra pars compacta has severe neuron loss and gliosis. Extra-neural neuromelanin is conspicuous. Scattered ghost tangles are identified. The locus ceruleus has mild to moderate neuron loss; tangles are not obvious.

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Microscopy

The cerebellum is appropriately configured. The dentate nucleus has at least mild neuron loss.

Some of the neurons are shrunken and hyperchromatic.