DEMENTIA Flashcards

1
Q

A. The 1,090-g brain shows prominent atrophy of the frontal lobes.

B. Transverse slice shows severe atrophy of the hippocampal convolutions and enlargement of the temporal horns.

WHAT DSE

A

AD

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2
Q

PT HAS AD. WHAT ARE SHOWN?

A

Alzheimer’s disease. A. Neuritic plaques in the frontal cortex are demonstrated in silver-stained section (Bodian stain). B. Some

are noticeable in hematoxylin-eosin (HE)-stained section. C. Higher-magnifi cation view shows a fi brillary pattern (HE

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3
Q

WHAT IS SHOWN IN THIS PT WITH AD

A

Alzheimer’s disease.

A. Diffuse (primitive) neuritic plaque (Bodian stain)

(B) contains amyloid precursor protein

(APP) (immunostain

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4
Q

THIS IS A NEURITIC PLAQUE. DESCRIBE THE CHARACTERISTIC PATTERN

A

A. The central amyloid core stains positively with Congo red and gives

the characteristic Maltese-cross pattern in polarized light

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5
Q

WHAT REACTION IS SHOWN IN THIS PATIENT WITH AD

A

Periplaque glial reaction (HE), consists

of (H) astrocytes (GFAP–immunostain), and (I) rod-shaped microglial cells (HE–Congo red).

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6
Q

WHAT DOES THIS NEURITIC PLAQUE CONTAIN?

A

Alzheimer’s disease. Burnt-out neuritic plaque consists of

amyloid core only, lacking dystrophic neurites (Bodian stain

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7
Q

WHAT STRUCTURES ARE SHOWN

A

Neurofi brillary tangles (NF). A. Argyrophilic cytoplasmic tangles showing torch- and basket-shaped

confi gurations. B. Twisted bundles. C, D, and E. Residual ghost tangles and neuropil threads (Gallyas silver stain). F. Tauimmunoreactivity

of tangles (immunostain

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8
Q

AD PT

WHAT IS SHOWN?

A

granulovacuolar degen eration

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9
Q

AD PT

WHAT STRUCTURE

A

HIRANO

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10
Q

AD PT

WHAT PROCESS IS SHOWN

A

Prominent replacement astrocytic

gliosis

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11
Q

AD PT

WHAT DEPOSIT

A

Amyloid angiopathy. Congo red–positive

amyloid deposits around and within the walls of small cortical

blood vessels (Congo-red stain).

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12
Q

A man diagnosed with dementia and Parkinson’s disease at age 55 years steadily deteriorated, at times reporting visual and auditory hallucinations. After an approximate 11-year clinical course, he died at age 66. A. Atrophy

of the frontal lobes

WHAT DSE AND WHAT STRUCTURES?

A

DLB

Lewy body in a cortical neuron (HE). C. Immunoreactivity of cortical Lewy bodies for α-synuclein

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13
Q

A 50-year-old man began to deteriorate mentally and, by age 55, was diagnosed with dementia. By age 62, he was mute, bedridden, and tube fed. Following a 15-year clinical course, he died at age 65. A. The 800-g brain shows the utmost degree of frontal and temporal lobe atrophy. B. The walls of the frontal lobes are reduced to a thickness of only 3 to 4 mm, and the anterior horns are enormously enlarged. C. The temporal lobes are severely atrophic, the cortex is spongy, and the temporal horns are markedly enlarged.

WHAT DSE

A

PICK

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14
Q

WHAT STRUCTURES ARE SHOWN

A

A. A Pick cell in temporal cortex displays swollen perikaryon and peripherally displaced nucleus

(HE).

B. Pick bodies appear as argyrophilic globular cytoplasmic inclusions, and

(C) are particularly numerous in the dentate gyrus (Bodian stain).

D. The Pick body immunoreacts for tau protein

(immunostain).

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15
Q

A. Ballooned, achromatic neurons in the hippocampus are devoid of Nissl substance (HE) and

(B) stain positively for tau protein (immunostain).

C. Astrocytic plaque in cerebral cortex (Gallyas stain).

WHAT DISEASE?

A

CBD

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16
Q

WHAT STRUCTURES IN PT WITH FTD

A

Amygdala shows (A) tau-immunopositive

neurons and (B) neuritic threads

(immunostain

17
Q

DESCRIBE THE IMMUNOREACTIVITY OF THESE STRUCTURES

A

A. Dentate gyrus displays neurons with

ubiquitin-immunoreactive inclusions.

B. Cortex shows ubiquitin-positive

threads (immunostain).

18
Q

WHAT DSE?

A. The anterior horn of the cervical cord is depleted of motor neurons.

B. The thoracic cord shows degeneration of the lateral and anterior corticospinal tracts (LFB-CV).

C. The spinal motor nerve root shows axonal and myelin degeneration (Holmes stain).

D. The skeletal muscle shows denervation with severely atrophic muscle fi bers arranged in groups (HE).

WHAT DSE?

A

ALS

19
Q

A 6-year-old boy suffered from generalized muscle wasting that had begun in early infancy. By 3 years of age, he was unable to sit, walk, or talk. Family history was negative. The medulla shows neuronal losses, neuronal atrophy, and neuronophagia in the hypoglossus nucleus (Cresyl violet stain)

WHAT CONDITION?

A

Infantile spinal muscular atrophy

20
Q

A 58-year-old man gave a 3-year history of diffi culty swallowing and 50- pound weight loss over the past 2 years.

On admission, he appeared emaciated, with severe generalized muscle wasting, especially of the small hand muscles. Fasciculations were noted in the arm and neck muscles. Refl exes were hypoactive, and the plantar responses were fl exor. Sensation was normal. The speech was hoarse. He died suddenly of hemorrhagic bronchopneumonia.

Family history was negative for neuromuscular diseases.

A. The hand shows atrophy of the small intrinsic muscles. B. Medulla shows neuronal losses in the hypoglossus nucleus.

C. Cervical cord shows losses of motor neurons (LFB-CV stain).

WHAT CONDITION?

A

Sporadic adult onset bulbospinal muscular atrophy

21
Q

PT WITH PD.

LEWY BODIES SHOW IHC REACTIVITY TO?

A

α-synuclein

22
Q

PT WITH PD.

LEWY BODIES SHOW IHC REACTIVITY TO?

A

UBIQUITIN

23
Q

WHAT STRUCTURE IS SHOWN IN THIS PD PT?

A

Hyalin body is present in a

pigmented neuroN

24
Q

PT WITH PD

WHAT STRUCTURE?

A

Locus ceruleus shows a Lewy body

25
Q

65-year-old man presented with a 6-year history of diffi culty walking and frequent falls, inability to look up or down, diffi culty speaking and swallowing, and declining memory. Family history was not contributory. On examination, he was not able to articulate words, but was able to follow simple commands. The vertical and horizontal eye movements were severely restricted. The muscle tone was increased in the neck, trunk, and extremities, but strength was good. No tremor was noted. He needed assistance to rise from a chair and ambulate. The gait was slow and shuffl ing. Tendon refl exes were brisk, plantar refl exes were fl exor, and the vestibulo-ocular refl ex was present. He died at age 66 following a 7-year clinical course. Midbrain shows degeneration of the substantia nigra (HE).

A

PSP

26
Q

PT HAS PSP

WHAT DO YOU CALL THIS STRUCTURE

A

NF TANGLES

27
Q

PT HAS PSP

DESCRIBE IMMUNOREACTIVITY

A

Tau-positive

neurons are present in the thalamus

(immunostain).

28
Q

PT HAS PSP.

DESCRIBE THE GLIAL INCLUSIONS

A

Progressive supranuclear palsy. Glial inclusions.

A. Astrocytes display argyrophilic (Gallyas stain) and (

B) tau-immunoreactive

inclusions (astrocytic plaque; immunostain). C. Oligodendrocytes display argyrophilic coiled bodies (Gallyas stain

29
Q

WHAT CONDITION

A and B. The putamen and the caudate

show marked atrophy and myelin losses

(LFB-CV), (C) neuronal losses (HE), and

(D) dense astrogliosis (Holzer stain).

A

HUNTINGTON

30
Q

a 5-year-old boy whose older brother,

aged 15 years, suffered from a similar illness. Spinal cord

shows degeneration of the posterior columns, pyramidal

tracts, and spinocerebellar tracts (Weil stain).

WHAT DSE?

A

Friedreich’s ataxia

31
Q

From the age of 9 years, he was confi ned to a wheelchair because of progressing gait ataxia. His medical history was remarkable for multiple episodes of infection. He died of pneumonia following resection of a pulmonary abscess. The cerebellar cortex shows Purkinje cell losses, empty basket cells and replacement Bergmann astrocytosis (Bodian stain).

WHAT CONDITION?

A

AT

32
Q

Cerebellum shows A. severe Purkinje cell losses and prominent Bergmann astrocytosis (HE), B. Axonal spheroid (torpedo) of the degenerated Purkinje cells (Bodian stain). C. Argyrophilic (Gallyas stain). D. Ubiquitin-positive cytoplasmic inclusions in oligodendrocytes of the white matter (immunostain)

WHAT CONDITION?

A

MSA

33
Q

PT HAS MSA.

WHAT INCLUSIONS?

A

argyrophilic conical cytoplasmic inclusions in oligodendrocytes (Gallyas stain

34
Q

PT HAS MSA

DESCRIBE IMMUNOREACTIVITY

A

α-Synuclein–immunoreactivity of inclusions

35
Q

Medulla shows (A) loss of myelin in olivocerebellar fi bers and inferior cerebellar brachia (LFB-CV),

(B) neuronal losses in the inferior olivary nuclei (LFB-CV), and (C) dense astrogliosis (Holzer stain).

WHAT CONDITION

A

MSA

36
Q

PT HAS MSA.

WHAT PART OF THE SC IS AFFECTED

A

Multiple system atrophy. Thoracic spinal cord shows loss

of sympathetic neurons in the intermediolateral column

37
Q
A