Eponyms

Question 1

Eosinophilic cytoplasmic inclusions in degenerating hepatocytes; represents tangled skeins of intermediate filaments

Answer 1

Mallory bodies

Seen in alcoholic liver disease, primary biliary cirrhosis, Wilson Disease, chronic cholestatic syndromes and hepatocellular CA.

Question 2

Increased iron intake which causes secondary hemochromatosis

Answer 2

Bantu siderosis

Question 3

Green to brown deposits in Descemet Membrane in the limbus of the cornea

Answer 3

Kayser-Fleischer rings

Question 4

Rare disease characterized by fatty change in the liver and encephalopathy

Answer 4

Reye syndrome

Question 5

Subclinical thrombosis of the portal vein (as from neonatal omphalitis or umbilical vein catheterization) produces a fibrotic, partially recanalized vascular channel presenting as splenomegaly or esophageal varices years after the occlusive event.

Answer 5

Banti syndrome

Question 6

Sharply demarcated area of red-blue discoloration due to acute intrahepatic thrombosis of a portal vein radicle

Answer 6

Infarct of Zahn

Question 7

Extrahepatic cholangiocarcinoma developing at the hilum

Answer 7

Klatskin tumor

Question 8

Structures resembling primitive glomeruli in a testicular mass. Mass reveals cuboidal to columnar epithelial cells forming microcysts, sheets, glands and papillae, often associated with eosinophilic hyaline globules

Answer 8

Schiller-Duvall bodies.

Mass described is an endodermal sinus tumor

Question 9

Caused by Chlamydial infection in patients who are HLA-B27 positive

Answer 9

Reiter syndrome

Urethritis
Conjunctivitis
Arthritis
Mucocutanous lesions

Question 10

Minute coccobacilli within vacuoles in macrophages

Answer 10

Donovan bodies
Seen in granuloma inguinale.
Causative agent is Calymmatobacterium donovani.

Question 11

Light purple, homogeneous intranuclear structure surrounded by a clear halo

Answer 11

Cowdry type A inclusion

seen in HSV

Question 12

Metastasis of mucinous tumor of the GI tract to the ovaries

Answer 12

Krukenberg tumor

Question 13

Solid unilateral ovarian tumor consisting of an abundant stroma containing nests of transitional-like epithelium

Answer 13

Brenner tumor

Question 14

Ovarian tumor producing ascites and hydrothorax

Answer 14

Meigs syndrome

Ovarian tumor is a fibroma

Question 15

RER of neurons

Answer 15

Nissl body

Question 16

Eosinophilic cytoplasmic inclusion consisting of a dense core surrounded by a halo; seen in neurons of patients with Parkinson disease

Answer 16

Lewy bodies

Question 17

Thick, elongated, brightly eosinophilic protein aggregates found in astrocytic processes in chronic gliosis and in some low-grade gliomas

Answer 17

Rosenthal fibers

Question 18

Progression of transtentorial herniation is often accompanied by linear or flame-shaped hemorrhagic lesions in the midbrain and pons.

Answer 18

Duret hemorrhages

Question 19

Chronic hypertension is associated with development of minute anuerysms in cerebral vessels, that are less than 300 um in diameter.

Answer 19

Charcot-Bouchard microaneurysm

Question 20

Small posterior fossa.
Misshapen midline cerebellum with downward extension of vermis through foramen magnum.
Association with HCP and lumbar myelomeningocele.

Answer 20

Arnold Chiari malformation

Chiari type II

Question 21

Low-lying cerebellar tonsils extend through the foramen magnum at the base of the skull leading to obstruction of CSF flow and compression of the medulla.
Results in headache and cranial nerve deficits

Answer 21

Chiari type I malformation

Question 22

Enlarged posterior fossa.

A large midline cerebellar cyst lined by ependyma replaces an absent cerebellar vermis.

Answer 22

Dandy-Walker malformation

Question 23

Progressive degenerative disease of the joints caused by nerve damage resulting in abnormal weight bearing

Answer 23

Charcot joints

Question 24

Leukodystrophy characterized by mutations in the glial fibrillary acidic protein.

Answer 24

Alexander Disease

Alexander likes to Go FAP

Question 25

Leukodystrophy characterized by galactocerebroside b-galatosidase deficiency.
Characterized by presence of multinucleated globoid cells

Answer 25

Krabbe Disease

Question 26

Confusion
Ophthalmoplegia
Ataxia

Triad of which syndrome; seen in which nutritional deficiency

Answer 26

Wernicke’s encephalopathy

Beriberi (Thiamine/B1 deficiency)

Question 27

Irreversible condition characterized with profound memory disturbances in patients with thiamine deficiency

Answer 27

Korsakoff Syndrome

Question 28

Patients who suffer multiple, bilateral, gray and white mater infarcts during the course of months develop dementia. When the pattern of injury preferentially involves large areas of subcortical white matter, the disorder is called.

Answer 28

Binswanger disease

Question 29

Autosomal recessive progressive illness, beginning in the first decade as gait ataxia, dysarhtria and then followed by loss of joint position and vibratory sense.

Answer 29

Friedreich ataxia

Question 30

X-linked adult onset disease affecting lower motor neurons characterized by distal limb amyotrophy and bulbar signs. Affected individuals also manifest androgen insensitivity.

Answer 30

Kennedy Disease aka
Bulbospinal atrophy

Like Huntington, characterized by triple repeats this time in the androgen receptor.

Question 31

Most common form of spinal muscle atrophy (loss of LMN and weakness associated with atrophy of muscle fibers often involving entire fascicles.) Onset at birth; death within 3 years. Where is the characteristic mutation?

Answer 31

Werdnig-Hoffman disease

SMN on chromosome 5

Question 32

Aggressive, clinically aggressive corticotroph adenoma developing after surgical removal of adrenal glands for the treatment of Cushing syndrome.

Answer 32

Nelson syndrome

Question 33

Most common form of clinically significant ischemic necrosis of the anterior pituitary?

Answer 33

Sheehan syndrome

Question 34

Hypothyroidism developing in older children and adults. Manifestations include generalized apathy, sluggishness, cold intolerance, constipation, mucopolysaccharide-rich edema, and broadening and coarsening of facial features.

Answer 34

Gull Disease

aka myxedema

Question 35

Eosinophilic, granular cytoplasm lining thyroid follicles. Represents metaplastic response of the normally low cuboidal follicular epithelium to ongoing injury. This is seen in what condition?

Answer 35

Hurthle (oxyphil) cells

seen in Hashimoto thyroiditis

Question 36

Hyperfunctioning thyroid nodule without accompanying infiltrative ophthalmopathy and dermopathy.

Answer 36

Plummer syndrome

Question 37

Ball-like deposits of a laminated matrix situated in the periphery of the glomerulus. These nodules are PAS(+) and contain trapped mesangial cells. These lesions are essentially pathognomonic of which condition

Answer 37

Kimmelstiel-WIlson lesion characteristic of DM nephropathy

aka nodular glomerulosclerosis (15-30%)

Question 38

Normal granular basophilic cytoplasm of the ACTH-producing cells in the anterior pituitary is replaced by homogenous, lightly basophilic material. This is seen in which conditions?

Answer 38

Crooke hyaline change.
In the pituitary of patients with Cushing Syndrome.

Due to accumulation of intermeiate keratin filaments.

Question 39

In psoriasis, neutrophils form small aggregates within both the spongiotic superficial epidermis - ______ - and the parakeratotic stratum corneum - _____.

Answer 39

Pustules of Kogoj

Munro microabscesses

Question 40

In lichen planus, anucleate necrotic basal cells are seen in the inflamed papillary dermis called ___.

Answer 40

Civatte bodies

Question 41

White dots or lines characteristic of pruritic, violaceous and flat topped plaques of lichen planus.

Answer 41

Wickham’s striae

Question 42

Explosive onset of seborrheic keratosis that occur as a paraneoplastic syndrome

Answer 42

Lesser-Trelat sign

Question 43

Sebaceous adenoma associated with an internal malignancy; this syndrome is characterized by microsatellite instability due to ioss of DNA mismatch repair.

Answer 43

Muir-Torre syndrome

Question 44

Familial basal cell Ca syndrome

Answer 44

Gorlin syndrome

Question 45

Multiple chondromas preferentially involving one side of the body

Answer 45

Ollier DIsease

Question 46

Multiple chondromas associated with benign soft tissue angiomas.

Increased risk of ovarian carcinomas and brain gliomas

Answer 46

Marfucci syndrome

Question 47

Characterized by polyostotic fibrous dysplasia, endocrine abnormalities (precocious puberty) and cafe au lait skin pigmentations. Associated with mutations in a G protein that constitutively activates adenyl cyclase

Answer 47

McCune Albright Syndrome

Question 48

Superficial fibromatoses in the:

1. Palmar fibromatosis
2. Penile fibromatosis

Answer 48

1. Dupuytren contracture

2. Peyronie disease