Info

Organs and Tissues

Procedures

Possible or Expected Findings

Abdominal cavity

Submit sample of subphrenic exudate for aerobic and anaerobic cultures (p. 102). Record location and volume of subphrenic exudate.

Possible causes of subphrenic empyema include appendicitis, cholecystitis,* diverticulitis, intrahepatic abscess, pancreatitis,* ruptured viscus; penetrating abdominal wound(s), perforated ulcer of stomach or duodenum,* and other conditions.

Organs and Tissues Procedures Possible or Expected Findings

Pleural cavities and lungs Record volume of effusion or exudate in Basal pleuritis and pneumonia, adjacent pleural space. to empyema.

Encephalitis, All Types or Type Unspecified

Synonyms and Related Terms: Acute disseminated encephalomyelitis;* acute hemorrhagic encephalitis; acute infective encephalitis or encephalomyelitis; acute poliovirus encephalitis or encephalomyelitis; amoebic encephalitis; Arbovirus encephalitis (Japanese encephalitis; eastern encephalitis, western encephalitis, Venezuelan equine encephalitis, St. Louis encephalitis); bulbar encephalitis;* brain stem encephalitis;* herpes encephalitis (cytomegalovirus encephalits, Epstein-Barr virus encephalitis, varicella-zoster encephalitis); herpes simplex encephalitis; HIV encephalitis; measles encephalitis; measles inclusion body encephalitis; postinfectious encephalitis; postvac-

cinal encephalitis; progressive multifocal leukoencephalitis or leukoencephalopathy; rabies* encephalitis; subacute encephalitis; subacute sclerosing panencephalitis; viral encephalitis, and many other terms (1), too numerous to mention. See also under "Note" and under "Possible or expected findings."

NOTE: If the condition that caused the encephalitis is known, see also under that heading. If the cause of the encephalitis is unknown, submit samples of tissue for microbiologic and toxicologic study, particularly if there is a suspicion of lead poisoning.* See also under "Encephalitis, brain stem," "Encephalomyelitis,...," "Encephalopathy" and "Myelopathy, Myelitis."

Organs and Tissues

Procedures

Possible or Expected Findings

Cerebrospinal fluid Blood

Brain and spinal cord; anterior and posterior spinal roots; sensory ganglia

Other organs

Submit sample for microbiologic study. Prepare cytospin.

Submit sample for microbiologic or toxicologic study, or both. Freeze serum sample for possible serologic study. For removal and specimen preparation, see pp. 65, 67, and 71, respectively. For microbiologic study, submit sample of fresh cerebral tissue. If infectious agent is known and need not be confirmed, fix intact brain in formalin. For toxicologic sampling, see Chapter 6.

Microbiologic, toxicologic, and histologic studies may be indicated, depending on the expected underlying disease.

Bacterial, fungal, rickettsial, viral, protozoal, or other infection, including amebiasis, cysticercosis, echinococcosis,* leptospirosis,* malaria* (falciparum), schistosomiasis,* syphilis,* toxoplasmosis,* trichinosis,* and trypanosomiasis.* Inclusion bodies may be present in various viral diseases or conditions.

References

1. Esiri MM, Kennedy PGE. Viral diseases. In: Greenfield's Neuropathology, vol. 2. Graham BI, Lantos PL, eds. Arnold, London, 1997, pp. 3-64.

2. Scaravilli F, Cook GC. Parasitic and fungal infections. In: Greenfield's Neuropathology, vol. 2. Graham BI, Lantos PL, eds. Arnold, London, 1997, pp. 65-112.

3. Gray F, Nordmann P. Bacterial infections. In: Greenfield's Neuropathology, vol. 2. Graham BI, Lantos PL, eds. Arnold, London, 1997, pp. 113152.

Encephalitis, Brain Stem

Synonyms and Related Terms: Brain stem abscess; infectious brain stem encephalitis; Listeria monocytogenes brain stem encephalitis; viral brain stem encephalitis.

NOTE: See also under "Encephalitis, limbic."

Organs and Tissues Procedures Possible or Expected Findings

Brain For removal and specimen preparation, Necrotizing encephalitis, with or without see p. 65. abscess formation (1).

Reference

1. Hall WA. Infectious lesions of the brain stem. Neurosurg Clin North Am 1993;4:543-551.

Encephalitis, Herpes Simplex (See "Infection, herpes simplex.") Encephalitis, Limbic

Synonyms and Related Terms: Brain stem encephalitis; limbic encephalopathy; paraneoplastic encephalomyelitis; paraneoplastic sensory neuropathy.

Organs and Tissues

Procedures

Possible or Expected Findings

Blood and cerebrospinal fluid

Brain, spinal cord, and dorsal root ganglia

Other organs

For removal and specimen preparation, see pp. 65, 67, and 69, respectively.

See also under "Tumor...," depending on expected primary site.

Commonly high titers of antibodies anti-Hu (anti neuronal nuclear antibodies, type 1 or ANNA 1) (1).

Neuronal degeneration; neuronophagia; microglial nodules; gliosis in hippocampus, brain stem, and dorsal root ganglia; perivascular lymphoid infiltrates, especially in nerve roots.

Carcinoma (bronchogenic small cell carcinoma in most instances; other primary tumors include non-small cell lung cancer or cancers of breast, ovary, uterus, and stomach).

Reference

1. Moll JWB, Vecht CH. Immune diagnosis of paraneoplastic neurological disease. Clin Neurol Neurosurg 1995;97:71-81.

Encephalomyelitis, Acute Disseminated

Synonyms and Related Terms: Acute hemorrhagic necrotizing encephalomyelitis; acute perivascular myelinoclasis; allergic encephalomyelitis; perivenous encephalomyelitis; postinfectious or parainfectious encephalomyelitis; postrabies vaccinal encephalomyelitis; postvaccinal encephalomyelitis.

Organs and Tissues

Procedures

Possible or Expected Findings

Brain and spinal cord; anterior and posterior spinal roots; sensory ganglia

For removal and specimen preparation, see pp. 65, 67, and 69, respectively. Microscopic findings vary and depend on the phase of the disease.

In acute phase, swelling and congestion of brain. Scattered perivenous demyelination with histiocytic and lymphocytic infiltrates, predominantly in white matter. Small perivascular hemorrhages may be present. In the hyperacute form of the condition (acute hemorrhagic necrotizing encephalopathy), swelling and congestion of the brain with signs of herniation. Petechial hemorrhages in the centrum semiovale white matter. Neutrophilic perivascular infiltrates with venule necrosis and fibrinous exudate.

Encephalomyelitis, All Types or Type Unspecified

Synonyms and Related Terms: Acute disseminated encephalomyelitis;* allergic encephalomyelitis; carcinomatous en-cephalomyelitis; hemorrhagic necrotizing encephalomyelitis; postinfectious or parainfectious encephalomyelitis; postrabies vaccinal encephalomyelitis; postvaccinal encephalomyelitis.

NOTE: For other related terms and for suggested procedures, see under "Encephalitis, ...," "Encephalomyelitis, acute disseminated," and "Myelopathy, Myelitis."

Encephalomyopathy (See "Myopathy.")

Encephalopathy, Hepatic

Synonyms and Related Terms: Acute hepatic encephalopathy; portal-systemic encephalopathy; Reye's syndrome.*

Organs and Tissues Procedures Possible or Expected Findings

Brain and spinal cord For removal and specimen preparation, In fulminant hepatic failure, cytotoxic see pp. 65 and 67, respectively. brain swelling with herniation and

Duret's hemorrhages. In portal systemic encephalopathy, brain may be grossly normal. Alzheimer type 2 astrocytes, with pale watery nuclei (common in globus pallidus, thalamus, and deep layers of cortex).

Liver Procedures depend on suspected underlying Alcoholic liver disease;* cirrhosis;* massive conditions as listed in right-hand column. or submassive hepatic necrosis;

microvesicular fatty changes in Reye's syndrome,* fatty liver of pregnancy, and other conditions; poisoning with hepatotoxic substances (e.g., mushroom poisoning with Amanita phalloides).

Encephalopathy, Hypertensive

Synonyms and Related Terms: Acute hypertensive encephalopathy; Binswangens disease; progressive subcortical encephalopathy; subcortical dementia.

NOTE: See also under "Hypertension (systemic arterial), all types or type unspecified."

Organs and Tissues

Procedures

Possible or Expected Findings

Brain

For removal and specimen preparation, see p. 65. Request Luxol fast blue-PAS stains (p. 172).

Heart, kidneys, vascular system, and other organs

Edema in sudden malignant hypertension. Focal ischemic changes; intracerebral hemorrhages. In Binswanger's disease, multiple small old infarctions or patchy or diffuse demyelination of the cerebral white matter is present, associated with sclerosis of small arteries. Demyelination and infarctions may occur together. Infarctions may be present in other portions of the brain. Causes (e.g., chronic renal disease) and manifestations of acute or chronic hypertension.

Encephalopathy, Type Unspecified Related Term: Toxic encephalopathy.

NOTE: If a specific toxic exposure is expected—for example, lead poisoning, see under that heading. Enchondromatosis, Multiple (See "Dyschondroplasia, Ollier's.") Endocarditis, Infective

Synonyms and Related Terms: Acute endocarditis; bacterial endocarditis; prosthetic valve endocarditis; subacute endocarditis. Possible Associated Conditions: See below under "Possible or Expected Findings."

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin; peripheral veins

If jaundice is present, search for evidence of gonococcal infection.

Record skin changes and prepare photographs.

Manifestations of malnutrition; jaundice; clubbing of fingers and toes; petechial hemorrhages of skin and mucous membranes; splinter hemorrhages of nail beds. Needle marks, furuncles, and other skin infections or scars may indicate dependence on intravenous drug(s).*

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin; peripheral veins (continued)

Eyes Blood

Heart

Arteries and veins Lungs

Intestinal tract and mesentery

Spleen

Liver

Kidneys

Internal genital organs

Bones

Brain

If intravenous catheter is present, leave in place, tie vessel proximally and distally from tip, and submit for microbiologic study. If this is not possible, prepare smears and sections of thrombus at tip of catheter. Request Gram and Grocott's methenamine silver stains (p. 172). Submit tip for culture, even if it is contaminated. Record appearance of oral cavity. Prepare chest roentgenogram. For removal and specimen preparation, see p. 85.

If cultures had not been prepared antemortem, submit samples for bacterial and fungal cultures (p. 102). Request aerobic and anaerobic bacterial cultures. Freeze serum sample for serologic study. For sterile removal of infectious material, see p. 103. Photograph valvular lesions; prepare sections of vegetations; request Gram and Grocott's methenamine silver stains (p. 172). For coronary arteriography, see p. 118. For collection of nonvalvular tissue for histo-logic study, see p. 30. For histologic sections, request Verhoeff-van Gieson stain (p. 173).

Dissect mesenteric arteries. Other procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column. Record size and weight.

For histologic sections, request 4-pm sections, stained with PAS and with methenamine silver for glomerular lesions (p. 173).

For removal; prosthetic repair, and specimen preparation, see p. 95.

For removal and specimen preparation, see p. 65. If cerebral involvement is suspected, submit sample for microbiologic study (p. 102).

Infected surgical arteriovenous shunts; infected intravenous catheters, including devices in surgically treated patients with hydrocephalus.*

Dental infection; petechial hemorrhages.

Petechial hemorrhages of conjunctivas;

Roth's spots.

Septicemia.

Rheumatic valvulitis; congenital cardiac malformations; prosthetic valve(s) with valvular ring abscesses; mycotic aneurysms of ascending aorta; valvular perforations. Coronary arterial emboli. Myocardial infarction; myocardial abscesses.

Mycotic aneurysms; septic thrombophlebitis.

Metastatic abscesses—for instance, after right-sided endocarditis in heroin addicts. Mesenteric emboli; intestinal infarction. Adenocarcinoma of colon may be associated with Strep. bovis endocarditis. Infarctions or abscesses, or both. Alcoholic liver disease. Glomerulitis. Macroscopically, minute hemorrhages, infarctions, and abscesses may be present.

Complications of abortion;* gonococcal infection.

Osteomyelitis.*

Infarctions, abscesses, or hemorrhages; mycotic aneurysms.

Endocarditis, Loffler's (See "Cardiomyopathy, restrictive [with eosinophilia].")

Endocarditis, Nonbacterial Thrombotic (NBTE)

Synonyms and Related Terms: Libman-Sacks verrucous nonbacterial endocarditis; marantic endocarditis; verrucous endocarditis.

NOTE: A history of multiple miscarriages may have been obtained.

Possible Associated Conditions: Disseminated intravascular coagulation;* antiphospholipid antibody syndrome; lupus anticoagulant.

Organs and Tissues Procedures Possible or Expected Findings

Heart If the diagnosis is suspected, photograph The mitral valve is usually affected, without and remove vegetations, as described for other valvular abnormalities.

infective endocarditis, and submit portions for microbiologic (p. 103) and histologic study.

Organs and Tissues

Procedures

Possible or Expected Findings

Heart

(continued)

Other organs

Prepare histologic sections of vegetations and of affected valve(s). If microorganisms appear to be present, request Gram stain (p. 172). Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Emboli and infarctions. Possible underlying conditions include carcinoma of the lung, pancreas, stomach, and other, primarily mucus-producing adenocarcinomas, systemic systemic lupus erythematosus,* antiphospho-lipid syndrome, and chronic debilitating diseases.

Enteritis, All Types or Type Unspecified (See "Enterocolitis,...," "Enteropathy,...," "Gastroenteritis, eosinophilic," and names of specific infectious diseases, such as "Fever, typhoid," or possible noninfectious underlying conditions, such as "Shock.")

Enteritis, Eosinophilic

(See "Gastroenteritis, eosinophilic.")

Enteritis, Granulomatous (See "Disease, Crohn's.")

Enteritis, Necrotizing

Synonyms and Related Terms: Clostridial gastroenteritis; Darmbrand; enteritis necroticans.

NOTE: Follow procedures described under "Enterocolitis, pseudomembranous." Clostridial enterotoxemia (C. perfrin-gens) seems to be the cause of necrotizing enteritis. Hemorrhagic necrosis of the small bowel mucosa with pseudomembranes, ulcers, and peritonitis is the main finding at autopsy.

Organs and Tissues Procedures

Enteritis, Other Types or Type Undetermined

(See "Enterocolitis, Other types or Type Undetermined.)

Enteritis, Regional (See "Disease, Crohn's.")

Enterocolitis, Ischemic

Synonyms and Related Terms: Hemorrhagic enteropathy; hemorrhagic necrosis (gangrene; infarction) of intestine; intestinal ischemia; ischemic enteritis; ischemic colitis; pseudo-membranous enterocolitis.*

NOTE: It is assumed here that the intestinal changes are clearly ischemic. If primary infection may be the cause of the condition, see below under "Enterocolitis, neutropenic," Enterocolitis, pseudomembranous," and "Enterocolitis, staphylococcal." In ischemic enterocolitis, superinfection should be ruled out and therefore, appropriate studies may be needed also: (1) Collect all tissues that appear to be infected. (2) Request aerobic and anaerobic bacterial cultures. (3) Request Gram stain (p. 172). (4) No special precautions are indicated. (5) Serologic studies are not available. (6) This is not a reportable disease.

Possible or Expected Findings

Intestinal tract and For mesenteric arteriography, see p. 55. Dissect Emboli, atherosclerosis, or other conditions mesentery mesenteric vessels. If infection is expected as a that may cause obstruction of mesenteric cause, submit portions of intestine for aerobic arteries. Primary or secondary thrombosis and anaerobic cultures (p. 102). For in situ of mesenteric veins. Fibrinous ischemic perfusion fixation of intestine, see p. 54. membranes or pseudomembranes and ulcers may be present in small and large intestine.

Other organs Manifestations of hypotension and shock.*

Enterocolitis, Neutropenic

Synonyms and Related Terms: C. septicum enterocolitis; necrotizing cecitis or typhlitis.

NOTE: (1) Collect all tissues that appear to be infected. (2) Request aerobic and anaerobic bacterial cultures. (3) Request Gram stain (p. 172). (4) No special precautions are indicated. (5) Serologic studies are not available. (6) This is not a reportable disease.

Organs and Tissues

Procedures

Possible or Expected Findings

Intestinal tract

Other organs and tissues

Collect material from lesions in cecum for aerobic and anaerobic culture. Sample for histologic study (p. 54).

C. septicum infection (or infection with other Clostridiae) with ulcers, hemorrhages, and pseudomembranes, primarily in cecum and ascending colon.

Malignancies that required chemotherapy or other conditions associated with neutropenia and treatment with antibiotics.

Enterocolitis, Other Types or Type Undetermined

NOTE: A multitude of infectious and noninfectious agents may cause inflammation of the small bowel, large bowel, or both. If the condition is not listed under "Colitis," "Enteritis," or "Enterocolitis" or under another specific heading such as "Dysentery, bacillary," obtain sufficient material for microbio-logic and histologic study to identify organisms such as Clos-tridium, Chlamydia, Shigella, Salmonella, Yersinia, Helicobacter, verotoxic E. coli, and others. If lymphogranuloma venereum,* or tuberculosis* are suspected, see also under these headings. See also under "Disease, inflammatory bowel" and "Disease, Crohn's."

Enterocolitis, Pseudomembranous

Synonyms and Related Terms: C. difficile colitis; Darmbrand; hemorrhagic necrosis (gangrene; infarction) of intes tine; ischemic enteritis or enterocolitis;* neutropenic enterocolitis;* pseudomembranous colitis.

NOTE: The name "Pseudomembranous enterocolitis" is descriptive; the condition may be infectious, ischemic, or both. If the intestinal changes are clearly ischemic, see above under "Enterocolitis, ischemic." If the cause is in doubt and if pseudomembranes can be identified, follow the procedures described here.

(1) Collect all tissues that appear to be infected. (2) Request aerobic and anaerobic bacterial cultures. (3) Request Gram stain (p. 172). (4) No special precautions are indicated. (5) Serologic studies are not available. (6) This is not a reportable disease.

For other infectious intestinal diseases, see under specific names, such as "Enterocolitis, neutropenic" or "Enterocolitis, staphylococcal."

Organs and Tissues

Procedures

Possible or Expected Findings

Intestinal tract and mesentery

Other organs

Collect material from pseudomembranes for aerobic and anaerobic culture (p. 102) and for C. difficile toxin assay.

Sample intestinal wall with pseudomembranes for histologic study.

If the condition is suspected to be caused by ischemia, follow procedures described above under "Enterocolitis, ischemic." Procedures depend on expected findings as listed in right-hand column.

Bacterial growth (C. difficile or verocyto-toxin producing E. coli or other organisms such as Shigella dysenteriae). Generally, the condition is confined to the colon. Lamellated pseudomembranes with much mucin and layers of neutrophils and necrotic epithelial cells. Mucous glands distended with mucin. Gram-positive bacilli in exudate. Occlusive vascular lesions or other conditions causing impaired intestinal perfusion.

Manifestations of hypotension and shock.* Conditions that were treated with antibiotics (which in turn allowed the selective proliferation of the intestinal pathogens).

Enterocolitis, Staphylococcal

Related Term: Staphylococcal diarrhea.

NOTE: (1) Collect all tissues that appear to be infected. (2) Request aerobic bacterial cultures. (3) Request Gram stain (p. 172). (4) No special precautions are indicated. (5) Usually, serologic studies are not helpful. (6) This is not a reportable disease.

Organs and Tissues

Procedures

Possible or Expected Findings

External examination Gastrointestinal tract

Other organs

Culture contents of stomach, small intestine, and large intestine (p. 102). Prepare sections and Gram-stained smears of mucus on intestinal wall. Procedures depend on expected findings as listed in right-hand column.

Dehydration.*

Staphylococcus aureus.

Conditions that may have required administration of antibiotics. Previous surgery.

Enteropathy, Gluten-Sensitive (See "Sprue, celiac.")

Enteropathy, Hemorrhagic (See "Enterocolitis, pseudomembranous.")

Enteropathy, Protein-Losing

NOTE: This a collective name for a diverse group of diseases and conditions that cause gastrointestinal protein loss. Carcinoma of the esophagus, heart diseases,* nephrosis, and primary immunodeficiency syndrome also may be causes of this condition.

Organs and Tissues

Procedures

Possible or Expected Findings

Heart

Esophagus Stomach

Small intestine

Colon

Other organs

Dissection procedures depend on the specific type of heart disease.

If a carcinoma is present, see also under "Tumor of the esophagus." For fixation and specimen preparation, see p. 53. If a carcinoma is present, see also under "Tumor of the stomach."

For postmortem lymphangiography, see p. 34. For in situ fixation and for preparation of intestinal mucosa for study under the dissecting microscope, see p. 54. For histologic sections, request PAS and azure-eosin stains (p. 172).

If infectious intestinal disease is suspected, submit portions of intestine for microbiologic study (p. 102).

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

Atrial septal defect;* primary cardiomyopathy;* constrictive pericarditis.* Other conditions associated with congestive heart failure.* Carcinoma.*

Allergic gastroenteropathy; carcinoma; giant hypertrophy of mucosa (Ménétrier's disease); atrophic gastritis. Status post gastrectomy.

Allergic gastroenteropathy; celiac* or tropical sprue;* Crohn's disease; intestinal lymphangiectasia; jejunal diverticulosis; lymphenteric fistula; lymphoma* and other malignancies; primary tuberculosis;* other infectious intestinal diseases (see also under "Enterocolitis,..."); Whipple's disease.*

Carcinoma and other malignancies; chronic ulcerative colitis or Crohn's disease;* megacolon.

Manifestations of malabsorption syndrome* with osteomalacia;* manifestations of congestive heart failure.* Conditions associated with nephrotic syndrome;* systemic sclerosis* (sclerodema) in cases with involvement of small intestine.

Eosinophilia, Tropical Pulmonary (See "Syndrome, eosinophilic pulmonary.")

Epiglottiditis (See "Laryngitis.")

Epilepsy, Idiopathic (Cryptogenic) Related Term: Status epilepticus.

Organs and Tissues Procedures Possible or Expected Findings

Brain For removal and specimen preparation, By definition, no gross changes or histologic see p. 65. Histologic sections should include lesions are demonstrable that could be

(as a minimum) both hippocampi, cerebellar responsible for seizures. In chronic epilepsy, cortex, cerebral cortex, and thalami. secondary tissue changes, attributable to repeated anoxic episodes, are found. These include hippocampal sclerosis and Purkinje cell loss in cerebellum and changes attributable to closed head injury,* such as superficial contusions in frontal or temporal lobes.

Other organs For possible side effects of therapy, see

"Epilepsy, symptomatic."

Epilepsy, Myoclonus

Synonyms and Related Terms: Baltic myoclonus; Lafora's disease; Lafora body disease; progressive myoclonus epilepsy with Lafora bodies; progressive myoclonus epilepsy without Lafora bodies; Unverricht-Lundborg disease.

NOTE: Myoclonic seizures also have been described in a number of progressive encephalopathies with complex neurological symptoms, such as GM1 and GM2 gangliosidosis,* and Niemann-Pick* and Krabbe's disease but also acquired disorders, including

Epilepsy, Myoclonus (continued)

Alzheimer's disease,* Creutzfeldt-Jakob disease,* posthypoxic encephalopathy, and subacute sclerosing panencephalitis. Mitochondrial encephalomyopathy also can present with myoclonus epilepsy and a mitochondrial myopathy with ragged red fibers (MERRF syndrome) in skeletal muscles.

Organs and Tissues

Procedures

Possible or Expected Findings

Brain

Other organs and tissues, including eyes and peripheral nerves

Skeletal muscles

For removal and specimen preparation, see p. 65. For histologic sections, request methyl violet or toluidine blue, Alcian blue, and PAS stains, with and without diastase digestion (p. 172).

For removal and specimen preparation of eyes, see p. 85. For sampling and specimen preparation of peripheral nerves, see p. 79. For histologic sections, request methyl violet or toluidine blue stain and PAS stain with and without diastase digestion (p. 173). For removal and specimen preparation, see p. 80. Request modified Gomori's trichrome stain.

Mild cortical atrophy. Diffuse neuronal loss with mild astrocytosis. In Lafora's disease, basophilic, metachromatic, PAS-positive, diastase-resistant, single or multiple (1-30 |jm diameter) intracytoplasmic neuronal inclusion bodies (Lafora bodies), primarily in cerebral cortex (central region and prefrontal motor cortex), thalamus, globus pallidus, substantia nigra, cerebellar cortex, and dentate nuclei. Cerebellar atrophy (Dilantin).

Lafora-body-type material in the heart, liver, retinas, peripheral nerves, skeletal muscles, and sweat gland ducts (especially axillary).

Ragged red fibers in mitochondrial myopathies.

Epilepsy, Symptomatic

NOTE: Possible causes or underlying conditions include cerebrovascular diseases, congenital malformations of the brain, degenerative and demyelinating diseases of the brain, head injury,* intracranial and cerebral infections, toxic or metabolic disorders (alcoholism,* barbiturate,* carbon monoxide,* and lead poisoning,* hemodilution, hypocalcemia, or hypoglycemia),* and tumors of the brain.*

Organs and Tissues

Procedures

Possible or Expected Findings

External examination

Brain

Other organs

If gum hypertrophy or hirsutism are present, record and prepare photographs. Record skin changes and presence or absence of lymph-adenopathy.

For removal and specimen preparation, see p. 65. For histologic sampling, see also under "Epilepsy, idiopathic (cryptogenic)." For cerebral arteriography, see p. 80. If intracranial infection is suspected, follow procedures described on p. 102. If a toxic or metabolic disorder is suspected, submit samples of body fluids and tissues for toxicologic study (p. 16).

Gum hypertrophy, hirsutism (in young women), and lymphadenopathy may be found in patients who received phenytoin (Dilantin); drug-related dermatitis may be found also.

See above under "Note."

Cerebrovascular abnormalities. Intracranial and cerebral infections.

Complications of anticonvulsive therapy: agranulocytosis (carbamazepine), megaloblastic anemia* (barbiturates) or liver damage (dilantin, valproic acid).

Erythema Multiforme

Synonyms and Related Terms: Erythema exudativum multiforme major; Stevens-Johnson syndrome; toxic epidermal necrolysis. NOTE: The histologic changes of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis may be quite similar (1).

Organs and Tissues

Procedures

Possible or Expected Findings

External examination and skin

Pleural cavities Lungs

Heart

Other organs

Eyes

Other organs and tissues

Record extent and character of skin lesions. Submit samples of affected and of unaffected skin for histologic study. Record extent and character of lesions in oral cavity.

Submit one lobe for microbiologic study (p. 103). Perfuse one lung with formalin (p. 47).

Record appearance of all mucosal surfaces. Submit samples for histologic study. Other procedures depend on expected findings or grossly identified abnormalities as listed in right-hand column.

For removal and specimen preparation, see p. 85.

Macules; papules; vesicles; bullae; hemorrhages. Vulvitis may be present. Ulcers, fissures, and hemorrhagic lesions of oral cavity. Effusion(s).*

Bronchitis;* bronchopneumonia. Pericarditis.*

Laryngitis;* pharyngitis; esophagitis; colitis; vaginitis; urethritis. Possible underlying diseases include nephritis, infectious disease, collagen disease, and malignant tumor. Radiation treatment may have been given also. Conjunctivitis; iritis, iridocyclitis; panophthalmitis.

Lymphoma* (with erythema multiforme as paraneoplastic syndrome) (2).

References

1. Rzany B, Hering O, Mockenhaupt M, Schroder W, Goerttler E, Ring J, Schopf E. Histopathological and epidemiological characteristics of patients with erythema exudativum multiforme major, Stevens Johnson syndrome and toxic epidermal necrolysis. Br J Dermatol 1996;135:6-11.

2. Kreutzer B, Stubiger N, Thiel HJ, Zierhut M. Oculomucocutaneous changes as paraneoplastic syndrome. Ger J Ophthalmol 1996;5:176-181.

Erythroblastosis Fetalis

Related Terms: Bilirubin encephalopathy; fetal hydrops; hemolytic anemia of the newborn; kernicterus. NOTE: Cytomegalovirus, Parvovirus, syphilis, and Toxoplasma infections can cause erythroblastosis fetalis. These may be sought with routine histological as well as immunohistochemical methods on tissue sections. Immune-mediated destruction of fetal red cells or platelets, causing fetal hemorrhages and erythroblastosis. Serologic tests are also available.

Organs and Tissues

Procedures

Possible or Expected Findings

Blood (maternal and fetal)

External examination and oral cavity

Thymus Heart and lungs

Liver

Spleen

Pancreas

Retroperitoneal tissues with adrenal glands and kidneys Lymph nodes Bone marrow

Perform a direct Coomb's test on fetal cells and antibody screen on fetal or maternal cells. Determine the hematocrit on the fetal blood. Record body weight and length.

Record weight.

Submit samples for histologic study.

Record weight. Submit samples for histologic study. Request Gomori's stain for iron (p. 172). Use immunohistochemical stains to confirm the presence of Parvovirus. Record weight. See also above under "Liver."

Submit sample for histologic study. Submit samples for histologic study.

For preparation of sections and smears, see p. 96.

Alloantibody-mediated hemolysis; anemia.

Generalized, severe edema (fetal hydrops); jaundice; purpuric rash. In long-term survivors, discolored deciduous teeth with hypoplastic enamel. Accelerated maturation. Erythroblasts in vessels of myocardium and of lungs. Look for intranuclear inclusions typical of Parvovirus.

Hepatomegaly with increased extramedullary hematopoiesis and hemosiderosis.

Splenomegaly with increased extramedullary hematopoiesis; hemosiderosis; small or absent Malpighian corpuscles. Increased extramedullary hematopoiesis. Extramedullary hematopoiesis in adrenal glands and in retroperitoneal (peripelvic and renal) soft tissues. Hypoplasia with hemosiderosis. Erythroblastic hyperplasia.

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