Organism
Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other | |
|Cushings Disease |Pituitary Hypersecretion of ACTH |↑ Cortisol |Moon Face |55-75% of endogenous cases, more common in women |
| |(usually a microadenoma) |Obesity, Moon Face, Buffalo Hump, Osteoporosis |Buffalo Hump |Diffuse Bilateral Adrenal Hyperplasia w/ thick yellow cortex |
| | |Hirsutism, ↑ Infections, HT, Thin Skin, | |Suppression by High Dose Dexamethasone |
| | |Easy Bruising, Red Stria & Muscle Weakness | |Inferior Petrosal Sinus gradient of 3:1 |
|Cushings Syndrome |Nonpituitary Neoplasm Secretion |↑ Cortisol |Small Cell Carcinoma |Most common in men |
|(Ectopic ACTH or CRH) |(Small Carcinoma, Bronchial & |Obesity, Moon Face, Buffalo Hump, Osteoporosis | |Diffuse Bilateral Adrenal Hyperplasia w/out nodularity |
| |Pancreatic Carcinoids, Thymoma, |Hirsutism, ↑ Infections, HT, Thin Skin, | |½ of Bronchial Carcinoids suppressed by ↑ dose dexamethasone |
| |Pheochromocytoma, Gastrinoma) |Easy Bruising, Red Stria & Muscle Weakness | | |
|Cushings Syndrome |Adrenal Adenoma |↑ Cortisol |↓ CRH & ACTH |↓ CRH & ACTH |
|(Adrenal Hypersecretion) |Adrenal Carcinoma |Obesity, Moon Face, Buffalo Hump, Osteoporosis | |Atrophy of adjacent cortex & contralateral adrenal cortex |
| |Adrenal Nodular Hyperplasia |Hirsutism, ↑ Infections, HT, Thin Skin, | | |
| | |Easy Bruising, Red Stria & Muscle Weakness | | |
|Cushings Syndrome |Exogenous Cortisol Excess |↑ Cortisol |Prednisone |Most common source is Prednisone |
|(Exogenous Cortisol Excess) | |Obesity, Moon Face, Buffalo Hump, Osteoporosis | |↓ Pituitary ACTH & Small Adrenal Glands with thin cortex |
| | |Hirsutism, ↑ Infections, HT, Thin Skin, | |Zona Glomerulosa not affected |
| | |Easy Bruising, Red Stria & Muscle Weakness | | |
|Pseudo-Cushings Syndrome |Depression, Alcoholism, Obesity, Eating|↑ Cortisol | | |
|(Temporary Cortisol Excess) |Disorder, Stress & Illness |Obesity, Moon Face, Buffalo Hump, Osteoporosis | | |
| | |Hirsutism, ↑ Infections, HT, Thin Skin, | | |
| | |Easy Bruising, Red Stria & Muscle Weakness | | |
|Primary Aldosteronism |Aldosterone secreting Adenoma |↑ Aldosterone |↓ Renin & ↑ Aldosterone |Small yellow encapsulated tumor found usually on left & in ♀ |
|(↓ Renin – Conn’s Syndrome) | |HT, ↓ Serum K+, ↑ Extracellular fluid, | |Low Plasma Renin Activity (PRA) |
| | |Alkalosis | |Aldosterone > 10 ng/dL after IV Saline (Saline Suppression Test) |
| | |Weakness, Paesthesias, Visual Disturbances | | |
| | |Headaches, Tetany, Cardiac decompensation | | |
|Primary Aldosteronism |Idiopathic |↑ Aldosterone |↓ Renin & ↑ Aldosterone |Bilateral idiopathic hyperplasia = less common than Conn’s |
|(↓ Renin – Adrenal Hyperplasia) | |HT, ↓ Serum K+, ↑ Extracellular fluid, | |Treat w/ spironolactone & something for HT |
| | |Alkalosis | |Low Plasma Renin Activity (PRA) |
| | |Weakness, Paesthesias, Visual Disturbances | |Aldosterone > 10 ng/dL after IV Saline (Saline Suppression Test) |
| | |Headaches, Tetany, Cardiac decompensation | | |
|Secondary Aldosteronism |Renal Artery Stenosis |↑ Aldosterone |↑ Renin & ↑ Aldosterone |High Plasma Renin Activity (PRA) |
|(↑ Renin) |Renin producing tunor |HT, ↓ Serum K+, ↑ Extracellular fluid, | | |
| |Chronic edema |Alkalosis | | |
| | |Weakness, Paesthesias, Visual Disturbances | | |
| | |Headaches, Tetany, Cardiac decompensation | | |
|Hyporenemic Hypoaldosteronism |Black Licorice |↓ Aldosterone | | |
| |Chewing Tobacco |Hypertension | | |
|Congenital Adrenal Hyperplasia |21-Hydroxylase Deficiency |↑ ACTH & DHEA → virilization | |Most common cause of CAH & of ambiguous genitalia |
|(21-Hydroxylase Deficiency) |(CYP21A2 Deficiency) |“Classic” = ↓ Aldosterone & Cortisol; ↑ Renin | |Hallmark is increased 17-Hydroxyprogesterone |
| | |“Classic w/out wasting” = nl Ald & ↓ Cortisol | |Treatment = replace aldosterone & cortisol |
| | |“Non-classical” = nl Aldosterone & Cortisol | | |
|Congenital Adrenal Hyperplasia |11β-Hydroxylase Deficiency |↑ ACTH & DHEA → virilization | | |
|(11β-Hydroxylase Deficiency) | |↑ Deoxycortisone | | |
| | |↓ Aldosterone, Renin & Cortisol | | |
|Congenital Adrenal Hyperplasia |17α-Hydroxylase Deficiency |↑ Aldosterone & ACTH | | |
|(17α-Hydroxylase Deficiency) | |↓ Cortisol & Sex Steroids | | |
| | |Hypertension & Hypokalemia | | |
Adrenal Pathology
Adrenal Pathology (contd.)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Cortical Hypofunction |Immediate steroid need | |↑ ACTH |1) Immediate need for steroids – glands unable to respond |
|(Primary Acute Insufficiency) |Rapid steroid withdrawal | | |2) Rapid Withdrawal of steroids |
| |Mass Destruction (DIC,Hemorrhage) | | |3) Massive destruction – hemorrhage, anticoagulants, DIC |
|Waterhouse-Friderichsen Syndrome |Bacteremic Infection |Adrenal Hemorrhage → Cortical Hypofunction |↑ ACTH |More common in children |
|Cortical Hypofunction |Autoimmune (majority) |Destruction of adrenal cortex only |↑ ACTH |Thin cortex w/ lymph infiltrates – unaffected medulla |
|(Primary Chronic Insufficiency) |TB, Histoplasmosis, Carcinoma |Fatigue, muscle weakness, weight loss, GI upset| | |
|(Addison’s Disease) |AIDS, Amyloidosis, Sarcoidosis |Hypoglycemia, Salt craving, Prerenal Azotemia | | |
| |Hemochromatosis |Acidosis, Hypotension & Hyperpigmentation | | |
|Cortical Hypofunction |Autoimmune (majority) |1) Adrenal insufficiency – same as above |↑ ACTH |Thin cortex w/ lymph infiltrates – unaffected medulla |
|(Primary Chronic Insufficiency) |TB, Histoplasmosis, Carcinoma |2) Hypoparathyroidism & Candidiasis | | |
|(Addison’s Disease) |AIDS, Amyloidosis, Sarcoidosis | | | |
|Subtype I |Hemochromatosis | | | |
|Cortical Hypofunction |Autoimmune (majority) |1) Adrenal insufficiency – same as above |↑ ACTH |Thin cortex w/ lymph infiltrates – unaffected medulla |
|(Primary Chronic Insufficiency) |TB, Histoplasmosis, Carcinoma |2) Autoimmune thyroid disease & Type I DM | | |
|(Addison’s Disease) |AIDS, Amyloidosis, Sarcoidosis | | | |
|Subtype II– Schmidt’s Syndrome |Hemochromatosis | | | |
|Cortical Hypofunction |Congenital Adrenal Hyperplasia | |↑ ACTH | |
|(Primary Chronic Insufficiency) |Enzyme Inhibitor (ex. = ketoconazole) | | | |
|(Failure of Cortisol Production) | | | | |
|Cortical Hypofunction |Carcinoma, Infection, Irradiation, |Fatigue, muscle weakness, weight loss, GI upset| |No aldosterone or ACTH deficiency |
|(Chronic 2º/3º Insufficiency) |Infarction |Hypoglycemia, Salt craving, | |Features of panhypopituitarism |
| |Prolonged use of steroids→↓ ACTH | | |Delayed response to prolonged ACTH stimulation test |
|Pheochromocytoma |MEN Type II or III |HT with or without paroxysmal attacks |Adrenomedullary Chromafin cells |Most are solitary & in medulla; pink colored; large = |
| | |Headaches, sweating, fever, GI upset, anxiety | |encapsulated |
| | |Palpitations orthostatic HT, numbness | |Diagnosis = Normetanephrine & metanephrine in plasma |
| | |Cardiac manifestations | |Clonidine useful in exclusion; Paroxysms can be provoked |
| | | | |Rule of 10’s = 10% extrarenal, bilateral, malignant, familial |
|MEN Syndrome Type I | |Parathyroid hyperplasia | | |
|(Wermer Syndrome) | |Pituitary adenomas | | |
| | |Pancreatic islet tumors | | |
|MEN Syndrome Type II | |Pheochromocytoma | |MEN IIA = Parathyroid Adenoma |
|(Sipple Syndrome) | |Medullary Thyroid carcinoma | |MEN IIB = Mucocutaneous ganglioneuromas & Marfanoid habitus |
| | |Parathyroid hyperplasia | | |
|MEN Syndrome Type III | |Pheochromocytoma | | |
| | |Medullary Thyroid carcinoma | | |
| | |Mucosal neuromas/Marfanoid features | | |
Endocrine Pancreas Pathology
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Primary Diabetes Mellitus |Genetic (HLA-DR3 &4) |Blindness (Lens, Retina, Optic Nerve) |HLA-DR3&4 |MI is #1 cause of death; Diabetic ketoacidosis |
|Type I |Autoimmune Antibodies |CV disease (MI, stroke, gangrene) |Diabetic ketoacidosis |Glycosylated collagens →Advanced Glycosylation End Products |
| |Environment (Viral Infection) |Nephropathy & Infections (Mucomycosis) | |Intracellular hyperglycemia→Sorbitol (lens & Schwann Cell) |
| | |Peripheral Neuropathy, Polydipsia & Polyuria | | |
|Primary Diabetes Mellitus |Genetic (not HLA related) |Blindness (Lens, Retina, Optic Nerve) |Hyperosmolar Coma |MI is #1 cause of death; Hyperosmolar Coma |
|Type II |Obesity |CV disease (MI, stroke, gangrene) | |Glycosylated collagens →Advanced Glycosylation End Products |
| |Insulin Deficiency |Nephropathy & Infections (Mucomycosis) | |Intracellular hyperglycemia→Sorbitol (lens & Schwann Cell) |
| |Insulin Resistance (receptor & GLUT) |Peripheral Neuropathy, Polydipsia & Polyuria | | |
|Secondary Diabetes Mellitus |Pancreatitis | | |Destruction of islets after disease |
| |Pancreatic Cancer | | | |
| |Drugs | | | |
Thyroid Pathology
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Diffuse Non-Toxic Goiter |Dietary |Hyperplastic enlargement of thyroid | |Involves whole gland |
|(Endemic) |↓ iodine |↓ T3/T4 | |More common |
| |goitrogens |↑ TSH | | |
|Diffuse Non-Toxic Goiter |Substance interfering with synthesis |Hyperplastic enlargement of thyroid |Young females |Typically young females |
|(Sporadic) |Hereditary enzyme defect |↓ T3/T4 | | |
| | |↑ TSH | | |
|Multinodal Goiter | |Hyperplastic enlargement of thyroid |Nodules/Cysts |Hemorrhage, scarring & calcification |
| | |↓ T3/T4 & ↑ TSH |Irregular | |
| | |Recurrent hyperplasia & involution | | |
| | |Irregular with Nodules & Cysts | | |
|Plummer’s Syndrome |Hyperfunctioning Goiter |↑ T3/T4 | |Toxic multinodal goiter |
| | | | |No skin/eye changes of Grave’s Disease |
|Graves Disease |Autoimmune (LATS/TSI & TGI) |Thyrotoxicosis (with diffuse hyperplasia) | |Typically females |
| |Genetic? (HLA-B8 & DR3) |Ophthalmopathy (Paralysis & exopthalmos) | |Treatment: PTU, methimazole, radioiodine & surgery |
| | |Dermopathy (Pretibial myxedema) | |Eye Muscle Paralysis & Exopthalmos NOT seen in Thyrotoxicosis |
|Thyrotoxicosis |Hyperfunctioning Thyroid |Cardiac: ↑HR,A-fib,Cardiomegaly, CHF |Heat Intolerance |Low TSH (suggestive) & High T4 (confirms) |
| |Leakage from Thyroid |Neuromuscular: Hyperreflexia,tremor,wasting |Lid Lag |Hyperpigmentation over extensor surfaces |
| |Ingestion of iodide or Synthroid |Skin: Heat Intolerance, ↑ Sweat & Pigmentation |↓ Cholesterol |Uncommon causes: Hydatiform mole, Struma ovarii, carcinomas |
| | |↑ Appetite, GI motility, Osteoporosis, Eyes | |Treatment: β Blockers, Propylthiuracil (PTU) , Iodide, Ablation |
|Primary Hypothyroidism |Insufficient Parenchyma |Anemia, ↑ Cholesterol, ↓ Na+, Myxedema |↑ Cholesterol |High TSH (suggestive) & Low Free T4 (confirms) |
| |Hashimoto Thyroiditis (Autoimmune) |Cold Intolerance, Fatigue, Depression, Lethargy|Myxedema |Therapy = T4 replacement (TSH takes 6-8 weeks to normalize) |
| |Developmental, Radiation |Weight ↑, Edema, Constipation, Cardiomyopathy | |Low TSH after Tx → osteoporosis & arrythmias |
| |Ablation |Delayed reflexes (achilles) & Coma (If severe) | | |
|Seondary Hypothyroidism |Pituitary Lesion | | |TSH is unreliable; Use T4 to make diagnosis |
| | | | |TRH stimulation Test = no response |
|Tertiary Hypothyroidism |Hypothalamic Lesion | | |TSH is unreliable; Use T4 to make diagnosis |
| | | | |TRH stimulation Test = delayed response (60 min) |
|Hypothyroidism - Other |↓ Synthesis | | |Use increased dose of T4 during pregnancy |
| |Idiopathic (block of TSH receptors | | | |
| |Heriditary, Hashimoto Thyroiditis | | | |
| |Lithium, Iodides, p-aminosalicylate | | | |
|Cretinism |Infant Hypothyroidism |Mental Retardation |Protruding Tongue | |
| |Early Childhood Hypothyroidism |Short Stature, Coarse Facial Features |Umbilical Hernia | |
| | |Umbilical Hernia | | |
| | |Protruding Tongue | | |
|Fetal Hypothyroidism |Thyroid Agenesis |Delayed Brain Development |Deaf & Mute | |
| |Iodine Deficiency |Deafness & Mutism | | |
| |Congenital Synthetic Defect |Spasticity | | |
| | |Severe Mental Retardation | | |
Thyroid Pathology (contd)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Chronic Autoimmune Thyroiditis |TPO Antibodies (99% sensitive) |Diffusely but painlessly enlarged Thyroid |Early transient Thyrotoxicosis |Female Predominance |
|(Hashimoto Thyroiditis) |Deficiency of Suppressor T Cells |Lymph infiltrates & follicles |Later Hypothyroidism |Early transient Thyrotoxicosis, Later Hypothyroidism |
| |Genetic? (HLA-DR3 &5) |Hurthle Cells (granular cytoplasm) | |Risk for B Cell Lymphomas |
| |TSH antibodies blocking receptor | | |Other antibodies: Thyroglobulin, Thyroid Peroxidase, I |
| | | | |transporter |
|Subacute Thyroiditis |Viral (Mumps, Influenza, Coxsackie) |Painful, diffusely enlarged Thyroid |Painful |Self Limited |
|(DeQuervain’s/Granulomatous) | |Giant Cells & Macrophages (like granuloma) | | |
|Palpation Thyroiditis |Vigorous Palpation |Giant Cells & Macrophages (like granuloma) | | |
| |Disuption of follicles | | | |
|Subacute Lymphocyte Thyroiditis |Idiopathic |Painless Goitrous Enlargement |Post-Partum |Self Limited |
| | | | |Often seen in post-partum women |
|Riedel’s Thyroiditis | |Fibrosing Thyroiditis - Painless | |Causes Laryngeal Nerve Paralysis (SOB & difficulty swallowing) |
| | |Hard gland | |Simulates malignancy |
| | | | |May be associated with fibrosis elsewhere |
|Follicular Adenoma | |Benign, solitary, encapsulated | |Majority of Thyroid neoplasms |
| | |Doesn’t take up iodine | |Not likely to cause thyrotoxicosis |
| | | | |May make more T3 than T4 |
|Papillary Carcinoma |RET gene mutation (MEN II) |Encapsulated or Infiltrating |Radiation |Most common thyroid cancer –↑ incidence in Gardner Syndrome |
| |RAS gene mutation |Complex branching papillae |Little Orphan Annie |Optically clear nuclei in “Little Orphan Annie Cells” |
| |Radiation exposure (most common) |Optically Clear nuclei & Psammoma bodies |Nodal Metastasis |Metastisize to regional nodes; Usually indolent growth |
| | |Intranuclear inclusions, grooves | |Treatment = Thyroidectomy & radioiodine therapy, Survival 98% |
|Follicular Carcinoma |RAS gene mutation |Vascular or Capsular Invasion |Invasion |2nd most common - Survival 92% |
| | | |Blood Metastasis |Hematogenously spread to bone, lungs & brain |
| | | | |Treatment lobectomy or thyroidectomy, radioiodine (if invasive) |
|Hurthle Cell Neoplasm |RAS gene mutation |Abundent granular cytoplasm | |Behave the same as follicular cell carcinoma/adenoma |
|(Adenoma or Carcinoma) | | | | |
|Medullary Carcinoma |RET gene mutation (MEN II) |Parafollicular C Cells (neuroendocrine) |Amyloid Stroma |Rare – most sporadic, 20% as MEN II or familial |
| |RAS gene mutation |Nodule or multifocal |Calcitonin |May be paraneoplastic (CEA, somatostatin, serotonin & VIP) |
| | |Not encapsulated, round or spindle cells |Not encapsulated |Metastasize in blood to lung, liver, bone & nodes |
| | |Amyloid( Calcitonin) tumor stroma | |Treatment = Thyroidectomy |
|Anaplastic Carcinomas |RAS gene mutation |Undifferentiated neoplasms |Elderly |Poor prognosis – uniformly fatal |
| |P53 gene mutation |Large, locally invasive, rapidly growing |Fatal |Loss of I uptake & ↓ Thyroglobulin |
| | | | |Survival less than 1 year from diagnosis |
| | | | |Possibly derived from Papillary Carcinoma |
Leukemia & Lymphoma
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Acute Lymphoblastic Leukemia |Trisomy 21, Bloom’s, Fanconi’s |Rapid Onset, > 20% Blasts |Children |Cells similar to lymphoblastic lymphoma |
|(ALL) |Radiation |Bone Pain, CNS & Testes Involvement |TdT+, |Generally good prognosis (70%) = ↓ WBC, t12:21, < 9yo, diploidy |
| |Alkylating Agents, Benzene |HSmegaly, Anemia & Thrombocytopenia |Scant agranular cytoplasm |Bad = WBC (>10K), T-Type, t9:22, 11q23, organomegaly |
| |Viral |T Type = lymphadenopathy & mediastinal mass | |3 types: Pre B (85%), Pre T (15%), B “like Burkitts” (2%) |
|Acute Myeloid Leukemia |Trisomy 21, Bloom’s, Fanconi’s |Rapid Onset, > 20% Blasts |Auer rods |Fair prognosis (40%), Usually adults |
|(AML) |Radiation |Anemia & Thrombocytopenia |CD13, CD15, CD33 |Frequent infections & mucosal bleeding |
| |Alkylating Agents, Benzene |Monocytic = gum hyperplasia |Granular Cytoplasm |Good = t8:21, t15:17 (treat w/ RA) & Chrom 16 abnormalities |
| |Viral |Promyelocytic (M3) = DIC, ↓WBC | |Bad = t9:22, 11q23, origin from myelodysplasia or treatment |
|Acute Leukemia |Trisomy 21, Bloom’s, Fanconi’s |Myeloid & Lymphoid features | |Poor prognosis |
|of Ambiguous Lineage |Radiation | | | |
| |Alkylating Agents, Benzene | | | |
| |Viral | | | |
|Chronic Myeloid Leukemia |Trisomy 21, Bloom’s, Fanconi’s |Gradual onset, Mature Cells |↓ LAP |Usually adults, Philadelphia Chromosome (t 9:22) |
|(CML) |Radiation |100% cellular Bone Marrow (no fat) |t9:22 or bcr-abl |bcr-abl ↑ Tyrosine Kinase activity ( Treat with Gleevec) |
| |Alkylating Agents, Benzene |Pluripotent Stem Cells→Granulocytes |Pluripotent Stem Cells |↓ Leukocyte Alkaline Phosphatase (LAP) |
| |Viral |Splenomegaly = Draggin Sensation | |Poor Prognosis - Always goes to blast crisis (AML or ALL) |
|Chronic Lymphocytic Leukemia |Trisomy 21, Bloom’s, Fanconi’s |Gradual onset, Mature Cells, Hepatosplenomegaly|Not radiation |Usually adults (most common adult leukemia in west) |
|(CLL) |Alkylating Agents, Benzene |Clonal B Cell Disorder (small lymphocytes) |CD5, CD20, CD23 |Same as CLL; indolent, not affected by therapy |
| |Viral |↑ Infections |Del 13q |Some have large cell transformation (Richter Syndrome) |
| | |Hypogammoglobulinemia |RichterSyndrome |Bad = trisomy 12, CD38 & de1 11q |
|Hairy Cell Leukemia |Trisomy 21, Bloom’s, Fanconi’s |Gradual onset |Adult Men |Usually adult men (2% of leukemias) |
|(HCL) |Alkylating Agents, Benzene |Bacterial & Mycobacterial Infections |Not radiation |Fair prognosis (40%) |
| |Viral |Beefy Red Splenomegaly, ↑ Reticulin in BM |“Fried Egg appearance” |Pancytopenia (70%), Neutropenia & Monocytopenia (90-100%) |
| | |TRAP+ B Cells |TRAP+ |Good Treatment - 2-CDA, pentostatin & interferon |
|Myelodysplastic Syndrome |Idiopathic |BM = Hypercellular, Periphery = Pancytopenia |Ringed Sideroblasts |Many subtypes based on ringed sideroblasts & blast counts |
| |Therapy related (ex. Radiation) |600,000) |Large Platelets |Least common of myeloproliferative disorders |
| | |Large Platelets |No myelofibrosis |Diagnosis by exclusion |
| | |Thrombosis & Hemorrhage | |Good prognosis (>10 year survival) |
|Hodgkin’s Disease |EBV |Predominance of lymphocytes/hystiocytes |No Typical Reed Sternberg Cells |Men more common |
|(Lymphocyte Predominance) | |Typically a cervical node |Lymphohystocytic Cells |Best Prognosis |
| | |Cd20 & CD45 |B Cell Lymphoma |Can turn into Diffuse Large B Cell Lymphoma |
| | | |CD20 & CD45 | |
|Hodgkin’s Disease |EBV |Predominance of lymphocytes/hystiocytes |No Typical Reed Sternberg Cells |Men more common |
|(Lymphocyte Rich) | |CD15 & CD30 |Lymphohystocytic Cells |Can turn into Diffuse Large B Cell Lymphoma |
Leukemia & Lymphoma (Contd.)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Hodgkin’s Disease |EBV |Cervical, Supraclavicular or mediastinal nodes |Lacunar Reed Sternberg Cells |Most common type; affects adolescent or young adult women |
|(Nodular Sclerosis) | |Variable cell composition & necrosis |Collagen Bands |Staging (usually I or II) determines treatment; Good prognosis |
| | |CD15 & CD30 |Women | |
|Hodgkin’s Disease |EBV |Variable cell composition & focal necrosis |Classic Reed Sternberg Cells |2nd most common type; men more common |
|(Mixed Cellularity) | |Disordered fibrosis | |Staging determines treatment; Guarded prognosis |
| | |Eosinophils, Plasma & T Cells | | |
| | |CD15 & CD30 | | |
|Hodgkin’s Disease |EBV |1) Diffuse Fibrosis – RS Cells embedded in |Poor Prognosis |Fairly common – usually in older patients |
|(Lymphocyte Depletion) | |fibrous stroma |Many Reed Sternberg Cells |Often Bone Marrow Involvement (Stage III or IV) |
| | |2) Reticular – RS Cells in sheets |Bone Marrow |Poor prognosis |
| | |↑ #s of Reed Sternberg Cells | |Usually not a true Hodgkin’s Lymphoma? |
|Small Lymphocytic Lymphoma |Trisomy 21, Bloom’s, Fanconi’s |Gradual onset, Mature Cells, Hepatosplenomegaly|Not radiation |Usually adults (most common adult leukemia in west) |
| |Alkylating Agents, Benzene |Clonal B Cell Disorder (small lymphocytes) |CD5, CD20, CD23 |Same as CLL; indolent, not affected by therapy |
| |Viral |↑ Infections |Del 13q |Some have large cell transformation (Richter Syndrome) |
| | |Hypogammoglobulinemia |RichterSyndrome |Bad = trisomy 12, CD38 & de1 11q |
|Follicular Lymphoma | |Nodular Growth Pattern with 2 cell types |CD10,CD20,CD23 |Fairly Common; indolent, not affected by therapy |
| | |1) Centrocytes = cleaved nuclear contours |BCL-2, t14:18 |May transform to diffuse large B cell lymphoma |
| | |2) Centroblasts = clear chromatin & | | |
| | |multinucleate | | |
| | |Bone Marrow frequently involved | | |
|Mantle Cell Lymphoma | |Atrophied germinal center |CD5 & CD20 |Not indolent or treatable |
| | |Small cells with cleaved nuclear contour |BCL-1, t11:14 | |
|Marginal Zone B-Cell Lymphoma |H. Pylori |Large B Cells, some plasma cells |CD20 |Common in GI Tract, Indolent, not dependent on treatment |
|(MALT Type) |Autoimmune (Sjogren, Hashimoto) | | | |
|Diffuse Large B-Cell Lymphoma |EBV |Large cell size, diffuse growth pattern |T14:18, t8:14, t13:14 |Very Common, Very Aggressive |
| |HSV8 | |BCL2,C-Myc,BCL6 |Prognosis depends on treatment, BCL-6 = good prognosis |
| | | | | |
|Burkitt’s Lymphoma |EBV |“Starry Sky picture” |“Starry Sky picture” |One of three bad types in kids |
| | | |CD10,CD19,CD20 |Highest growth fraction of all tumors |
| | | |C-Myc |Most manifest as extra-nodal sites |
| | | | |Responsive to treatment, but poor prognosis |
|Lymphoblastic Lymphoma | |Mediastinal Mass like Hodgkins |CD3, TdT+, TCL-1 (T Type) |One of three bad types in kids |
|(T/B) | | | |Responsive to treatment, but poor prognosis |
|Anaplastic Large T/Null Cell | | |CD3, CD30 |One of three bad types in kids |
|Lymphoma | | |ALK, EMA t2:5 |Uncommon in adults, Common in children |
| | | | |Presents in extra-nodal tissue |
| | | | |Usually T, not B cell |
|Peripheral T-Cell Lymphoma |EBV |Extranodal NK/T Cell Lymphoma |CD3 |Poor Prognosis; Common in Asia, uncommon in US |
| | |Nasal Type – eats face off | | |
Leukemia & Lymphoma (Contd. 2)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Mycosis fungoides | |Cerebriform nuclei |CD4 |Indolent Cutaneous disorder |
| | | |Polyclonal Ig |Extracutaneous spread |
| | | | |(Leukemia = Sezary Syndrome) |
|Multiple Myeloma |Radiation, Chemicals, Asbestos |Painful Bone Destruction, ↓ Humoral Immunity |M Protein |Most common 1º malignant tumor of bone |
| |Black Race |Plasmacytosis with IL-6, Vertebral Fractures |Bence Jones Protein |Complications: Renal failure, Amyloidosis, AML (rarely) |
| |HSV8? |M Protein, Bence Jones proteins, Hypercalcemia |Hypercalcemia |Fair prognosis (3 yr) |
| | |Can spread to nodes, skin, etc |IL-6 | |
|1º Amyloidosis |Radiation, Chemicals, Asbestos |Plasmocytosis with ↑ Light Chain Production |Large Tongue |Usually immunoglobulin light chain |
| |Black Race |Fibrils of β-pleated sheets that stain Congo | | |
| |Multiple Myeloma |Red | | |
| | |Large tongue, Neuropathy, GI | | |
| | |Arrythmias, CHF | | |
|2º Amyloidosis |Chronic Infection or Inflammation |Fibrils of β-pleated sheets that stain Congo |HSmegaly, | |
| | |Red |Proteinuria | |
| | |HSmegaly, | | |
| | |Proteinuria | | |
|Monoclonal Gammopathy of |Aging |M Proteins without other symptoms | |Most common monoclonal gammopathy |
|Undetermined Significance | | | |Diagnosis of exclusion, IgG < 3.5 |
| | | | |May develop into multiple Myeloma |
| | | | |Requires no treatment |
Pituitary Pathology
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Prolactinoma | |↑ Prolactin | |Most common secretory adenoma of anterior lobe |
| | |Amenorrhea, Galactorrhea, Infertility, | |Effects less obvious in postmenopausal women |
| | |Loss of Libido | |Treated with bromocriptine |
|Hyperprolactinemia |Injury to pituitary stalk |↑ Prolactin |Prolactin |Due to decreased dopamine to the anterior lobe |
| |Compression | | |Non-prolactin secreting adenomas may compress stalk |
| |Trauma to Hypothalamus, Infection | | | |
| |Drugs (Phenylthiazines) | | | |
|Somatotroph Adenoma | |↑ Growth Hormone → ↑ Hepatic IGF-1 |IGF-1 |Second most common secretory adenoma of anterior lobe |
| | |Some produce prolactin as well | |Treatment = transsphenoidal resection, bromocriptine & radiation |
| | |Acromegaly (adults) & Gigantism (children) | |Acromegaly = hands, feet, face skin & viscera |
| | |DM, HT, CHF, arthritis, weakness, GI carcinoma | | |
|Corticotroph Adenoma | |↑ ACTH→ ↑ Cortisol (Cushing’s Disease) |ACTH |90% are microadenomas |
| | |Moon facies, central obesity, striae, bruising |Microadenoma |Treatment = transsphenoidal resection |
| | |Osteoporosis, DM & HT | | |
|Gonadotroph Adenoma | |No clinical syndrome | |Secrete hormones inefficiently and variably |
|Thyrotroph Adenoma | | | |Rarest |
|Sheehan’s Syndrome |Pregnancy & Lactotroph Hypertrophy |Hypopituitarism | |Most common cause of anterior lobe ischemic necrosis |
| |Hypotension, DIC | | | |
| |Sickle Cell Anemia, ↑ ICP | | | |
|Empty Sella Syndrome |Maldevelopment of diaphragma sella |Depends on number of residual cells | |Maldevelopment = Arachnoid Herniation via enlarged opening |
| |Pituitary Apoplexy |Hypopituitarism | | |
| |Sheehan’s Syndrome |No syndrome | | |
| |Ablation | | | |
|Diabetes Insipidus |Interruption of DA to Posterior Lobe |Large volume of dilute urine |↓ ADH |Treatment = Administer ADH |
| |Trauma | | | |
| |Tumor | | | |
| |Inflammation | | | |
|Syndrome of Inappropriate ADH |Ectopic ADH (Small Cell Carcinoma) |Water Retention, Hyponatremia |↑ ADH |Treatment = Water Restriction, Slow normalization of Na+ |
|(SIADH) |Lung Disease (TB) |GI Upset, Cerebral Edema | |(Rapid Normalization can cause Central Pontine Myelinolysis) |
| |Intracranial Lesions (↓ Inhibition) | | | |
| |Drugs | | | |
|Hypothalamic Tumors | |Hyperpituitarism, Hypopituitarism | |Glioma & Craniopharyngioma are most common |
| | |Diabetes Insipidus | | |
CNS Pathology
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Concussion |Blunt non-penetrating injury | |Reversible |Reversible Traumatic Paralysis Ocurring Immediately After Injury |
| | | |Paralysis | |
| | | |Immediate | |
|Contusion |Stress on parenchymal vessels |Seizures |Intact pia-glial membrane |Most common sites: Frontal Pole, Temporal Pole, Medial orbital |
| |Coup |Cognitive & Personality Changes | |surface of the temporal lobe |
| |Countercoup |Headaches | |Pia-arachnoid not penetrated |
| | |Dizziness | |Occur at crests of gyri |
|Lacerations |Penetrating Wounds |Subarachnoid bleeding |Meningocerebral cicatrix |Traumatic disruption of pia-arachnoid and brain surface |
| | |Satellite petichial hemorrhages | |Meningocerebral cicatrix (glial scar) = epileptogenic focus |
| | |Edema in tissue | | |
|Epidural Hemorrhage |Fracture |Immediate Loss of Consciousness | |Collection of blood between dura and skull |
| |Most = Arterial (Middle Meningeal) |Lucid Interval | |Lenticular shaped hematoma |
| |Rarely = Dural Sinus, Bridging Veins |Later Loss of Consciousness | | |
|Acute Subdural Hemorrhage |Superficial Cortical Bridging Veins |48 hours-days; appears as clotted blood |Clotted Blood |More Common than epidural hemorrhage |
| |Dural Sinus Laceration |Gradual Loss of Consiousness | |Typically seen in infants, elderly & alcoholics |
| |Depressed Fractures |Hemiparesis → Hemiplegia | |Sheet of blood between dura & arachnoid; forms hyaline sac |
| |Bullet Wounds |Evidence of Herniation (if > 60 cc) | | |
|Subacute Subdural Hemorrhage |Superficial Cortical Bridging Veins |Clotted & Fluid Blood |Clotted & Fluid Blood |More Common than epidural hemorrhage |
| |Dural Sinus Laceration |Gradual Loss of Consiousness | |Typically seen in infants, elderly & alcoholics |
| |Depressed Fractures |Hemiparesis → Hemiplegia | |Sheet of blood between dura & arachnoid; forms hyaline sac |
| |Bullet Wounds |Evidence of Herniation (if > 60 cc) | | |
|Chronic Subdural Hemorrhage |Superficial Cortical Bridging Veins |> 3 weeks & Liquefied Hematoma |Liquefied Hematoma |More Common than epidural hemorrhage |
| |Dural Sinus Laceration |Gradual Loss of Consiousness |Minor Precipitating Event |Typically seen in infants, elderly & alcoholics |
| |Depressed Fractures |Hemiparesis → Hemiplegia | |Sheet of blood between dura & arachnoid; forms hyaline sac |
| |Bullet Wounds |Evidence of Herniation (if > 60 cc) | |Slow Bleeding, No immediate symptoms, Minor precipitating event |
|Subarachnoid Hemorrhage |Trauma |Stiff Neck, Alterations in Consciousness |Stiff Neck |Usually multiple and small |
| |Superficial Cortical Veins |Vasospasm & Hydrocephalus | | |
| |Surface cortical lacerations | | | |
| |Surface cortical contusions | | | |
|Intracerebral Hematoma |Rupture of Intrinsic Cerebral Vessels | | |Not in contact with the cortical surface, usually solitary |
| | | | |Frontal & Temporal Lobes are most common sites |
|Cranial Nerve Damage |Cranial Fractures | | |Damage usually occurs where nerves exit through foramina |
| | | | |Most Common = CNI, CNII, CN V & CN VI |
|Pontomedullary Avulsion |Marked Hyperextension of neck | | |Poor prognosis, Immediately fatal if complete, die later if |
| | | | |incomplete |
|Diffuse Axonal Injury |Diffuse Shearing of axons |Unconscious from moment of impact |Calpain |Results in disconnection of distal axonal segment |
|(1º Axotomy) | |No lucid interval | |Most common sites = Corpus Callosum & S. Cerebellar Peduncles |
| | |Ca2+ influx & swelling | | |
| | |Microtubule depolymerization, Calpain | | |
| | |activation | | |
CNS Pathology (Contd.)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Diffuse Axonal Injury |Small axonal membrane tears that reseal|Unconscious from moment of impact |Axoplasmic Transport |Results in disconnection of distal axonal segment |
|(2º Axotomy) | |No lucid interval | |Most common sites = Corpus Callosum & S. Cerebellar Peduncles |
| | |Ca2+ activated proteases, repair fails | | |
| | |Axoplasmic transport causes swelling | | |
|Hypoxic Brain Injury | | | |Greatest Damage = Hippocampus, Caudate, Putamen, |
| | | | |Cortical Layers 3 & 5, Purkinje Cells of Cerebellum |
| | | | |Diffuse or localized to watershed zone |
|Brain Swelling |Mechanical Damage |White Matter Swelling | |White matter swelling adjacent to contusion |
| |Blood Vessel Impairment |Diffuse Swelling | |Diffuse Swelling of One or Both Hemispheres |
| |Vasodilation | | | |
|Multiple Petechial Hemorrhages |Acceleration/Deceleration? |Frontal & Temporal Lobes (White Matter) | |Usually seen in patients who die soon after head injury |
| | |Thalamus | | |
| | |Brainstem | | |
|Fat Embolism |Fractures of Limbs or Limb Giurdles |3-4 d = white matter petichiae, capillary | |Usually only seen in adults (children have less fat) |
| |(Fat enters veins) |necrosis | | |
| | |4-7d = grey matter alterations, fat in | | |
| | |capillaries | | |
| | |12d-3m = many infarcts in cortex & pons | | |
| | |>3 m = atrophy of white matter | | |
|Early Post Traumatic Epilepsy | |Within first week | |Occurs during first week |
| | | | |More common in children than adults |
| | | | |Associated with: Hematoma, Depressed Fracture, Amnesia |
|Late Post Traumatic Epilepsy |Blunt Head Injury |After first week | |Most common complication of blunt head injury |
| | | | |Occurs later than one week |
| | | | |↑ Risk = Hematoma, Depressed Fracture, Early Epilepsy |
|Multiple Sclerosis |T-Cell Autoimmune against Myelin |Diagnosis = 2 lesions & 2 symptoms |Young Women, CNS |Commonest in young women; Affects CNS myelin, Variable course |
| |Unknown |Visual Impairment, Weakness, Dysarthria |Plaques |Acute Plaque: Sharp Border, Inflammation & Edema, Demyelination |
| |Distance from equator, Environment |Ataxia, Vertigo, Urinary Symptoms |Variability |Inactive Plaque: Sharp Border, No Inflammation Edema or Myelin |
| |Genetic |CSF = ↑ Mono’s & IgG (OCB-not in serum) |Oligoclonal Bands (OCB) |Shadow Plaque: Poor Border, Thin Myelin at Periphery |
|Devic Disease | | |Spinal Cord & CN II Only |Severe necrotic lesions in spinal cord and optic nerve only |
|(Neuromyelitis Optica) | | | |Rapidly progressive |
|Acute Multiple Sclerosis | | | |Fatal in 1-6 months |
|(Marburg Type) | | | |Severe & Rapid with extensive involvement of brain & spinal cord |
|Central Pontine Myelinolysis |Rapid Correction of Hyponatremia |Rapidly evolving quadriplegia |CNS |Symmetric demyelination in the center of the base of the pons |
| |Sever Electrolyte Imbalance Alcoholism,| | |Can affect tegmentum occasionally |
| |Liver Transplant | | |Outcome is variable: Complete Recovery→Fatal |
| |Burns, Malnutrition | | | |
|Guillian-Barre Syndrome |Acute Influenza-like Illness |Inflammation/Demyelination of Nerves & roots |PNS |Treatment: Supportive, Plasmapheresis, IV Immunoglobulin |
| |CMV & Campylobacter jejuni |Cranial & Spinal Motor Roots = Severe |Ascending Paralysis |Outcome: Complete recovery is most common, rarely fatal |
| |Immunization |Ascending Paralysis | |Death due to respiratory paralysis |
| |Autoimmune? | | | |
CNS Pathology (Contd. 2)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Spina Bifida Occulta |Folate Deficiency |Usually assymptomatic |Stigmata (Hypertrichosis) |Variable cord anomaly |
| | |Non-closed vertebral arches without cyst | | |
| | |Stigmata: Hypertrichosis,Dimple,Lipoma,Nevus | | |
| | |Sacral, Anorectal or UG Defect | | |
|Spina Bifida Cystica |Folate Deficiency |Non-closed vertebral arches with cyst |Cyst |Meningocele: Meninges protrude through vertebral defect |
| | |Meningocele or Myelomeningocele | |Myelomeningocele: Meninges & Spinal cord protrude through defect |
| | | | |Most survive > 1 year, but frequently have progressive |
| | | | |deterioration |
| | | | |Disabilities are usual: paraplegia, incontinence, infection, |
| | | | |learning |
|Arnold-Chiari Type II | |Mental Retardation | |1) Myelomeningocele |
| | | | |2) Elongation of Inferior Vermis & Brain Stem with displacement i|
| | | | |into spinal canal |
| | | | |3) Hydrocephalus |
|Annencephaly |Folate Deficiency |Absence of cerebrum & calvarium | |Most common congenital malformation of the brain |
| | | | |Stillborn or die shortly after birth |
|Intraventricular Hemorrhage |Preterm Birth | |Preterm |Most common neonatal intracranial hemorrhage |
|(IVH) |Periventricular Germinal Matrix | |Periventricular Germinal Matrix |PV Germinal Matrix is fragile, fibrinolytic & persists until 34 |
| | | | |weeks |
| | | | |Variable: May be focal & asymptomatic, may spread into ventricles|
|Periventricular Leukomalacia |Infarction of Periventricular White |Initially non-specific |Preterm |Common ischemic lesion of preterm infant |
|(PVL) |Matter |Later: Spastic Motor Dysfunction |Centrum Semiovale |Centrum Ovale = vulnerable boundry zone |
| |(Centrum Semiovale) |Paraplegia/Quadraplegia develops in surviving | |(Ventriculopetal & Ventriculofugal Arteries) |
| | | | |Usually not without permanent sequelae |
|Diffuse Astrocytoma | |Diffusely infiltrate w/o clear margins |Diffusely Infiltrative |20% of gliomas, Rarely Resectable, Progress towards anaplastic |
| | |Found in white matter of cerebrum |No Clear Margins |Graded on: Hypercellularity, endothelial changes & necrosis |
| | | |Cerebrum |Poor Prognosis |
|Brainstem Glioma | |Occur in Pons, infiltrate widely |Children (2nd decade) |Range of grades includes glioblastoma |
|(Atrocytoma Subgroup) | |CN Palsies, Long Tract signs, Gait |Pons |Surgical removal not possible |
| | |abnormalities | | |
| | |Emesis & Cerebellar Signs | | |
|Pilocytic Astrocytom | |Circumscribed, low grade, histology = biphasic |Circumscribed |Young>Old |
| | |Cerebellum, Hypothalamus, Optic Chiasm/Nerve |Midline |Optic Nerve Gliomas associated with Von Recklinghausen’s (NF I) |
| | |Rosenthal Fibers |Rosenthal Fibers | |
| | | |Von Recklinghausen’s (NF I) | |
|Cerebellar Pilocytic Astrocytoma | |60% cystic |Children (2nd decade) |Most common astrocytoma of childhood |
| | |Endothelial proliferation & pleomorphism | |Good Prognosis |
|Subependymal Giant Cell Tumor | |Vascular Intraventricular Mass |Intraventricular |Benign but cause problems due to location & hemorrhaging |
| | |Cells = Large, mix between astrocyte & neuron |Tuberous Sclerosis | |
| | | |Hemorrhage | |
|Glioblastoma Multiforme | |Supratentorial |Common |Most common glioma, Usually in older adults |
| | |Rapid growth, endothelial proliferation, |Adults |Most invasive & aggressive, highly infiltrative |
| | |necrosis |Always Recurs |Tumor always recurs with resection, poor prognosis |
| | |Hemorrhagic Foci, Often zones of mixed tumor |Metastasis |Mix: Oligodendroglioma,Ependymoma,Astrocytoma,Neuroectederm |
| | |May Metastasize (CSF) | | |
CNS Pathology (Contd. 3)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Oligodendroglioma | |Cerebral White Matter, Focal Calcification |Focal Calcification |Unusual in children |
| | |Fried Egg Appearance, Highly vascularized |LOH 1p, LOH 19q |Good = both LOH 1p & 19q |
| | | |CDKN2A |Bad = CDKN2A |
|Ependymoma | |Ventricular System (4th Ventricle is usual) |4th Ventricle |All ages, but more often young>old & children = infratentorial |
| | |Rosette & Pseudorosette Arrangement |NF II |Most common spinal intramedullary glioma, Associated w/ NF II |
| | |Vascularized, differentiated & demarcated |Rosettes & Pseudorosettes |Best to Worst: Spinal Cord, Cerebrum, Posterior Fossa |
| | |May Metastasize |Metastasis | |
|Myxopapillary Ependymoma | |Mucinous Degeneration of Stroma |Cauda Equina |Rare in Children, Usually benign |
| | | |Fila Terminale |Can be locally destructive, Metastatic & can recur |
| | | |Adults | |
| | | |Mucinous Degeneration of Stroma | |
|Subependymoma | |Usually 4th Ventricle |4th Ventricle |Benign, cured by excision |
| | |Extend of caudate or Septum into Lat Ventricle | | |
|Medulloblastoma | |Children = midline, young adults = lateral |Sheets or Rosettes |Young>Old, Accounts for 1/3 of posterior fossa neoplasms |
| | |Grows in sheets or rosettes |CSF Metastasis |Invades leptomeninges & seeds CSF, Good prog except large cell |
| | |May Metastasize into CSF |Myc Amp & LOH 17p |Bad = < 3yo, Mets, Large Cell Variant, Myc Amp, LOH 17p |
| | | |Retinal S, Rhodopsin, TrkC R |Good = Retinal S Antigen, Rhodopsin & TrkC receptor |
|Meningioma | |Grows in sheets w/ poor cytoplasmic borders |Women |Rare in children, More common in adult women |
| | |Whorls & Psammoma Bodies |Attached to Dura |Slight tendency to recur after excision |
| | |Vascular, Attached to dura, Benign |Benign but Compress Brain |Numerous Variants |
| | |Hyperostic Rsponse |Hyperostic Rsponse |Bad = Clear Cell, Rhabdoid, Chordoid & Papillary Subtypes |
|Intracranial Schwannoma | |Encapsulated, Displaces, not Replaces nerves |Encapsulated & Displaces |Spindle = Antoni A (Verocay Bodies); Stellate = Antoni B |
| | |Biphasic (Spindle & Stellate) Growth Pattern |Female |Bilateral Acoustic Schwannomas = von Recklinghausen’s (NF I) |
| | |CN VIII @ Cerebellopontine Angle or IAM |CN VIII |Also common in Neurofibromatosis Type II (NF II) |
| | |CN V (less common) |von Recklinghausen’s | |
|Intraspinal Schwannoma | |Encapsulated, Displaces, not Replaces nerves |Encapsulated & Displaces |Spindle = Antoni A (Verocay Bodies); Stellate = Antoni B |
| | |Biphasic (Spindle & Stellate) Growth Pattern |Cystic |30% of spinal tumors |
| | |All Spinal levels | | |
| | |Cystic | | |
|Peripheral Schwannoma | |Encapsulated, Displaces, not Replaces nerves |Encapsulated & Displaces |Spindle = Antoni A (Verocay Bodies); Stellate = Antoni B |
| | |Biphasic (Spindle & Stellate) Growth Pattern |von Recklinghausen’s |Usually solitary, if multiple = von Recklinghausen’s |
|Neurofibroma | |Arise from small peripheral nerves or trunks |Replaces nerve |Usually multiple, part of von Recklinghausen’s |
| | |Tumor mixed with collagen, reticulin & nerve |Multiple |Plexiform Neurofibroma = von Recklinghausen’s |
| | |Hyperplasia of nerve supporting elements |von Recklinghausen’s | |
|Capillary Hemangioblastoma | |Cerebellum, Retina, Medulla, Cervical Cord |Men, Cerebellum |Adult Male Predominance |
| | |Supratentorial Meninges |Foamy Cytoplasm |Lindau’s: Hemangioblastomas, Cysts, Adrenal/Renal Tumors |
| | |Foamy Cytoplasm |Erythropoietin Substance |Add Retinal Angiomatosis = Von Hippel Lindau Syndrome |
| | |Erythropoietin Substance→Polycythemia |Lindau Syndrome | |
|Primary Sarcoma | |From Fibroblasts | |Rare |
| | |Meninges, Perivascular, Choroid, Tela Choroidea| | |
| | |Attached to meninges | | |
CNS Pathology (Contd. 4)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Primary Lymphoma | |Diffuse Large B Cell |Concentric Laminae |Rare outside of immunosuppressed, Common in AIDS |
|(Reticulum Cell Sarcoma) | |Diffuse Large B Cell Immunoblastic |Reticulin | |
| | |Diffuse Small B Cell Cleaved 9.5 |B Cell | |
| | |Angiocentric growth, laminae, reticulin | | |
|Craniopharyngioma | |Suprasellar (Rathke’s Pouch) |Children |Most Common Supratentorial Tumor in children |
| | |Solid or Cystic, Cords or Sheets (Epithelial) |Hydrocephalus |Often compresses Optic Chiasm or Hypothalamus |
| | |Keratin, Cholesterol Rich Cyst Fluid |Keratin & Cyst Fluid |May bulge into 3rd ventricle & compress CSF flow |
| | |Rosenthal Fiber, Sharp Margins |Rosenthal Fiber |Slow growing, benign (difficult to irradicate due to islands) |
|Germ Cell Neoplasms | |Pineal or 3rd Ventricle |Pineal or 3rd Ventricle |Most common in adult males |
| | |May Metastasize |Males |Most common Germ Cell Tumor of CNS |
| | | |Metastasis |Same as Dysgerminoma/Sminoma, Embryonal Carcinoma, |
| | | | |Chorangiocarcinoma, Teratoma & Endodermal Sinus Tumor |
|Epidermoid Cyst | |Stratified Squamous Lining with Keratin |Skin |Rupture can cause Meningitis→Granuloma |
| | |Partially Calcified |Pons (Cerebello-Pontine Angle) | |
| | |Pons (Cerebello-Pontine Angle) |Keratin | |
| | | |Calcified | |
|Dermoid Cyst | |More Sebacious Glands |Skin |Rupture can cause Meningitis→Granuloma |
| | |Midline of Posterior Fossa |Sebacious “Cheese” | |
| | | |Midline of Posterior Fossa | |
|Meningeal Metastasis | |Rare in Cranium, more common in Spine |Usually Multiple |Most Common = Lung>Breast>Melanoma>Kidney>GI Tract |
|(Metastatic Neoplasms to CNS) | |Hematogenous Seeding, Dissemination via CSF |Spine | |
| | |May enter canal through Dural Root Sleeves | | |
|Parenchymal Metastasis | |More common in Cerebrum than Cerebellum |Usually Multiple |Most Common = Lung>Breast>Melanoma>Kidney>GI Tract |
|(Metastatic Neoplasms to CNS) | |Lodge in grey matter at junction with white |Cerebrum | |
| | |matter |Grey Matter | |
|Transient Ischemic Attack | | | |Deficits occur & disappear completely; Important Predictor of |
|(TIA) | | | |Stroke |
| | | | |(if greater than 30-60 minutes causes infarction) |
| | | | |½ of patients have a diffusion weighted MRI abnormality |
|Cerebral Infarction |Carotid,Vertebral, Basilar |Middle>Posterior>Anterior Cerebral Arteries |Stuttering Onset, at night |Ischemic Necrosis; Most common of all CV Disease |
| |Atherosclerosis |Vasodilation & Immediate Metabolic Change |Older Patients & TIA |1-2d: loss of definition of grey & white, edema; 2-10d: |
| |Age>60, Arteriorsclerosis (DM & HT) |Acidosis; Edema; ↑ Glu; Ca2+ Influx; Free |Atherosclerosis |demarcation |
| |Inflammation, Compression, Spasm |Radical |c-fos, cjun & HSPs |>10d: liquefaction & cyst formation |
| |Polycythemia, Hypotension, ↓ O2 |PMNs; c-fos, cjun & HSPs | |Goal of focal ischemic stroke is to protect penumbra |
|Cerebral Embolus |Heart Arrhythmias & MI |Middle Cerebral Artery |Arrhythmias & MI |Fat emboli cause petichial hemorrhages in white matter of |
| |Aorta & Carotid Plaques |Lesions in cortex & corticomedullary junction |Sudden Onset |cerebrum |
| |Endocarditis | |Multiple Regions |Change of “no flow/reflow” hemorrhage |
| |Surgery & Trauma | |Rapid Improvement (some) | |
|Cerebral Hemorrhage |Hypertension |Charcot-Bouchard Aneurysms |Hypertension |Putamen & Pons = Death;Thalamus & Cerebellum = Recoverable |
| |Amyloid |Disection of parenchyma, rupture into |Waking Hours |Older pts with amyloid can get lobar hemorrhage w/o hypertension |
| | |ventricles |50% Coma |High 30 day mortality |
| | |Hematoma ringed by petichiae |Some stiff neck/headache | |
| | |Large amounts of hemosiderin after recovery | | |
|Subarachnoid Hemorrhage |Head Trauma |Arterial Spasms & Cerebral Infarction |Head Trauma (Saccular Aneurysm) | |
| |Ruptured Aneurysms, Neoplasms |Hydrocephalus |Sudden Onset during Day | |
| |Arteriovenous Malformations, Dyscrasias| |Headache & Stiff Neck in all | |
| |Extension of Hemorrhage | |Younger Patients; Seizures | |
CNS Pathology (Contd. 5)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Lacunar Infarction |Hypertension |Fibrinoid necrosis | |Small (< 1 cm) |
| | |Hyalinization of media & narrowing of lumen | | |
| | |Grey Matter, Internal Capsule, Basis Pontis & | | |
| | |Centra Semiovales | | |
|Mycotic Aneurism |Bacterial Emboli |Distal to bifurcation of Circle of Willis |Cerebral Embolus |Cause Subarachnoid & Intracerebral Hemorrhage |
|Charcot-Bouchard Aneurysms |Hypertension |Found on penetrating intraparenchymal arteries |Cerebral Hemorrhage |Microaneurysms |
| | |Cerebral Hemispheres, Cerebellum & Brainstem | | |
|Saccular Aneurysms |Congenital Defect at Bifurcation |85% in anterior circulation |Subarachnoid Hemorrhage |Berry Aneurysm |
| |Degeneration of IEL |Internal Carotid, Anterior Cerebral | |Multiple in 20% of patients |
| |Atherosclerosis |& Anterior Communicating | | |
| | |Hemorrhage or Compression | | |
|Fusiform Aneurysms | |Vertenral, Basilar or Internal Carotid |Don’t Rupture | |
| | |Segmentally Dilated | | |
| | |Compression | | |
|Capillary Telangiectasis | |Pons or Cerebral White Matter |Pons or Cerebral White Matter |Small Dilated Capillaries separated by neural tissue |
| | |Focal Seizures | | |
| | |Repeated Subarachnoid Hemorrhage | | |
| | |Intracranial Bruits | | |
|Cavernous Malformations | |Focal Seizures | |Dilated Sinusoidal Vessels without neural tissue |
| | |Repeated Subarachnoid Hemorrhage | | |
| | |Intracranial Bruits | | |
|Arteriovenous Malformation | |Found along Middle Cerebral Artery |Middle Cerebral Artery |Composed of arteries & Veins |
| | |Focal Seizures | | |
| | |Repeated Subarachnoid Hemorrhage | | |
| | |Intracranial Bruits | | |
|Venous Malformation | |Found over Subarachnoid Space over Cord |Subarachnoid Space over Cord |Large Numbers of Veins |
| | |Focal Seizures | | |
| | |Repeated Subarachnoid Hemorrhage | | |
| | |Intracranial Bruits | | |
|Venous Occlusive Disease |Infection, Heart Disease, Post-op |Superior Sagital Sinus or Superior Cerebral |Sinus or Veins |Bad = If thrombosis spreads into bridging veins (don’t use TPA) |
| |Trauma |Veins | | |
| |Carcinoma, Fever, Dyscrasias, |Hemorrhage into leptomeninges | | |
| |Dehydration, Cachexia, Hypotension |Hemorrhagic Infarction | | |
| |Puerperium |Hemorrhage in cortex (white matter) | | |
|Acute Pyogenic Meningitis |Neonates: Group B Strep & E. coli |Fever, Headache, Photophobia, Nuchal Rigidity |↓ Glucose (CSF) |A leptomeningitis (leptomeninges & subarachnoid CSF) |
|(Bacterial) |2 y: N. meningitides |↑ CSF Pressure, PMNs, Protein; ↓ Glucose |Medical Emergency |Complications: Hydrocephaly, Infarct, Blindess, Deafness, Ocular |
| |Elderly: S. pneumoniae & Listeria |Hydrocephaly, Infarct, Seizures, CN Palsies | |Palsy, Seizures |
|Aseptic Meningitis |Enterovirus |Effects more mild than Bacterial |Lymphocytes |Usually self-limiting, no long term effects |
|(Viral) |(Echovirus, Coxsackie or Poliovirus) |↑ CSF Pressure, Lymphocytes, Protein | | |
CNS Pathology (Contd. 6)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Chronic Meningitis |Mycobacteria (TB) |Less Fulminating than Bacterial |↑↑ Protein |Meningoencephalitis (Meninges & Brain Parenchyma) |
|(Mycobacterial) | |↑ CSF Pressure, Mono’s or Mixed, Protein |Mononuclear Cell |Very hard to treat, high rate of complications |
| | |Basal Meninges |Basal Meninges |CSF Protein may be very high with TB |
| | |Granulomatous Reaction (not pyogenic) | | |
|Chronic Meningitis |Candida |Less Fulminating than Bacterial |↑↑ Protein |Meningoencephalitis (Meninges & Brain Parenchyma) |
|(Fungal) |Mucor (with Diabetic Ketoacidosis) |↑ CSF Pressure, Mono’s or Mixed, Protein |Mononuclear Cell |Very hard to treat, high rate of complications |
| |Aspergillus |Basal Meninges |AIDS & Diabetes |CSF Protein may be very high with TB |
| |Cryptococcal (with AIDS) |Granulomatous Reaction (not pyogenic) |Basal Meninges |Mucor & Aspergillus tend to invade blood vessels (hemorrhagic) |
|Brain Abscess |Bacteria (Staph & Strep) |Herniation due to mass effect | |Predisposed: Endocarditis; Heart Disease; |
| |Fungi (often immunodeficient) |Rupture into ventricles | |Lung, Sinus & Tooth Infections |
| |Protozoa (Toxoplasma - AIDS) | | | |
|Viral Encephalitis |Eastern Equine-Epidemic |Mononuclear Infiltrate, Microglial Nodules | |Eastern Equine = most virulent, 20-40% mortality |
|(Arbovirus) |West Nile Epidemic |Neuronophagia, Viral Inclusion Bodies | | |
| |Western Equine | | | |
|Viral Encephalitis |HSV-1 |Mononuclear Infiltrate, Microglial Nodules |Inferior Temporal & Frontal Lobes |HSV-1 = Most common cause of sporadic acute encephalitis |
|(Herpes Simplex Type I) | |Neuronophagia, Viral Inclusion Bodies | |Labial Herpes not a significant risk factor |
| | | | |Treatment = Acyclovir |
| | | | |Tropism for Temporal & Frontal Lobes except in neonates (pan) |
|Viral Encephalitis |HSV-2 |Mononuclear Infiltrate, Microglial Nodules | |In immunodeficient pts & neonates; In others = aseptic meningitis|
|(Herpes Simplex Type IIK) | |Neuronophagia, Viral Inclusion Bodies | |High Risk = Babies born to mothers with active genital herpes |
| | | | |Neonates = generalized panencephalitis (no tropism) like above |
|Viral Encephalitis |VZV |Mononuclear Infiltrate, Microglial Nodules |Posterior Ganglionitis |Exemplifies latency |
|(Varicella Zoster Virus) | |Neuronophagia, Viral Inclusion Bodies |Latency | |
| | |Inflammation of a sensory ganglion | | |
|Viral Encephalitis |Rabies |Mononuclear Infiltrate, Microglial Nodules |Hyperexcitability |Peripheral Nervous System as route of entry |
|(Rabies) | |Neuronophagia, Viral Inclusion Bodies |Pharyngospasm |(slowly spreads along peripheral nerves) |
| | |Hyperexcitability | |Slight Touch: Pain,Violent Motor Response, Convulsions |
| | | | |Swallowing: Pharyngospasm (fear of water) |
|Viral Encephalitis |CMV |Mononuclear Infiltrate, Microglial Nodules |Periventricular |Most common opportunistic virus infecting CNS in AIDS |
|(Cytomegalovirus) | |Neuronophagia, Viral Inclusion Bodies | |Succeptability in fetuses & immunodeficient |
| | | | |Periventricular localization |
|Progressive Multifocal |JC Virus |Mononuclear Infiltrate, Microglial Nodules |Oligodendrocytes |Only immunodeficient individuals succeptable |
|Leukoencephalopathy | |Neuronophagia, Viral Inclusion Bodies |Loss of myelin |Tropism for oligodendrocytes |
| | |Widespread loss of myelin | |Poor Prognosis |
|Viral Encephalitis |HIV-1 |Mononuclear Infiltrate, Microglial Nodules |Doesn’t Infect Neurons |Infected: Macrophages, Giant Cells Microglia (NOT neurons) |
|(HIV) | |Neuronophagia, Viral Inclusion Bodies | |Can lead to: Toxo, CMV, Cryptomeningitis, PML |
| | |Dementia, Ataxia, Incontinence, Seizures | |2 most common focal lesions: Toxo Abscess & 1º Lymphoma |
| | |Cerebral Atrophy, Ventriculomegaly | |Infection is rare in congenital AIDS |
|Neurosyphilis |Treponema pallidum | |Absence of Deep Tendon Reflexes |Meningovascular: Meningitis, Meningeal Arteritis |
|(Tertiary Syphilis) | | |Charcot Joints |Paretic: Invastion of parenchyma (dementia & motor instability) |
| | | |“Lightning Pains” |Tabes Dorsalis: Destruction of Dorsal Roots (Ataxia, Skin & Joint|
| | | | |Lesions[Charcot Joints], Dysesthesia, No deep tendon reflexes) |
CNS Pathology (Contd. 7)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Neuroborreliosis |Borrelia burgdorferi |Meningitis | |Major arthropod born disease in US; Evolves through 3 stages |
|(Lyme Disease) | |Encephalopathy | |Nervous System involved in Stage 2 (months) & 3 (years) |
| | |Facial-nerve paralysis | | |
| | |Polyneuropathies | | |
|Transmissible Spongiform |Prion (PrPSc) |Dementia, Ataxia, Insomnia, Paraplegia |PrPSc |Most Common is Sporadic = Creutzfield-Jakob Disease (CJD) |
|Encephalopathy |Sporadic |Paresthesias, Deviant Behavior |No Inflammation |Infection: Corneal Transplants, Electrodes, Dural Grafts & GH |
| |Genetic |No Inflammation; Vacuolization of Tissue | |Course is relentlessly progressive, fatal in a year |
| |Infectious |(Dementia & Startle Myoclonus = CJD) | | |
|Alzheimer’s Disease |Head Trauma, Early Low Linguistic Skill|Progressive decline in memory & cognitive fxn |APP, PS1,PS2 |½ of pts over 85 have AD |
| |Low Educational Achievement |Personality & Behavioral Changes |Tau Tangles |Areas Affected: Basal Nucleus (Ach), Locus Ceruleus (NE) & Dorsal|
| |APO E-4 |Neurofibrillary Tangles (Tau) & Plaques (Aβ) |Aβ Plaques |Tegmentum (5HT); Entorhinal Cortex & Hippocampus |
| |APP(21), PS1, PS2 & Chr 10 Mutations |Loss of CAT & Acetylcholineesterase | |Treatment: Cholinesterase Inhibitors, NMDA Antagonists & Vit. E |
|Frontotemporal Dementia |Inherited |Onset: Personality & Behavioral Changes |Pick Bodies |Pick Bodies = silver positive cytoplasmic inclusions |
|(Pick’s Disease) |Chr 17 & Tau gene mutations |Impaired Judgement, Language & Memory |Chr 17 | |
| | |Frontal & Temporal Lobe Atrophy | | |
| | |Pick Bodies, Balloon Cells | | |
|Parkinson’s Disease |Age, |Tremor, Cogwheel Rigidity, Akinesia |Substantia Nigra |Most Frequent Basal Ganglia Disorder |
| |Mitochondrial Dysfunction |Disturbances of Posture, Equilibrium & |Locus Ceruleus |12 genetic mutations: PARK 1-10, NR4A2, Synphilin-1 |
| |Oxidative Stress |Autonomic Funciton; Dementia |Lewy Bodies | |
| |Excitotoxicity, Toxins (MPTP) |Substantia Nigra & Locus Ceruleus Damaged |Loss of melanin & Dopamine | |
|Dementia with Lewy Bodies | |Dementia, Psychoitc Behavior, Parkinsonism | |Second Most Common Dementia of Elderly |
| | |Lewy Bodies, Senile Plaques, Tangles | | |
|Huntington’s Disease |Genetic (AD) |Choreoathetoid Movement & Dementia |↓ GABA, Glu Decarboxylase, CAT |Genetic Test available for confirmation |
| |HD gene = Chr 4 p16.3 (36-121 CAGs) |GABAergic medium spiny striated neurons | |Patients have a high suicide rate |
| | |Atrophy of Caudate & Putamen | | |
| | |Ventricular Enlargement & Frontal Lobe Atrophy | | |
|Friedreich’s Ataxia |Genetic (AR) |Limb & Trunk Ataxia |Small Spinal Cord |Most Common Inherited Ataxia |
| |Frataxin = 9q13-q21.1 (200-900 GAAs) |Loss of limb proprioception & deep tendon | |Degeneration of posterior spinal cord |
| | |reflex | |Frataxin = unknown function, but disease due to loss of function |
| | |Dysarthria & Extensor Plantar Response | | |
| | |Kyphoscoliosis, Cardiomyopathy & DM | | |
|Spinocerebellar Ataxia Type I |Genetic | | |Second Most Common |
| |Triplet Repeats within gene | | | |
|Dentato-rubro-pallido-luysian |Genetic | | | |
|Atrophy |Triplet Repeats within gene | | | |
|Machado-Joseph Disease |Genetic | | | |
| |Triplet Repeats within gene | | | |
|Amyotrophic Lateral Sclerosis |Excitotoxins, Viral Infection |Upper & Lower Motor Neuron Degeneration |Upper & Lower Motor Neuron |More common in men; rapid progression toward death |
| |Immunological Abnormalities |Lower: Atrophy, Fasiculations, No reflexes |No inflammatory reaction |Familial ALS = Superoxide Dismutase gene on Chr 21 |
| |Trace Elements & Free Radicals |Upper: Hyperreflexia, spasticity | |Difficulty swallowing & chewing; Cell loss most intense in |
| |Genetic (10%) Chr 21 |Demyelination & atrophy of anterior cord | |cervical |
| | | | |No inflammatory reaction |
CNS Pathology (Contd. 8)
|Disease |Cause/Risk Factors |Symptoms |Buzzwords |Other |
|Progressive Bulbar Palsy | |Brainstem Motor Nuclei | |Rapidly Progressive |
|Progressive Spinal Muscular | |Anterior Horn Cells & Nerve Root |Anterior Horn |No change in corticospinal tract |
|Atrophy | | | | |
|Primary Lateral Sclerosis | |Corticospinal Tract |Corticospinal Tract | |
|ALS-Parkinsonism Dementia Complex| | | | |
|of Guam | | | | |
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