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 +0  (nbme23#22)

Bortezomib, carfilzomib

Mechanism = Proteasome inhibitors, induce arrest at G2-M phase and apoptosis.

Use = Multiple myeloma, mantle cell lymphoma.

Adv Effects = Peripheral neuropathy, herpes zoster reactivation

 +0  (nbme23#21)


Mechanism = Monoclonal antibody against CD20, which is found on most B-cell neoplasms.

Use = B-cell Non-Hodgkin lymphoma, CLL, ITP, rheumatoid arthritis, TTP, AIHA, MS.

Adv effects = Increased risk of progressive multifocal leukoencephalopathy in patients with JC virus.

 +0  (nbme23#23)

One of the tx strategies for drug induced Parkisonism or otherwise is to Curb excess cholinergic activity. Done by Benz-tropine, trihexyphenidyl (Antimuscarinic; improves tremor and rigidity but has little effect on bradykinesia in Parkinson disease). Park your Mercedes-Benz.

 +0  (nbme23#6)

Borderline = Unstable mood and interpersonal relationships, fear of abandonment, impulsivity, self- mutilation, suicidality, sense of emotional emptiness.

Females > males.

Splitting is a major defense mechanism.

Treatment: dialectical behavior therapy.

 +0  (nbme23#37)

Dorsal column (pressure, vibration, fine touch, proprioception)


• Fasciculus graciLis (Lower body, legs) • Fasciculus cUneatus (Upper body, arms)

Dorsal columns are organized as you are, with hands at sides. “Arms outside, legs inside.”

 +0  (nbme23#31)

Male child, recurrent infections, no mature B cells = Bruton disease (X-linked agammaglobulinemia)

Common variable immunodeficiency - Defect in B-cell differentiation. Cause unknown in most cases. May present in childhood but usually diagnosed after puberty. Increased risk of autoimmune disease, bronchiectasis, lymphoma, sinopulmonary infections Decreased plasma cells, Decreased immunoglobulins

 +1  (nbme23#40)

Spleen—arises in mesentery of stomach (hence is mesodermal) but has foregut supply (celiac trunk -> splenic artery.

Splenorenal ligament = Connects Spleen to left pararenal space. It contains Splenic artery and vein, tail of pancreas. It is a derivative of dorsal mesentery.

 +0  (nbme23#46)

Rough endoplasmic reticulum Site of synthesis of secretory (exported) proteins and of N-linked oligosaccharide addition to lysosomal and other proteins. Nissl bodies (RER in neurons)—synthesize peptide neurotransmitters for secretion. Free ribosomes—unattached to any membrane; site of synthesis of cytosolic, peroxisomal, and mitochondrial proteins. Mucus-secreting goblet cells of the small intestine and antibody-secreting plasma cells are rich in RER. Proteins within organelles (eg, ER, Golgi bodies, lysosomes) are formed in RER.

Smooth endoplasmic reticulum Site of steroid synthesis and detoxification of drugs and poisons. Lacks surface ribosomes. Location of glucose-6-phosphatase (last step of glycogenolysis). Liver hepatocytes and steroid hormone– producing cells of the adrenal cortex and gonads are rich in SER.

 +0  (nbme23#23)

I-PRA-tropium, tiotropium Use - COPD, asthma (“I pray I can breathe soon!”).

Muscarinic antagonists competitively block muscarinic receptors, preventing bronchoconstriction. Tiotropium is long acting.

 +0  (nbme23#9)

Q fever Coxiella burnetii, no arthropod vector. Spores inhaled as aerosols from cattle/sheep amniotic fluid. Presents with headache, cough, influenza-like symptoms, pneumonia, possibly in combination with hepatitis. Common cause of culture ⊝ endocarditis. Q fever is caused by a Quite Complicated Bug because it has no rash or vector and its causative organism can survive outside in its endospore form. Not in the Rickettsia genus, but closely related.

 +0  (nbme23#19)

Regulatory T cells Help maintain specific immune tolerance by suppressing CD4 + and CD8 + T-cell effector functions. Identified by expression of CD3, CD4, CD25, and FOXP3.

Activated regulatory T cells (Tregs) produce anti-inflammatory cytokines (eg, IL-10, TGF-β).

IPEX (Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked) syndrome— genetic deficiency of FOXP3 = autoimmunity. Characterized by enteropathy, endocrinopathy, nail dystrophy, dermatitis, and/or other autoimmune dermatologic conditions. Associated with diabetes in male infants.

 +0  (nbme23#24)

Primary polycythemia = Disorder of Increased no of RBCs, usually due to acquired JAK2 mutation. May present as intense itching after shower (aquagenic pruritus). Rare but classic symptom is erythromelalgia (severe, burning pain and red-blue coloration) due to episodic blood clots in vessels of the extremities. There is DECREASED EPO (vs 2° polycythemia, which presents with endogenous or artificially raised EPO). Treatment: phlebotomy, hydroxyurea, ruxolitinib (JAK1/2 inhibitor).

 +0  (nbme23#40)

Early PCT—contains brush border. Reabsorbs all glucose and amino acids and most HCO 3 – , Na + , Cl – , PO 4 3– , K + , H 2 O, and uric acid. Isotonic absorption. Generates and secretes NH 3 , which enables the kidney to secrete more H + . PTH—inhibits Na + /PO 4 3– cotransport = Increased PO 4 3– excretion

 +0  (nbme23#11)

Ingested—Enterobius, Ascaris, Toxocara, Trichinella, Trichuris You’ll get sick if you EATTT these!

Cutaneous—Strongyloides, Ancylostoma, Necator These get into your feet from the SANd

Bites—Loa loa, Onchocerca volvulus, Wuchereria bancrofti Lay LOW to avoid getting bitten

 +0  (nbme23#47)

Shoulder muscles that form the rotator cuff: Supraspinatus (suprascapular nerve)— abducts arm initially (before the action of the deltoid); most common rotator cuff injury (trauma or degeneration and impingement; tendinopathy or tear [arrow in A ]), assessed by “empty/full can” test

Infraspinatus (suprascapular nerve)—externally rotates arm; pitching injury

teres minor (axillary nerve)—adducts and externally rotates arm

Subscapularis (upper and lower subscapular nerves)—internally rotates and adducts arm

 +0  (nbme22#13)

The encounter should begin with the teen and parents discussing the teen's past medical history and parents' concerns

Parents should be asked to wait in the waiting room and be reassured that they will be invited back in to discuss any remaining issues

Once alone with the teen, establish ground rules for confidentiality

The teen's history yields far more information than the physical examination and results of diagnostic tests

 +0  (nbme22#43)

Therapeutic effect of danazol may have two mechanisms of action: (1) promotion of C4 synthesis by anabolic effect resulting in an improvement of the complement system with the disappearance of CIC and (2) a minor increase in C1 inh level primarily due to the lack of its consumption.

Most cases of HAE are caused by mutations of the SERPING1 gene, which contains the information necessary to generate a protein called C1 esterase inhibitor (C1INH). People with HAE produce nonfunctional or insufficient amounts of C1INH, leading to spontaneous swelling in the body. As a consequence of C1INH deficiency, C4 protein levels are also almost always low in HAE patients.

Winstrol = Stanozolol increases levels of C1INH and C4 protein in the blood by enhancing their production. Elevating levels of the two proteins close to the normal range helps prevent HAE attacks. Although the exact mechanism of how it acts on protein production is not fully understood, Winstrol is a well-established treatment for managing HAE.

 +0  (nbme22#43)

There are three main types of HAE.[3] Type I and II are caused by a mutation in the SERPING1 gene that makes the C1 inhibitor protein while type III is often due to a mutation of the factor XII gene.[3] This results in increased amounts of bradykinin which promotes swelling.[3] The condition may be inherited from a person's parents in an autosomal dominant manner or occur as a new mutation.[3] Triggers of an attack may include minor trauma or stress, but often occurs without any obvious preceding event.[3] Diagnosis of type I and II is based upon measuring C4 and C1-inhibitor levels.

 +0  (nbme22#17)

A better way to visualize this is the two hands of a clock, with one hand facing 12 and the other facing 6. The vertical line they form represents the fibula of the person's right leg. The lateral force approaches from 3 o'clock, sending the lower hand snapping outwards to point at 5 o'clock.

Pott's fracture, also known as Pott's syndrome I and Dupuytren fracture, is an archaic term loosely applied to a variety of bimalleolar ankle fractures.[1] The injury is caused by a combined abduction external rotation from an eversion force. This action strains the sturdy medial (deltoid) ligament of the ankle, often tearing off the medial malleolus due to its strong attachment. The talus then moves laterally, shearing off the lateral malleolus or, more commonly, breaking the fibula superior to the tibiofibular syndesmosis.

 -1  (nbme22#24)

This question in it's essence is asking that after inserting the catheter in the femoral artery, which landmark should we use beyond which superiorly lies the renal artery, and the answer is the origin of the testicular artery. What a weirdly worded question though. :?

makinallkindzofgainz  I don't think it's worded that weirdly. The guide wire should be advanced superiorly just beyond the testicular artery to approach the right renal artery.

 +0  (nbme22#42)

Disseminated intravascular coagulation (DIC) involves abnormal, excessive generation of thrombin and fibrin in the circulating blood. During the process, increased platelet aggregation and coagulation factor consumption occur. DIC that evolves slowly (over weeks or months) causes primarily venous thrombotic and embolic manifestations; DIC that evolves rapidly (over hours or days) causes primarily bleeding. Severe, rapidly evolving DIC is diagnosed by demonstrating thrombocytopenia, an elevated partial thromboplastin time and prothrombin time, increased levels of plasma D-dimers (or serum fibrin degradation products), and a decreasing plasma fibrinogen level. Treatment includes correction of the cause and replacement of platelets, coagulation factors (in fresh frozen plasma), and fibrinogen (in cryoprecipitate) to control severe bleeding. Heparin is used as therapy (or prophylaxis) in patients with slowly evolving DIC who have (or are at risk of) venous thromboembolism.

 +0  (nbme22#4)

The right ventricle (RV) receives its arterial blood supply primarily from the right coronary artery (RCA), which arises from the right coronary cusp of the aorta. The division produces the conus artery which supplies blood flow to the right ventricular outflow tract. The sinoatrial node (SA) is also supplied by the RCA from the second division. Coursing in the atrioventricular groove, the RCA then gives off multiple, small branches to supply the anterior RV before dividing terminally into the acute marginal branch (AM) that runs anteriorly along the diaphragm, and the posterior descending artery (PDA) that runs posteriorly. The PDA also supplies the atrioventricular node (AV) in 90% of patients, with a branch of the left circumflex artery providing flow in the remainder of patients. The PDA supplies the inferior wall of both ventricles, and is a terminal branch of the RCA in 85% of patients, but may arise from the left coronary circulation in 15% of the population.

The primary effects of RV ischemia and infarction result from decreased RV contractility. This leads to a reduction in blood flow from the venous system to the lungs, and finally to the left side of the heart. The clinical signs of this are increased right sided heart pressures, increased pulmonary artery (PA) systolic pressures, and decreased left ventricular preload. Symptoms may include peripheral edema especially distention of the jugular vein, hypoxemia, and hypotension.

Additionally, as the RV dilates the motion and function of the interventricular septum is altered. If the RV is dilated secondary to overload or if the septal myocardium is jeopardized by simultaneous left ventricle (LV) ischemia the symptoms of hypotension and cardiac failure may be pronounced. If the septum shifts leftward during diastole it impedes left ventricular filling and as a result cardiac output is decreased. This is termed loss of biventricular interdependence.

 +1  (nbme22#9)

acute hemolytic transfusion reaction (AHTR), also called immediate hemolytic transfusion reaction, is a life-threatening reaction to receiving a blood transfusion. AHTRs occur within 24 hours of the transfusion and can be triggered by a few milliliters of blood. The reaction is triggered by pre-formed host antibodies destroying donor red blood cells. AHTR typically occurs when there is an ABO blood group incompatibility, and is most severe when type A donor blood is given to a type O recipient.Antibodies against A and B blood groups (isohemagglutinins) present in the recipient's blood destroy the donor red blood cells.They also activate the coagulation cascade (blood clotting system) via factor XII, which can lead to disseminated intravascular coagulation and kidney damage. Isohemagglutinins also activate the complement cascade via C3a and C5a, which then promote inflammatory cytokine release from white blood cells. These inflammatory cytokines include IL-1, IL-6, IL-8, and TNF-alpha, which cause symptoms of low blood pressure, fever, chest pain, nausea, vomiting, and wheezing

 +0  (nbme21#19)

Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease resulting from a mutation in the C1-inhibitor gene. HAE is characterized by recurrent attacks of intense, massive, localized subcutaneous edema involving the extremities, genitalia, face, or trunk, or submucosal edema of upper airway or bowels. Routine long-term prophylaxis with either attenuated androgens or C1-esterase inhibitor has been shown to reduce the frequency and severity of HAE attacks.

 +0  (nbme21#13)

Hypoxic pulmonary vasoconstriction (HPV), also known as the Euler-Liljestrand mechanism, is a physiological phenomenon in which small pulmonary arteries constrict in the presence of alveolar hypoxia (low oxygen levels). By redirecting blood flow from poorly-ventilated lung regions to well-ventilated lung regions, HPV is thought to be the primary mechanism underlying ventilation/perfusion matching.[1][2] The process might initially seem counterintuitive, as low oxygen levels might theoretically stimulate increased blood flow to the lungs to increase gas exchange. However, the purpose of HPV is to distribute bloodflow regionally to increase the overall efficiency of gas exchange between air and blood. While the maintenance of ventilation/perfusion ratio during regional obstruction of airflow is beneficial, HPV can be detrimental during global alveolar hypoxia which occurs with exposure to high altitude, where HPV causes a significant increase in total pulmonary vascular resistance, and pulmonary arterial pressure, potentially leading to pulmonary hypertension and pulmonary edema. Several factors inhibit HPV including increased cardiac output, hypocapnia, hypothermia, acidosis/alkalosis, increased pulmonary vascular resistance, inhaled anesthetics, calcium channel blockers, positive end-expiratory pressure (PEEP), high-frequency ventilation (HFV), isoproterenol, nitric oxide, and vasodilators.

Subcomments ...

submitted by sajaqua1(347),

From First Aid- schizotypal personalities exhibit eccentric appearance, odd beliefs or magical thinking, and interpersonal awkwardness. These describe the several layers of clothes, belief in psychic abilities and crystals, and poor eye contact.

A) Borderline- unstable moods with impulsivity, self-mutilation, suicidality, and a sense of emptiness, along with a greater likelihood in women B) Dependent- those with an excess need for support and low self-confidence C)Histrionic- excessively emotional, seeks attention, sexually provocative, concerned with appearance ("actor's disease") D) Narcissistic- grandiosity, entitled, lacks empathy, demands 'the best' E) Schizoid- voluntary social withdrawal, limited emotional expression, content to be on their own

abhishek021196  SCHIZOTYPAL = Included on the schizophrenia spectrum. Pronounce schizO-TYPE-al: Odd-TYPE thoughts. +  

submitted by nwinkelmann(187),

I literally just realized why this question confused me so much (and I've tried to figure it out a couple of times, lol). I let the colloquial definition of bulimia (i.e. vomiting) stick in my mind, that I forgot the actual medical definition = normal BMI (>18) + binge eating and purging (where purging could be induced vomiting or diuretic use or laxative use or/and excessive exercise). So really, what this question was asking is simply what is the electrolyte balance of excessive diarrhea? GEEZ! I made it so much harder in my head when trying to answer it originally.

Diarrhea causes non-anion gap (i.e. hyperchloremic) metabolic acidosis. Stool predominantly contains HCO3- and K+, so excessive diarrhea = excessive loss of HCO3- and K+. Chloride levels in the serum will be increased due to the normal HCO3-/Cl- equilibrium, so as negative charge dissipates due to loss of HCO3-, Cl- will increase correcting the anion-gap.

drdoom  Bulimia comes from Greek "ravenous hunger"; the term is a literal concatenation of the words for ox (bous) + hunger (limos). So, in Greek, bou-limia is literally "ox hunger", which is a nod to how the word is used in medicine = perpetual and insatiable appetite for food (the very "opposite" of vomiting). +1  
abhishek021196  I agree with your reasoning but the classic case description of Bulima lists electrolyte disturbance of HypOkalemia, HypOchloremia, and Metabolic Alkalosis, along with other things like parotid hypertrophy and dorsal hand calluses due to the induced vomiting. I tripped up there. :/ FA 20 Pg 567 +  
llamastep1  Take home lesson: reasoning > memorizing +  

submitted by smc213(79),

To be completely clear!

This patient has Cystinosis a rare autosomal recessive lysosomal storage disorder and most common cause of Fanconi syndrome in children. Cystinosis is systemic and leads to cystine crystal deposits in cells and tissues throughout the body.

Although Wilsons disease can lead to FS, the crystals in the corneas does not correlate with Wilsons disease.
More info: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4841061/

highyieldboardswards  Thank you! You are a legend for figuring this out! +  
paulkarr  Appreciate you. +  
drzed  And even if it was Wilson disease, it would have the exact same consequence leading to Fanconi syndrome. +1  
abhishek021196  Fanconi syndrome Generalized reabsorption defect in PCT = Increased excretion of amino acids, glucose, HCO 3 – , and PO 4 3– , and all substances reabsorbed by the PCT May lead to metabolic acidosis (proximal RTA), hypophosphatemia, osteopenia Hereditary defects (eg, Wilson disease, tyrosinemia, glycogen storage disease), ischemia, multiple myeloma, nephrotoxins/drugs (eg, ifosfamide, cisplatin), lead poisoning. Polyuria, renal tubular acidosis type II, growth failure, electrolyte imbalances, hypophosphatemic rickets = Fanconi syndrome (multiple combined dysfunction of the proximal convoluted tubule). +  

submitted by lfsuarez(114),

This question asks about the mechanism of phototherapy as it relates to neonatal jaundice. With phototherapy, bilirubin is simply converted to water soluble isomers that are then able to be excreted by the kidney. This however does not conjugate the bilirubin.

almondbreeze  FA 2019 pg 387 +  
abhishek021196  Physiologic neonatal jaundice At birth, immature UDP-glucuronosyltransferase = unconjugated hyperbilirubinemia = jaundice/ kernicterus (deposition of unconjugated, lipid-soluble bilirubin in the brain, particularly basal ganglia). Occurs after first 24 hours of life and usually resolves without treatment in 1–2 weeks. Treatment: phototherapy (non-UV) isomerizes unconjugated bilirubin to water-soluble form. +  

submitted by sajaqua1(347),

Wouldn't total AV nodal ablation destroy to autorhythmicity of the pacemaker? That would mean that below the AV node the rhythm would be provided by a ventricular foci, and those usually create wide QRS complexes.

haliburton  that was my reasoning as well. guess not. +  
yotsubato  Shitty NBME grammar strikes again. +1  
charcot_bouchard  No. No guys. Bundle of his located below AV node and it can generate impulse. it calls junction escape rhythm and narrow complex. Below this is purkinje, bundle branch & ventricular muscle. those are wide complex +3  
abhishek021196  Third-degree (complete) AV block The atria and ventricles beat independently of each other. P waves and QRS complexes not rhythmically associated. Atrial rate > ventricular rate. Usually treated with pacemaker. Can be caused by Lym3 disease +1  

submitted by sajaqua1(347),

The marrow shows precursors to erythrocytes, as well as megakaryocytes. This is to show you that the issue isn't underproduction, which means that we are losing RBC and platelets somewhere ie destruction. That rules out D and E. There is nothing to indicate tha the child has disseminated tuberculosis (B). At this point we are left with A or C. A would indicate Disseminated Intravascular Coagulation (DIC) or something similar, which would result in low platelets and RBC but we would also see abnormal RBC like schistocytes ("helmet" cells). We are explicitly told that the erythrocytes are normochromic and normocytic. However, immune destruction of platelets explains it all- the destruction of platelets leads to some hemorrhaging and so a drop in RBC, and ITP classically arises after a recent upper respiratory tract viral infection.

meningitis  Just in case anyone is wondering like I did, the low platelet count explains thethose multinucleated cells. They are Megakaryocytes in Bone Marrow Biopsy. +9  
nwinkelmann  Also, don't forget that autoimmune thrombocytopenia purpura has 2 demographics: young kids, which generally resolves spontaneously fairly quickly, and then young adult females which is a true autoimmune condition that doesn't resolve. Patient's age + thrombocytopenia + essentially normal rest of heme pannel = autoimmune thrombocytopenia purprua in child. +4  
abhishek021196  That is exactly how I approached this question. Normal heme panel and a decreased Platelet count in a young boy after an infection just made me intuitively select ITP. +  

submitted by sajaqua1(347),

This patient has widespread destruction of both adrenal glands, which means that she will lose both her mineralocorticoids and her glucocorticoids. The loss of the mineralocorticoids and the effect on the kidneys is what is being tested here. When aldosterone is functioning and abundant, it has several critical functions on the nephron. First, it upregulates Na+/K+ ATPase expression on the basolateral membrane, establishing a strong electrochemical gradient that better absorbs Na+, while the increased intracellular K+ concentration makes it easier to lose K+ from the cell into the lumen. Aldosterone also upregulates the expression of epithelial sodium channels (ENaC) on the collecting duct, reabsorbing more Na+. Finally, aldosterone increases expression of H+ ATPases on alpha-intercalated cells of the collecting duct, which are responsible for secreting H+ (this is part of contraction alkalosis).

With the loss of aldosterone, we will see a decreases Na+/K+ gradient and decreased Na+ reabsorption; this leads to decreased serum sodium and increased serum potassium. At the same time, not expressing H+ ATPases will lead to H+ not being secreted, creating a more acidic environment (pH will decrease).

abhishek021196  Very well written explanation. Bravo +1  

submitted by sugaplum(122),

This is ridiculous but I could never keep these straight so meet my family:
Achey grandpa Meynert
Dope cousin VT with a side Ho* (who's names are just initials) SNc
Uncle and aunt Raphe and sara Cousin Gabby always screaming Na-Na-Na
norepi reminds me of the color blue, so locus ceruleus

paulkarr  LOL. Achey Granpa Meynert. I'm gonna steal this from you. +  
abhishek021196  Achey grandpa Meynert = ACh / Basal Nucleus of Meynert Dope Cousin VT = Dopamine / Ventral tegmentum, SNc Uncle and aunt Raphe and Sara = Serotonin / Raphe nuclei(medulla, pons) Cousin Gabby always screaming NA-NA-NA = GABA / Nucleus Accumbens Norepi = Locus ceruleus. +  
llamastep1  Amazing +  

submitted by usmleuser007(220),

1) Superficial (first-degree) = Epidermis ~ presents as red skin without blisters

2) Superficial partial thickness (second-degree) = Extends into superficial (papillary) dermis ~ Presents with redness with clear blister & blanches with pressure

3) Deep partial thickness (second-degree) = Extends into deep (reticular) dermis ~ presents as yellow or white skin with less blanching. May be blistering.

4) Full thickness (third-degree) = Extends through entire dermis ~ presents as stiff and white/brown skin. No blanching.

5) Fourth-degree = Extends through entire skin, and into underlying fat, muscle and bone ~ presents as black skin; charred with eschar

endochondral1  what is rhus dermis? +2  
endochondral1  nvm its urshiol +  
btl_nyc  Allergic contact dermatitis because of contact with poison ivy. +  
abhishek021196  Urushiol-induced contact dermatitis (also called Toxicodendron dermatitis or Rhus dermatitis is a type of allergic contact dermatitis caused by the oil urushiol found in various plants, most notably species of the genus Toxicodendron: poison ivy, poison oak, poison sumac, and the Chinese lacquer tree. +1  

submitted by seagull(721),

! I hate these with a burning F***ing passion. Thumbs up if you agree

mcl  Amen brother +  
praderwilli  Every morning: "I think i'll go over glycogen storage diseases, lysosomal storage diseases, and dyslipidemias after questions this afternoon." Every afternoon: Nah +17  
mcl  oh my god are you me +  
praderwilli  I recently found a program called Pixorize. It's pretty much Sketchy for biochem. Wish I discovered it sooner cuz it has helped for a lot of the painful things like this! +3  
tentorium  [special] +  
tentorium  [special] +1  
tentorium  [special] +  
drhello  hello +  
burak  Cherry red spot basically means niemann-pick or tay sachs. Two differences between is: 1- No HSM in Tay Sachs, HSM in niemann-pick. 2- Both of them has muscle weakness but there is hyperreflexia in Tay Sachs, but areflexia in niemann pick disease. In stem cell HSM is not described and hyperreflexia noted. +1  
abhishek021196  What is HSM? +  
mysticsoul  HSM - HepatoSplenoMegaly. Cherry red spots think of Tay Sachs, deficient enzyme - HeXosaminidase A, accumulated substrate GM2 ganglioside. Niemann-Pick - Spingomyelinase, Spingomyelin <- which is not even a choice. FA18 Pg 88 +