Lysosomal Storage Dz & Glycogen storage dz

All AR except Fabry and Hunter XR : so the Hunter is pointing his gun to the X target. Hurler who's like Hunter [is Mucopolysaccharidosis] but because of corneal clouding he can't hunt well. By the way both Hunter and Hurler accumulate Heparan sulfate [dermatan sulfate is easy to remember once you get the first]. All you need to really memorize then is Hurler is alfa iduronidase and you'll remember the other by exclusion.

Neiman Picks his nose with his "SPHING"er ( finger...so defeciency of sphingomylinase) Because he picks his Nose, he eats his boggers ( nose crap) and gets fatter ( Hepatosplenomegaly- fat stomach)

Using this mneumonic + the other one someone mentioned above ( hyphenated words= Cherry-red macula spot) + the order listed in FA.

FA order:-1- Gauchers 2- Neiman -Pick 3- Tay-sachs

1 and 2- have hepatosplenomegaly in common

2 and 3- Are both hyphenated , i.e- Cherry-Red macula, in common.

So, if u see patient has BOTH HEPATOSPLENOMEGALY AND CHERRY-RED MACULA, it has to be the one in the middle- Neiman-Pick.

(Neiman likes picking things-- just like he likes picking his nose- so, he picks BOTH!! --CHERRY red macula AND HSmegaly!)

--Gaucher-Glucocerebrosidase-Glucocerebroside 3G "have u Got ur iPhone 3G!"

• -Cherry-Red spot is a hyphenated term and so as Nieman-Pick and Tay-Sach.
• -Ashkenazi Jews have Tay-Sachs Nieman-Pick and Gaucher common.
• -Angiokeratoma is a Fabric of vessels and Galaxy of skin spots: which means that Angiokeratoma is seen in Fabry disease in which there's Galactosidase deficiency and epidermal keratosis.
• -Krabbe's disease: just remeber Globoid Cells
• -Metachromatic Leukodystrophy: ... metachromasia (Yellow brown color shift of the sulfatides crystals with toluidine blue). Arysulfatase A deficiency

Glycogen

MNEMONIC for Glycogen Storage Diseases:

Very Poor Casing And Metabolism of Hers