This helps me!My mneumonic is kinda adjusted :
Now here is how i think of it :
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. ( from other poster's mneumonic)
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!)
This mnemonic was told to us in kaplan by one of the profs ( i dont remmber name at the moment).. But since then i have forgotten the original wording- but can somehow keep the story strung together. So, bear with me. If this helps u, that's good.. if not im soo sorry for confusion!
There was a boy named TAY who couldnt see well because of cherry red spots on his macula. One night, despite having bad vision, this RETARDED kid took his GM2 out at night and crashed into a witch. Before dying the witch placed a HEX on him.
I think of this story and analyze it this way=
"boy- i think small boy- he can not be fat-- so no hepatosplenomagaly
"named TAY" - Tay sachs disease
"cant see well" b/c of the cherry red macula spot"
"drove at night time"-- also helps me think -- vision, cherry red spot
"took his GM2 car"- GM- stands for general motors : In our case its "gm2"ganglioside
"retarded"- this one is a bit confusing bc this is not mental retardation- but think progressive neurodegeneration and developmental delay-- so BRAIN ISSUES
"placed a HEX on him" A hex in normal language is a curse.. However to us, this means "hexosaminidase A" defeciency.
If this helps even one person, ill feel better
I'll walk you through them and by the end of my post you should feel better
First of all when there's two confusing points "focus on one of them and in the exam you'll remember the other by being not what the first is" have to say that I learned this tip from Goljan
Now let's get started
--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.
--No man picks is nose with hi- Sphinger "Nieman Pick - Sphingomyelinase".
--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: ... well I don't know how to remember Aryl sulfatase