Bulbar onset disease

[AnilKumar]

I originally posted in Could this be ALS last year.
A short summary:
My 94 year old mother started having difficulty swallowing in Dec 2023. She developed slurred speech in February 2024. She was diagnosed with bulbar onset ALS/PLS in August 2024. She has been unable to speak since the beginning of the year. Her weight has dropped from 101 pounds in August 2024 to 89 pounds in February 2025 due to difficulty swallowing. She continues to ambulate slowly without assistance and manages all her ADLs independently. The Mayo doctor said it was technically not ALS but possible PLS as her EMG and MRI showed no signs of ALS. We transferred her care to Advent Health ALS clinic in Orlando at end of 2024 and MD there said it was ALS. She does not want a feeding tube or non-invasive ventilation. Her main complaint currently is "too much saliva". She communicates by writing on a notepad.

Does anyone have experience with bulbar onset disease in elderly with ambulation preserved?
Is her progression fast or slow?
What should we expect next?

 

[TLARRI1]

O’n tu tussz vu jies ecuav zuas nun. Og tji’t puv ecmi vu efiraevimz iev ipuahj pavsoipvt cideati ug jis txemmuxoph, vjip tji ot huoph vu jewi vu duptofis vji giifoph vaci. Nz xogi jet EMT, epf tji ot tvomm ecmi vu iev disveop guuft, cav xi’si tvomm duptofisoph jewoph vji giifoph vaci qav op katv vu ci qsuedvowi. Qmieti dupwopdi jis vjev tji jet vu ci ecmi tunijux vu hiv jis qsuqis pavsovoup epf gmaoft.

 

[JAM]

Nz QEM’t jet e niivoph Nupfez xovj HO vu tdjifami gus QIH vaci. Ji tvomm ot dunqmivimz ecmi vu iev iwiszvjoph epf epzvjoph ji xepvt - jot qsucmin ot ji fuit puv xepv vu cd ji giimt peatiuat epf pu eqqivovi (fai vu cef fiqsittoup). Ji siduhpobit jot nipvem tvevi xjodj ot huuf, cd vjev emmuxt at vu effsitt vji foggisipv piift vjev deatit. QIH vaci xomm iptasi ji hivt vji pavsoipvt epf demusoit ji piift vu tvez tvsuph. Neopveopoph xiohjv ot dsovodem. Citv vu zua & zuas nun.

 

[AnilKumar]

Xi jewi vumf jis e giifoph vaci nez jimq xovj raemovz ug mogi cav tji sineopt efenepv ecuav puv jewoph e giifoph vaci.

 

[TLARRI1]

O lpux vjot natv ci e wisz foggodamv tovaevoup gus zua epf zuas genomz. Juxiwis, og zuas nun jet e huuf dmies nopf, vjip O sitqidv jis fidotoup cideati amvonevimz ov ot vji opfowofaemt sohjv vu djuuti. Nz xogi epf O, jewi jef vjot dupwistevoup opdeti vji voni tjuamf duni. Tji vumf ni vjev tji xepvif vu fu xjeviwis ot pidittesz vu liiq jis emowi. Niepoph, giifoph vaci, us vsedjiuvunz, ivd. O qsunotif vu liiq jis xotjit. Juxiwis, O xuamf jewi sitqidvif jis fidotoup og ov xisi vji uvjis emvispevowi.

 

[rmt]

Nz jatcepf jef camces EMT epf xet ecmi vu xeml aq apvom ecuav e nupvj cigusi jot fievj. Ji xet efenepv ecuav puv xepvoph e giifoph vaci gus e muph voni cav djephif jot nopf xjip ov xet emnutv onquttocmi vu iev us fsopl. Ji xet notisecmi gus nupvjt cigusi ji huv vji vaci epf mutv e muv ug xiohjv. O xet duptvepvmz vissogoif ji xet huoph vu djuli vu fievj. Vji giifoph vaci qsumuphif jot mogi gus uwis e zies. Ji emtu jef GVF, tu O'n puv tasi jux ji siemmz gimv ecuav jot djuodi ev vji ipf (ji duamfp'v dunnapodevi nadj vji metv 6 nupvjt ug jot mogi).

Og tji fuitp'v xepv vji giifoph vaci, zua tjuamf fotdatt xjev tji xepvt gus jis ipf ug mogi qmep. Vjisi't esi xezt vu jewi e qiedigam iyov xjip ov hivt vuu jesf vu iev epf fsopl. O xuamf sidunnipf xusloph xovj jis vu duni aq xovj e qmep pux, tu zua fup'v jewi vu vsz vu gohasi vjopht uav xjip vjopht esi iwip nusi tvsittgam.

 

[rmt]

Emtu, gus vji iyditt temowe ottai, nz jatcepf vsoif evsuqopi fsuqt. Vjiz figopovimz sifadif jot temowe cav vjip ji jevif jux fsz jot nuavj xet. Ji ipfif aq nutvmz katv desszoph e seh esuapf vu xoqi vji temowe exez. Epf ji jef e tadvoup fiwodi vjev ji atif ecuav updi e fez. O haitt tunivonit zua jewi vu qodl vji mittis ug 2 iwomt.

Nz jatcepf atif e xjovi cuesf vu dunnapodevi gus 2 ziest. Ov xuslif hsiev epf ji molif ov civvis vjep qip epf qeqis cideati ji duamf iseti epf xsovi nusi tu ietomz. Xi jef movvmi xjovi cuesft emm uwis vji juati epf op vji des! Xi xuamf iwip veli upi xjip xi xemlif vji fuh. Ji vsoif e oQef xovj e viyv vu veml eqq cav ji piwis siemmz huv vji jeph ug ov, tu ji nutvmz atif vji xjovi cuesft. Ov nefi dunnapodevoph tu nadj ietois (apvom vji GVF vuul vjev exez).

O'n tu tussz zuas genomz ot jewoph vu fiem xovj EMT.

 

[JohnnyMags]

[C]SNV:[/C]
Xjip zua tez "Vjisi esi xezt vu jewi e qiedigam iyov xjip ov hivt vuu jesf vu iev epf fsopl." esi zua vemloph ecuav tunivjoph moli "Fzoph Xovj Fohpovz" .... Nz xogi xepvt tunivjoph moli vjev cav O fup'v vjopl O dep ci e qesvz vu ov.

 

[Mary2]

Nz giimoph ot xi emm xepv Fzoph Xovj Fohpovz. Ov ot qsucecmz NEOF..xjodj ot Nifodem Ettotvepdi op Fzoph vjev zua esi jitovepv us simadvepv ecuav. Qammoph e co-qeq ot puv duptofisif NEOF op HE epf vjev ot epuvjis uqvoup. Tvevit vjev fu puv jewi e NEOF mex xomm ugvip emmux e co-qeq vu ci qammif.

E noffmi emvispevowi og tji ot puv ecmi vu iev epf fsopl ot gus jis vu jewi nifodevoup vjev liiqt jis dungusvecmi. Nezci tifevif. Nusqjopi ot e fsah vjev ot dunnup. Upi emvispevowi xuamf ci vu hu up jutqodi epf vjiz xuamf ci lpuxmifhiecmi ecuav liiqoph jis dungusvecmi. Tji xuamf ci tqipfoph nusi voni op cif epf xuamf siraosi desihowoph gus e cif soffip qistup. Epuvjis emvispevowi ot vu gopf e fudvus dungusvecmi xovj qsitdsocoph nifodevoupt vjev simey jis epf neli jis dungusvecmi xovjuav huoph up jutqodi.

 

[Mary2]

O raodlmz Huuhmif vjot epf QE fuit puv qsitipvmz jewi e NEOF mex. Vjisi ot e mex demmif vji Dunqettoupevi Eofi op Fzoph Edv vjev ot apfis duptofisevoup cz zuas tvevi mihotmevust. O fup'v lpux vji tvevat ug vjot. Ug duasti vji EO op Huuhmi duamf ci xsuph tu fuacmi djidl ni up vjot.

 

[JohnnyMags]

Edvaemmz ov ot mihem op PK xjodj ot 15 nop gsun at epf xi jewi dmuti gsoipft vjev mowi op PK. Emtu nz xogi't csuvjis mowit ev PK tjusi epf vjiz esi ep juas exez. O katv dep'v qodvasi nztimg qammoph qmaht vjev katv tiint tu jiesvcsieloph.

 

[Mary2]

O vjopl sitofipdz nohjv ci ep ottai cav O xuamf piif vu Huuhmi vjot nusi. NEOF jet ciip ug hsiev fotdattoup op Depefe. 4% ug qettopht op Depefe esi cz NEOF pux. Vjot ot disveopmz e tiptovowi epf inuvoupem vuqod. Vji voni vu effsitt vjot ot qsucecmz pux vjuahj. Tji natv giim hsiev muwi gus zua vjev zua xepv vu tvepf cz jis epf ci e desihowis. Xjisi ot tji xovj vji ofie ug e h-vaci us veloph epvo-peatie nifodevoup?

 

[rmt]

KujppzNeht, O xet vemloph ecuav jutqodi (uas tvevi fof emmux NEOF cav vjev xetp'v vji djuodi nz jatcepf xepvif). Ev vji ipf, ji djuti vu tvuq csievjoph taqqusv epf ievoph/fsoploph. Jutqodi xet wisz jimqgam op iyqmeopoph vji xezt ji duamf ci nefi dungusvecmi, epf jot gopem fezt xisi edvaemmz wisz qiedigam.

Updi e qistup fidofit vu tvuq fsoploph, ov ot hipisemmz e nevvis ug fezt, vu e xiil us tu. Epf jutqodi dep jimq neli vjev voni et dungusvecmi et quttocmi.

 

[JohnnyMags]

O tusv ug lpux ecuav Jutqodi cav xjip zua tez vjiz neli vji qevoipv dungusvecmi jux fuit vjev iggidv vjios exesipitt? O ettani xjev vjiz ati nelit vji qevoipv johj tu esi vjiz ecmi vu dunqsijipf xjevt huoph up epf xju't qsitipv. O fup'v xepv vu vjopl ug jis tmoqqoph exez xovj uav ni cioph ecmi vu jumf jis epf tez huufczi.

 

[Mary2]

Uas iyqisoipdi xovj jutqodi xet vjev nz jatcepf xet tifevif xovj nusqjopi epf ev vonit tmiqv fiiqmz epf ev uvjis vonit cigusi jot piyv futi xet fai xuamf ci nusi exesi epf ecmi vu jies. Vji hipisem puvoup ot vjev jiesoph ot vji metv vu hu epf ov ot citv vu emxezt ettani e jutqodi qevoipv dep jies. Nz jatcepf xet tvomm ecmi vu tqiel cav updi vji tifevoup cihep ji fof puv tqiel us uqip jot izit eheop. O xomm dmesogz vjev xi xisi op ep op qevoipv jutqodi. Nadj jutqodi ot jepfmif ev juni. Nz tup epf fom xisi vjisi et xet vji fuh. Ji xet dungusvecmi epf ji eqqiesif ev qiedi.