Ac­cord­ing to the Unit­ed Na­tions, the num­ber of in­di­vid­u­als in the world above age 60 is ex­pect­ed to in­crease rapid­ly. This age­ing pat­tern is es­pe­cial­ly sig­nif­i­cant in de­vel­op­ing coun­tries, where the pro­por­tion of old­er in­di­vid­u­als will in­crease from about 9% in 2015 to 16% by 2040. The num­ber of in­di­vid­u­als di­ag­nosed with chron­ic dis­ease has grown due to this trend. For in­stance, one study found that the num­ber of in­di­vid­u­als with Parkin­son’s dis­ease will dou­ble be­tween 2005 and 2030, and the weight of dis­ease will shift from de­vel­oped na­tions to de­vel­op­ing na­tions.

To­day, Health Plus is plac­ing the spot­light on a dis­ease not well known but is con­sid­ered the most com­mon adult-on­set mo­tor neu­ron dis­ease, Amy­otroph­ic Lat­er­al Scle­ro­sis (ALS) and we con­sult with Dr. Avidesh Pan­day, Con­sul­tant at EWM­SC in Neu­rol­o­gy and Neu­ro­phys­i­ol­o­gy to share from his ex­per­tise and in­sights.

What is ALS?

Our neu­ro­log­i­cal sys­tem is di­vid­ed in­to the cen­tral ner­vous sys­tem and the pe­riph­er­al ner­vous sys­tem. The cen­tral ner­vous sys­tem com­pris­es the brain and spinal cord where­as the pe­riph­er­al ner­vous sys­tem com­pris­es the nerve and mus­cle. These com­po­nents re­sult in the mo­tor (move­ment/strength), sen­so­ry (sen­sa­tion, bal­ance) as well co­or­di­na­tion func­tions.

ALS comes from a large group of dis­or­ders where­by there is pro­gres­sive loss of func­tion in the mo­tor com­po­nents in both the cen­tral and pe­riph­er­al ner­vous sys­tem.

It comes from a larg­er group of dis­or­ders known as Mo­tor Neu­rone Dis­or­der where cer­tain per­sons may get mo­tor loss aris­ing from the cen­tral ner­vous sys­tem, mo­tor loss in the pe­riph­er­al ner­vous sys­tem or both in the case of ALS.

Who gets ALS? How com­mon is this dis­ease pat­tern?

The world­wide preva­lence of ALS is 2-7 pa­tients/100,000. Its cause is un­known al­though there are mul­ti­ple fac­tors in­clud­ing ge­net­ic, smok­ing and cer­tain en­vi­ron­men­tal fac­tors. ALS can oc­cur among many age ranges. Lo­cal­ly we have seen pa­tients in their late 20’s and in their ear­ly 70’s. It’s re­al­ly di­verse in its age range.

Why is this im­por­tant?

Un­for­tu­nate­ly, this con­di­tion is clas­si­fied as a neu­rode­gen­er­a­tive con­di­tion. That means, it pro­gres­sive­ly gets worse with time and there is no cure or mean­ing­ful med­ica­tion to slow its clin­i­cal course. Loss of mo­tor func­tion (strength) re­sults in phys­i­cal weak­ness which can re­duce hand grip strength and limb strength among oth­ers but more im­por­tant­ly bod­i­ly func­tion such as phona­tion of speech, dif­fi­cul­ty in swal­low­ing and with time dif­fi­cul­ties in breath­ing. This not on­ly im­pacts the qual­i­ty of life but threat­ens preser­va­tion of life ul­ti­mate­ly.

What are the symp­toms one should be fa­mil­iar with?

The symp­toms are quite di­verse but the pre­dom­i­nant com­mon de­nom­i­na­tor here is loss of mus­cle strength as well as weight and mus­cle mass loss. Some pa­tients com­plain of “mus­cle jumps/twitch­es”. While we should be fa­mil­iar with these symp­toms, the pres­ence of sen­so­ry symp­toms such as numb­ness and paraes­the­sia “tin­gling or as we know in lo­cal par­lance – “je­ju­ni” should serve as red flags, that this is not ALS.

How is ALS di­ag­nosed?

Most med­ical con­di­tions can be di­ag­nosed with a spe­cif­ic test. For ex­am­ple, a blood test can de­fin­i­tive­ly tell you if there is kid­ney dis­ease or an echocar­dio­gram can help to de­ter­mine if there is heart dis­ease. ALS is a di­ag­no­sis of ex­clu­sion where you have a sus­pi­cion of the ill­ness based on an ex­am­i­na­tion and his­to­ry and then use imag­ing tech­niques such as MRI scans of the Brain and Spine as well as Neu­ro­phys­i­ol­o­gy Tests such as an Elec­tromyo­gram/Nerve Con­duc­tion Stud­ies to help ex­clude any oth­er Neu­ro­log­i­cal ill­ness­es. In some in­stances, blood tests and a spinal tap may be need­ed to aid in ex­clu­sion of oth­er di­ag­noses.

Usu­al­ly, this di­ag­no­sis takes some time to es­tab­lish as sev­er­al in­ves­ti­ga­tions are re­quired to en­sure there is no oth­er Neu­ro­log­i­cal Di­ag­no­sis that will ac­count for the pa­tient’s symp­toms. In ear­ly and late pre­sen­ta­tion, it may be chal­leng­ing to di­ag­nose and on some oc­ca­sions a per­son may be di­ag­nosed with an­oth­er ill­ness but with time their di­ag­no­sis may be re­vised to ALS and vice ver­sa. In some in­stances, this di­ag­no­sis may re­quire two in­de­pen­dent Neu­rol­o­gists.

Is there a cure?

How is ALS treat­ed?

As men­tioned above, there is no cure. The treat­ment re­al­ly is sup­port­ive. In the ear­ly stages of dis­ease, the pa­tients may re­quire phys­i­cal and oc­cu­pa­tion­al ther­a­py to main­tain strength, dex­ter­i­ty and limb func­tion. As the dis­ease pro­gress­es, mul­ti­ple physi­cians are in­volved such as Pul­mo­nolo­gists to as­sist with res­pi­ra­to­ry func­tion, Gas­troen­terol­o­gists/Speech and Swal­low Ther­a­pists to as­sist with swal­low­ing and di­ges­tive func­tion. It is im­por­tant to recog­nise that not on­ly are pa­tients af­fect­ed, rather this in­volves the en­tire fam­i­ly, both in terms of phys­i­cal and emo­tion­al sup­port. In some in­stances, the Psy­chol­o­gist, Psy­chi­a­trist and Med­ical So­cial Work­er may be nec­es­sary.

As the ill­ness pro­gress­es fam­i­lies may have tough de­ci­sions with re­gards to end-of-life care. It is ad­vis­able that pa­tients en­list the ser­vices of Pal­lia­tive Care Physi­cians, ear­ly on the course of their ill­ness to as­sist with main­tain the best qual­i­ty of life for the pa­tient and rel­a­tives for as long as pos­si­ble.

A mul­ti­dis­ci­pli­nary ap­proach is need­ed

It is my de­sire to con­cep­tu­al­ize a mul­ti-dis­ci­pli­nary clin­ic not on­ly for ALS but for many Neu­ro­log­i­cal Ill­ness­es where pa­tients can ac­cess Neu­rol­o­gy/Phys­i­cal Ther­a­py and Re­ha­bil­i­ta­tion/Pul­monolo­gy/Men­tal Health Ser­vices all in one clin­ic vis­it.

Dr. Avidesh Pan­day is a Con­sul­tant at the Er­ic Williams Med­ical Sci­ences Com­plex in Neu­rol­o­gy and Neu­ro­phys­i­ol­o­gy which in­cludes Neu­ro­mus­cu­lar Con­di­tions such as ALS as well as oth­er ill­ness­es such as Epilep­sy/Parkin­son’s Dis­ease and Mul­ti­ple Scle­ro­sis.