amyotrophic lateral sclerosis ( als ) is a progressive neurodegenerative disorder characterized by relentless loss of motor neuron function . the selective degeneration of upper and lower motor neurons results in progressive weakness of the limb , bulbar , abdominal , and thoracic muscles . the reported annual prevalence and incidence rates of als were only 9.9 and 2.3 per 100,000 people per year respectively in asian country , japan . however , the importance of als should not be underestimated since there are more than one in 500 people who will die of the disease in the industrialized world . without mechanical ventilation , most patients die within 2 to 5 years after symptom onset because of respiratory failure , although 5% to 10% of patients may live more than 10 years . up to now , riluzole was the only drug approved by the food and drug administration as neuroprotective treatment / disease - modifying treatment for als that has been shown to slightly slow the course of als . oral administration of riluzole 100  mg daily for als patients is reasonably safe and prolongs a limited lengthening of median survival by 2 to 3 months after 18 months treatment . to our knowledge , other than riluzole , there are no other new treatment that can halt or reverse the progressive loss of neurons , leading to an increase of the als life expectancy . given the fatalness of the illness and lack of effective neuroprotective treatment / disease - modifying agents , complementary and/or alternative medicine ( cam ) is thus increasingly sought to treat als worldwide . traditional chinese medicine ( tcm ) is a main form of cam originating in ancient china and has been practiced for a history of 3000 years . accordingly , chinese herbal medicines ( chms ) are frequently used in the treatment of als . studies in vivo and in vitro showed that chms have a great potential for treatment of als , with neuroprotective function against excitatory amino acid toxicity , oxidative stress , calcium cytotoxicity , and neuroinflammation . during song dynasty ( 9601279 ad ) , the tcm prescription book shengji zonglu ( complete record of sacred benevolence ) compiled by zhao ji who was the eighth emperor of song dynasty of china in 1111 to 1117 ad . this book lists 20,000 tcm prescriptions and describes the causes , symptoms , and cures for different diseases . dihuang yinzi ( dhyz ) , a classical tcm prescription for neurological disorders yinfei syndrome that centered on the symptoms of the speech and language disorders such as aphasis and logopathy ( yin syndrome ) and disorders of motility such as motor paralysis and difficulty in walking ( fei syndrome ) , was first recorded in this book with the name dihuang yin . it is composed of 12 kinds of chms : ( a ) radix rehmanniae preparata 15  g ; prepared rehmannia root ( shudihuang ) , the dried roots of radix rehmanniae recens ; ( b ) fructus corni 15  g ; asiatic cornelian cherry fruit ( shanzhuyu ) , the dried ripe pulp of cornus officinalis sieb . ( c ) herba cistanches 15  g ; desertliving cistanche ( rousongrong ) , the dried roots of cistanche deserticola y. c. ma ; ( d ) radix morindae officinalis 15  g ; morinda root ( bajitian ) , the dried roots of morinda officinalis how . ; ( e ) radix aconiti lateralis preparata 15  g ; prepared common monkshood branched root ( fuzi ) , the processed root of aconitum carmichaeli debx . ; ( f ) cortex cinnamomi 15  g ; cassia bark ( rougui ) , the dried bark of cinnamomum cassia presl ; ( g ) herba dendrobii 15  g ; dendrobium ( shihu ) , the dried roots of dendrobium loddigesli rolfe . or dendrobium fimbriatum hook . var . oculatum hook . or dendrobium chrysanthum wall . or dendrobium officinale kimra et migo or dendrobium nobile lindl . ; ( h ) radix ophiopogonis 15  g ; dwarf lilyturf tuber ( maidong ) , the dried roots of ophiopogon japonicus ( thunb . ) ker - gawl . ; ( i ) fructus schisandrae chinensis 15  g ; chinese magnoliavine fruit ( wuweizi ) , the dried ripe fruit of s chinensis ( turcz . ) baill . ; ( j ) rhizoma acori tatarinowii 15  g ; grassleaf sweetflag rhizome ( shichangpu ) , the dried roots of acorus tatarinowii schott . ; ( k ) radix polygalae 15  g ; milkwort root ( yuanzhi ) , the dried roots of polygala tenuifolia willd . or polygala sibirica l. ; ( l ) poria ; indian bread ( fuling ) 15  g , the dried sclerotia of poria cocos ( schw . ) wolf . ( table 1 ) . in modern times , dhyz is still used continuously and widely for treatment of neurological disorders such as stroke , parkinson disease dementia , and spinal cord injury . here , we reported a case of als patient using modified dhyz who has survived 12 years with significant improvement in bulbar paralysis . on 31 july 2004 , a 41-year - old chinese han nationality woman was admitted to the hospital with complaints of weakened bilateral grip , slurred speech , stumbling , and muscular twitchings for 3 years . the initial symptom , the intermittent weakness of right upper limb without numbness and pain , appeared in november 2001 . after 6 months , uncontrollable twitching was observed in the right upper limb and the patient 's speech became slurred . on 5 march 2002 , the electromyography ( emg ) done at the huashan hospital affiliated to fudan university showed neurogenic injury in bilateral upper limbs and tongue ( table 2 ) . based on the revised el escorial criteria , clinically als was diagnosed . the patient was orally administrated with riluzole ( 50  mg , twice a day ) for 10 months . she stopped the riluzole by herself because symptoms continuously deteriorated and could not bear the economic burden . on 31 july 2004 , she was admitted to the hospital with weakness of limbs , dysarthria , dysphagia , and the clumsiness in daily activities . on examination , the four main vital signs , temperature , heart rate , blood pressure , and respiratory rate , were normal . motor system examination showed widespread muscle wasting and fasciculation of the bilateral thenar and interosseous muscles . strength in the distal muscle of the upper bilateral limbs was of grade 2/5 to 3/5 and muscle strength in the lower bilateral limbs was of grade 3/5 to 4/5 , graded on the medical research council muscle strength grading system . the bilateral hoffman signs were present , whereas the bilateral babinski signs were not present . tcm symptoms and signs were summarized as follows : sluggish speech , faint low voice , choke when drinking , weakness of limbs , muscle wasting of hands , muscular twitchings of upper limbs , pale facial complexion , soreness and weakness of waist and knees , excessive phlegm and saliva , constipation once every 3 days , pink tongue quality , tongue muscle atrophy and fibrillation , deep weak and thready pulse . the electromyography examination atrophy and fasciculations , the onset symptom is insidiously developing asymmetric upper limb weakness and then bulbar muscle . the clinical features are accompanied with the pathological signs : overactive tendon reflexes and clonus . based on the revisited el escorial criteria , she was diagnosed with clinically definite als , depending on the clinical manifestations and consistent electrodiagnostic studies . when the patient experienced bulbar paralysis with symptoms such as dysarthria and dysphagia , the clinical features were classified to yin syndrome in tcm . the kidney meridian dominates feet , throat , and tongue according to meridian theory of tcm . thus , the kidney deficiency leads to yinfei syndrome affecting both bulbar muscles and limbs muscles . following the tcm theory , the patient was diagnosed with yinfei syndrome because of kidney deficiency . once the patient was diagnosed with als firstly in 2002 , she was started on riluzole ( 50  mg , twice a day ) for 10 months . when she noted that the treatment can not arrest the disease condition and she can not bear the economic burden , the patient discontinued treatment because riluzole is not a cure for als . in that time modified dhyz was chosen because it has the function of replenishing kidney essence to treat yinfei syndrome . the compositions and dosage of modified dhyz are as follows : ( a ) radix rehmanniae preparata ( shudihuang ) 15  g ; ( b ) fructus corni ( shanzhuyu ) 12  g ; ( c ) herba cistanches ( rousongrong ) 15  g ; ( d ) herba epimedii 15  g ; epimedium herb ( yinyanghuo ) the dried above ground parts of epimedium brevicornum maxim . , , epimedium wushanensis t. s. ying and epimedium koreanum nakai ; ( e ) radix aconiti lateralis preparata ( fuzi ) 6  g ; ( f ) radix ophiopogonis ( maidong ) 15  g ; ( g ) rhizoma anemarrhenae 15  g ; common anemarrhena rhizome(zhimu ) , the dried rhizome of anemarrhena asphodeloides bge . ; ( h ) rhizoma acori tatarinowii ( shichangpu ) 6  g ; ( i ) radix polygalae ( yuanzhi ) 6  g ; ( j ) fructus trichosanthis seed 30  g ; snakegourd seed ( gualouren ) , the dried seed of trichosanthes kirilowii maxim . or trichosanthes rosthorinii harms . ; ( k ) scorpio 6  g ; scorpion ( quanxie ) , the dried body of artificial breeding of buthus martensii karsch . ; ( l ) agkistrodon 9  g ; long - nosed pit viper ( qishe ) , the dried body of artificial breeding of agkistrodon acutus ( guenther ) . the prescription was prepared from the water decoction and oral for twice daily and the patient has been using it continuously for 12 years . during the 12 years , dhyz has been modified according to the mainly accompanied syndrome or symptoms as follows ( table 3 ) : ( 1 ) qi deficiency : plus radix astragali seu hedysari , milkvetch root ( huangqin ) , the dried roots of astragalus membranaceus ( fisch . ) bge . mongolicus ( bge . ) hsiao or a membranaceus ( fisch . ) bge . ; and radix ginseng , ginseng ( rensheng ) , the dried roots of panax ginseng c. a. mey . ; ( 2 ) obvious yin deficiency : plus radix rehmanniae recens , unprocessed rehmannia root ( shengdihuang ) , the dried roots of rehmannia glutinosa libosch . ; fructus ligustri lucidi , glossy privet fruit ( nvzhenzi ) , the dried ripe fruit of ligustrum lucidum ait . ; and herba ecliptae , yerbadetajo herb ( hanliancao ) , the dried stems of eclipta prostrata l. ; ( 3 ) accumulation of phlegm - fire : minus radix aconiti lateralis preparata ( fuzi ) and cortex cinnamomi ( rougui ) ; plus concretio silicea bambusae , tabasheer ( tianzhuhuang ) , the saps from bambusa textilis mcclure or schizostachyum chinense rendle , succus bambusae ; fresh bamboo sap ( zhuli ) , the saps from bambusa tuldoides munro or sinocalamus beecheyanus ( munro ) mcclure var . henonis ( mitf . ) stapf ex rendle ; and rhizoma arisaematis cum bile ; bile arisaema ( dannanxing ) , the dried roots of arisaema erubescens ( wall . ) schott or arisaema heterophyllum bl . or arisaema amurense maxim . ; ( 4 ) deficiency of heart blood : plus semen ziziphi spinosae , spine date seed ( suanzaoren ) , the dried ripe seeds of ziziphus jujuba mill . spinosa ( bunge ) hu ex h. f. chou ; semen platycladi , chinese arborvitae kernel ( baiziren ) , the dried ripe seeds of platycladus orientalis ( l. ) franco ; and caulis polygoni multiflori , tuber fleeceflower stem ( yejiaoteng ) , the dried stems of polygonum multiflorum thunb . ; ( 5 ) constipation : optionally plus rhizoma anemarrhenae ( zhimu ) ; semen trichosanthis ( guolouren ) ; radix polygoni multiflori , fleeceflower root ( heshouwu ) , the dried roots of p multiflorum thunb . ; semen persicae , peach seed ( taoren ) , the dried seeds of amygdalus persica l. or amygdalus davidiana ( carr . ) c. de vos ex henry ; fructus cannabis , hemp seed ( maziren ) , the dried ripe fruit of cannabis sativa l. ; radix angelicae sinensis , chinese angelica ( danggui ) , the dried roots of a sinensis ( oliv . ) diels ; radix puerariae , kudzuvine root ( gegeng ) , the dried roots of pueraria lobata ( willd . ) ohwi or pueraria thomsonii benth ; semen pruni , chinese dwarf cherry seed ( yuliren ) , the dried ripe seeds of cerasus humilis ( bge . ) sok . or cerasus japonica ( thunb . ) lois . or amygdalus pedunculata pall . ; and herba cynomorii , songaria cynomorium herb ( suoyang ) , the dried stems of cynomorium songaricum rupr . ; ( 6 ) obvious muscular twitchings : plus bombyx batryticatus , stiff silkworm ( jiangcan ) , the dried body of bombyx mori linnaeus . ; periostracum cicadae , cicada slough ( chantui ) , the dried shell of cryptotympana pustulata fabricius ; lumbricus , earthworm ( dilong ) , the dried body of pheretima aspergillum ( e. perrier ) or pheretima vulgaris chen or pheretima guillelmi ( michaelsen ) or pheretima pectinifera michaelsen ; scolopendra , centipede ( wugong ) , the dried body of scolopendra subspinipes mutilans l. koch ; ( 7 ) cervicomuscular weakness : plus radix puerariae ( gegeng ) ; and colla corni cervi , deerhorn glue ( lujiaojiao ) , the glue of horn of cervus nippon temminck or cervus elaphus l. ; ( 8) pain of limbs : plus radix paeoniae alba , white peony root ( baishao ) , the dried stems of paeonia lactiflora pall . ; fructus chaenomelis , common floweringqince fruit ( mugua ) , the dried ripe fruit of chaenomeles speciosa ( sweet ) nakai ; and herba siegesbeckiae , siegesbeckia herb ( xixiancao ) , the dried stems of siegesbeckia orientalis l. or siegesbeckia pubescens makino or siegesbeckia glabrescens makino . accompanied with syndrome or symptom and corresponding modified dihuang yinzi for amyotrophic lateral sclerosis after 12-year treatment and follow - up , no obvious adverse event occurred during the treatment period . in addition , the patient still survived with als and does not require permanent continuous ventilator till today ( figure 1 ) . the symptoms of choking on liquids are improved , and the utility of 30 ml water swallow test was improved with grade 2 . however , muscle strength worsened slowly as follows : tongue muscle atrophy and tongue fasciculations , the slurring of speech , difficulty in communication and use of facial expression , difficulty in activity with both hands and in neck - lifting , muscle wasting of limbs , and presenting with claw hand . strength in the distal and proximal muscle of the upper bilateral limbs was of grade 0/5 and 1/5 to 2/5 respectively , and muscle strength in the lower bilateral limbs was of grade 2/5 , graded on the medical research council muscle strength grading system . at last , we will follow - up the patient continuously . on 7 january 2014 , the repeated emg showed motor conduction amplitude reducing gradually and velocity not changing more when compared with the initial emg ( table 2 ) . a case of amyotrophic lateral sclerosis patient was treated by using a classical chinese herbal prescription dihuang yinzi , who has survived 12 years with significant improvement in bulbar paralysis and do nt require permanent continuous ventilator till now . ( a ) the patient 's appearance ; ( b ) the patient 's tongue muscle atrophy ; ( c ) the patient 's feet ; ( d ) the patient 's palms . on 31 july 2004 , a 41-year - old chinese han nationality woman was admitted to the hospital with complaints of weakened bilateral grip , slurred speech , stumbling , and muscular twitchings for 3 years . the initial symptom , the intermittent weakness of right upper limb without numbness and pain , appeared in november 2001 . after 6 months , uncontrollable twitching was observed in the right upper limb and the patient 's speech became slurred . on 5 march 2002 , the electromyography ( emg ) done at the huashan hospital affiliated to fudan university showed neurogenic injury in bilateral upper limbs and tongue ( table 2 ) . based on the revised el escorial criteria , clinically als was diagnosed . the patient was orally administrated with riluzole ( 50  mg , twice a day ) for 10 months . she stopped the riluzole by herself because symptoms continuously deteriorated and could not bear the economic burden . on 31 july 2004 , she was admitted to the hospital with weakness of limbs , dysarthria , dysphagia , and the clumsiness in daily activities . on examination , the four main vital signs , temperature , heart rate , blood pressure , and respiratory rate , were normal . motor system examination showed widespread muscle wasting and fasciculation of the bilateral thenar and interosseous muscles . strength in the distal muscle of the upper bilateral limbs was of grade 2/5 to 3/5 and muscle strength in the lower bilateral limbs was of grade 3/5 to 4/5 , graded on the medical research council muscle strength grading system . the bilateral hoffman signs were present , whereas the bilateral babinski signs were not present . tcm symptoms and signs were summarized as follows : sluggish speech , faint low voice , choke when drinking , weakness of limbs , muscle wasting of hands , muscular twitchings of upper limbs , pale facial complexion , soreness and weakness of waist and knees , excessive phlegm and saliva , constipation once every 3 days , pink tongue quality , tongue muscle atrophy and fibrillation , deep weak and thready pulse . the electromyography examination the patient is a middle - aged woman . with muscles atrophy and fasciculations , the onset symptom is insidiously developing asymmetric upper limb weakness and then bulbar muscle . the clinical features are accompanied with the pathological signs : overactive tendon reflexes and clonus . based on the revisited el escorial criteria , she was diagnosed with clinically definite als , depending on the clinical manifestations and consistent electrodiagnostic studies . when the patient experienced bulbar paralysis with symptoms such as dysarthria and dysphagia , the clinical features were classified to yin syndrome in tcm . the kidney meridian dominates feet , throat , and tongue according to meridian theory of tcm . thus , the kidney deficiency leads to yinfei syndrome affecting both bulbar muscles and limbs muscles . following the tcm theory , once the patient was diagnosed with als firstly in 2002 , she was started on riluzole ( 50  mg , twice a day ) for 10 months . when she noted that the treatment can not arrest the disease condition and she can not bear the economic burden , the patient discontinued treatment because riluzole is not a cure for als . in that time modified dhyz was chosen because it has the function of replenishing kidney essence to treat yinfei syndrome . the compositions and dosage of modified dhyz are as follows : ( a ) radix rehmanniae preparata ( shudihuang ) 15  g ; ( b ) fructus corni ( shanzhuyu ) 12  g ; ( c ) herba cistanches ( rousongrong ) 15  g ; ( d ) herba epimedii 15  g ; epimedium herb ( yinyanghuo ) the dried above ground parts of epimedium brevicornum maxim . , epimedium wushanensis t. s. ying and epimedium koreanum nakai ; ( e ) radix aconiti lateralis preparata ( fuzi ) 6  g ; ( f ) radix ophiopogonis ( maidong ) 15  g ; ( g ) rhizoma anemarrhenae 15  g ; common anemarrhena rhizome(zhimu ) , the dried rhizome of anemarrhena asphodeloides bge . ; ( h ) rhizoma acori tatarinowii ( shichangpu ) 6  g ; ( i ) radix polygalae ( yuanzhi ) 6  g ; ( j ) fructus trichosanthis seed 30  g ; snakegourd seed ( gualouren ) , the dried seed of trichosanthes kirilowii maxim . or trichosanthes rosthorinii harms . ; ( k ) scorpio 6  g ; scorpion ( quanxie ) , the dried body of artificial breeding of buthus martensii karsch . ; ( l ) agkistrodon 9  g ; long - nosed pit viper ( qishe ) , the dried body of artificial breeding of agkistrodon acutus ( guenther ) . the prescription was prepared from the water decoction and oral for twice daily and the patient has been using it continuously for 12 years . during the 12 years , dhyz has been modified according to the mainly accompanied syndrome or symptoms as follows ( table 3 ) : ( 1 ) qi deficiency : plus radix astragali seu hedysari , milkvetch root ( huangqin ) , the dried roots of astragalus membranaceus ( fisch . ) bge . mongolicus ( bge . ) hsiao or a membranaceus ( fisch . ) bge . ; and radix ginseng , ginseng ( rensheng ) , the dried roots of panax ginseng c. a. mey . ; ( 2 ) obvious yin deficiency : plus radix rehmanniae recens , unprocessed rehmannia root ( shengdihuang ) , the dried roots of rehmannia glutinosa libosch . ; fructus ligustri lucidi , glossy privet fruit ( nvzhenzi ) , the dried ripe fruit of ligustrum lucidum ait . ; and herba ecliptae , yerbadetajo herb ( hanliancao ) , the dried stems of eclipta prostrata l. ; ( 3 ) accumulation of phlegm - fire : minus radix aconiti lateralis preparata ( fuzi ) and cortex cinnamomi ( rougui ) ; plus concretio silicea bambusae , tabasheer ( tianzhuhuang ) , the saps from bambusa textilis mcclure or schizostachyum chinense rendle , succus bambusae ; fresh bamboo sap ( zhuli ) , the saps from bambusa tuldoides munro or sinocalamus beecheyanus ( munro ) mcclure var . henonis ( mitf . ) stapf ex rendle ; and rhizoma arisaematis cum bile ; bile arisaema ( dannanxing ) , the dried roots of arisaema erubescens ( wall . ) schott or arisaema heterophyllum bl . or arisaema amurense maxim . ; ( 4 ) deficiency of heart blood : plus semen ziziphi spinosae , spine date seed ( suanzaoren ) , the dried ripe seeds of ziziphus jujuba mill . spinosa ( bunge ) hu ex h. f. chou ; semen platycladi , chinese arborvitae kernel ( baiziren ) , the dried ripe seeds of platycladus orientalis ( l. ) franco ; and caulis polygoni multiflori , tuber fleeceflower stem ( yejiaoteng ) , the dried stems of polygonum multiflorum thunb . ; ( 5 ) constipation : optionally plus rhizoma anemarrhenae ( zhimu ) ; semen trichosanthis ( guolouren ) ; radix polygoni multiflori , fleeceflower root ( heshouwu ) , the dried roots of p multiflorum thunb . ; semen persicae , peach seed ( taoren ) , the dried seeds of amygdalus persica l. or amygdalus davidiana ( carr . ) c. de vos ex henry ; fructus cannabis , hemp seed ( maziren ) , the dried ripe fruit of cannabis sativa l. ; radix angelicae sinensis , chinese angelica ( danggui ) , the dried roots of a sinensis ( oliv . ) diels ; radix puerariae , kudzuvine root ( gegeng ) , the dried roots of pueraria lobata ( willd . ) ohwi or pueraria thomsonii benth ; semen pruni , chinese dwarf cherry seed ( yuliren ) , the dried ripe seeds of cerasus humilis ( bge . ) sok . or cerasus japonica ( thunb . ) lois . or amygdalus pedunculata pall . ; and herba cynomorii , songaria cynomorium herb ( suoyang ) , the dried stems of cynomorium songaricum rupr . ; ( 6 ) obvious muscular twitchings : plus bombyx batryticatus , stiff silkworm ( jiangcan ) , the dried body of bombyx mori linnaeus . ; periostracum cicadae , cicada slough ( chantui ) , the dried shell of cryptotympana pustulata fabricius ; lumbricus , earthworm ( dilong ) , the dried body of pheretima aspergillum ( e. perrier ) or pheretima vulgaris chen or pheretima guillelmi ( michaelsen ) or pheretima pectinifera michaelsen ; scolopendra , centipede ( wugong ) , the dried body of scolopendra subspinipes mutilans l. koch ; ( 7 ) cervicomuscular weakness : plus radix puerariae ( gegeng ) ; and colla corni cervi , deerhorn glue ( lujiaojiao ) , the glue of horn of cervus nippon temminck or cervus elaphus l. ; ( 8) pain of limbs : plus radix paeoniae alba , white peony root ( baishao ) , the dried stems of paeonia lactiflora pall . ; fructus chaenomelis , common floweringqince fruit ( mugua ) , the dried ripe fruit of chaenomeles speciosa ( sweet ) nakai ; and herba siegesbeckiae , siegesbeckia herb ( xixiancao ) , the dried stems of siegesbeckia orientalis l. or siegesbeckia pubescens makino or siegesbeckia glabrescens makino . accompanied with syndrome or symptom and corresponding modified dihuang yinzi for amyotrophic lateral sclerosis after 12-year treatment and follow - up , no obvious adverse event occurred during the treatment period . in addition , the patient still survived with als and does not require permanent continuous ventilator till today ( figure 1 ) . the symptoms of choking on liquids are improved , and the utility of 30 ml water swallow test was improved with grade 2 . the symptoms of muscle twitching of limbs were also reduced . however , muscle strength worsened slowly as follows : tongue muscle atrophy and tongue fasciculations , the slurring of speech , difficulty in communication and use of facial expression , difficulty in activity with both hands and in neck - lifting , muscle wasting of limbs , and presenting with claw hand . strength in the distal and proximal muscle of the upper bilateral limbs was of grade 0/5 and 1/5 to 2/5 respectively , and muscle strength in the lower bilateral limbs was of grade 2/5 , graded on the medical research council muscle strength grading system . on 7 january 2014 , the repeated emg showed motor conduction amplitude reducing gradually and velocity not changing more when compared with the initial emg ( table 2 ) . a case of amyotrophic lateral sclerosis patient was treated by using a classical chinese herbal prescription dihuang yinzi , who has survived 12 years with significant improvement in bulbar paralysis and do nt require permanent continuous ventilator till now . ( a ) the patient 's appearance ; ( b ) the patient 's tongue muscle atrophy ; ( c ) the patient 's feet ; ( d ) the patient 's palms . this is a long - term follow - up study on a case with als treated by tcm prescription . a middle - aged woman was diagnosed with als based on the revised el escorial criteria . after consuming orally administrated riluzole 100  mg daily for 10 months , she stopped this drug and then started dhyz that she has been using for 12 years . the main findings were that dhyz therapy for als may potentially improve bulbar paralysis , delay use of ventilator support , and prolong survival time ; there were fewer adverse effects . approximately 90% of als cases are sporadic , but the remaining 10% of the cases are familial . the mean age of onset is 58 to 63 years in sporadic and 43 to 52 years in familial cases of als . only 5% of the cases of als have an onset  < 30 years of age . an increased risk in the sex incidence ratio was male : female  =  1.5:1 . in the present case , a female suffering from als was of age 38 years in sporadic who presented with limb onset and subsequently bulbar symptoms . als is primarily a clinically diagnosed disease because of the lack of an established biological marker . diagnosis of als is usually straightforward according to the progressive , generalized symptoms in the limb and bulbar regions . this can result in a delay in diagnosis because als inclines to be focal in onset presented with few clinical signs in the early disease course . the mean lag time between the onset of symptoms of als and confirmation of the diagnosis is 10 to 18 months . in 1994 , the el escorial criteria for diagnosing als clinically were published by a subcommittee on als of the world federation of neurology and the revisited criteria were revised in 1999 , which included the clinical neurophysiolgical measurements as diagnostic tools to exclude differential diagnosis . in 2006 , the awaji criteria placed equal emphasis on both electromyographic evidence of degeneration and clinical abnormalities , thereby potentially enabling an earlier secure diagnosis of als , and this criterion has successfully increased in sensitivity to detect patients with als but additionally showed that this is achieved without increasing the number of false - positives . in 2015 , an updated version of el escorial criteria for the diagnosis of als was published with the purpose of both clinical practice and clinical trial . the new diagnostic criteria of als require at least one of the following : ( 1 ) progressive upper and lower motor neuron deficits in at least one limb or region of the human body ; that is , that is meeting the revised el escorial criteria for possible als ; ( 2 ) lower motor neuron deficits as defined by clinical examination ( one region ) and/or by emg in two body regions ( defined as bulbar , cervical , thoracic , and lumbosacral ) . the emg findings consist of neurogenic potentials and fibrillation potentials and/or sharp waves . in the present study , the patient was diagnosed with als according to revised el escorial criteria 2000 and confirmed both clinical and electrophysiological evidences during long - term follow - up . repeated investigations were required and the diagnosis can be confirmed with disease progression over time . in the initial emg , the damages of different extents occurred in motor nerves which were controlled by neurogenic changed muscle of the patient . motor conduction velocity in the upper and lower limbs was almost normal despite the amplitude decreased in the initial and repeated examination , because the primary abnormality of the peripheral nerve was axonal loss , rather than demyelination . it is critical that tongue innervated by a cranial nerve demonstrated evidence of acute reinnervation confirmed the diagnosis for the positive changes of resting potential , for example , fibs and wave . from the repeated emg , we can find motor conduction amplitude reducing gradually and velocity not changing more when compared with the initial emg . despite advances in the treatments and interventions , there are no medications that stop or reverse the progressive loss of motor neurons of als because of uncertainty on the pathogenic mechanisms underlying degeneration of motor neuron . riluzole remains the only available neuroprotective / disease - modifying drug for als , with only marginal effects on disease survival . although still incurable , als is not untreatable . over the past two decades , remarkable progression in integrative and aggressive supportive care in addition , emphasis has been made in therapies that may even improve the disease course of als . presently , als is considered as a complex disease with broad pathophysiological framework and numerous theories , including oxidative stress , glutamate and neuronal cytotoxicity , protein aggregation , mitochondrial impairment , cytoskeletal disruption , inflammation , apoptotic cell death , and altered regulation of gene expression . thus , combined therapies that focus on more than one pathogenic pathway may slow disease progression in multiple targets / organs interactions . impressively , the key to tcm prescription is to choose a combination of chms guided the combinatorial principle of sovereign - minister - assistant - envoy according to the patient 's syndrome in order to regain the balance state of body functions . over the past decades , many experimental and clinical studies demonstrated tcm prescriptions ; herbal components may have multiple targets and exert neuroprotection or treatment of als . for example , dhyz possesses neuroprotective and antidementia properties through preventing the loss of neural cells and synapses in rats of ischemic brain injury . many studies have demonstrated that each ingredient or its active components of dhyz exerted potential neuroprotective functions ( table 1 ) . rehmannia root and catalpol have antioxidation , antiapoptosis , antiinflammation , and other neuroprotective properties and act in neuroprotection of neurodegenerative diseases.p - coumaric acid , ursolic acid , and gallic acid from corni fructus exerted antioxidation , antiapoptosis , and antineuroinflammation against a-induced toxicity . herba cistanches and its active components possess neuroprotective function , hormone regulation , immunomodulatory , antioxidative , antiapoptotic , and antiinflammatory activities , making them potential treatment for various neurodegenerative disorders . - d - fructofuranosyl ( 22 ) -d - fructofuranosyl from radix morindae officinalis enhanced antioxidative activity , antiapoptotic activity , and energy metabolism against a-induced neurotoxicity . cortex cinnamomi has a neuroprotective effect on glutamate - induced neuronal death through the inhibition of ca influx ; 2-hydroxycinnamaldehyde has antineuroinflammatory and neuroprotective effects in the central nervous system by targeting lipoprotein receptor - related protein 1 . an active compound from dendrobium and chrysotoxine exhibit antioxidant , antiapoptosis , and neuroprotective activities against neurotoxion - induced cell death.o japonicus displays antiinflammatory , antioxidative , and immunomodulative activities . schisantherin a , schisandrin b , and schisandrin c from fructus schisandrae chinensis attenuate abnormal oxidative stress and modulate the apoptotic signal pathway , exhibiting their neuroprotective function on neurodegenerative diseases . -asarone and eugenol , components of rhizoma acori tatarinowii , have neuroprotective function cells through inhibiting apoptosis against a- induced neurotoxicity . polygalae radix has neuroprotective effects against toxin - induced neuronal death through its antioxidant and antiapoptotic activities ; its ethanol extracts as a novel autophagy inducer modulate neurodegenerative disorders via reducing toxicity and clearing of mutant proteins in the cellular level.p cocos water extract has neuroprotective action against a-induced neuronal injury through suppressing the oxidative stress and the apoptosis . in conclusion , we reinforce that tcm prescription , especially dhyz , can be potentially used in als patients because of its multitargeted neuroprotection and general safety , although als has not a cure . in addition , we identified the area that is worthy of further study and dhyz as a promising candidate for further clinical application and als trials .