Journal of Neurology Research, ISSN 1923-2845 print, 1923-2853 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Neurol Res and Elmer Press Inc
Journal website http://www.neurores.org

Review

Volume 3, Number 1, February 2013, pages 1-11

Patient Care and Treatment in Amyotrophic Lateral Sclerosis

Figures

Figure 1.
Figure 1. Respiratory management algorithm by AAN ALS Practice Parameter [1]. PFT: pulmonary function tests. PCEF: peak cough expiratory flow. NIV: noninvasive ventilation. SNP: sniff nasal pressure. MIP: maximal inspiratory pressure. FVC: forced vital capacity (supine or erect). Abnl. nocturnal oximetry =pO2 < 4% from baseline. *Symptoms suggestive of nocturnal hypoventilation: frequent arousals, morning headaches, excessive daytime sleepiness, vivid dreams. †If NIV is not tolerated or accepted in the setting of advancing respiratory compromise, consider invasive ventilation or referral to hospice.
Figure 2.
Figure 2. Nutrition management algorithm by AAN ALS Practice Parameter [1]. *e.g., Bulbar questions in the Amyotrophic Lateral Sclerosis Functional Rating Scale, or other instrument.†Prolonged meal time; ending meal prematurely because of fatigue; accelerated weight loss due to poor caloric intake; family concern about feeding difficulties. ‡Percutaneous endoscopic gastrostomy: rule out contraindication.

Tables

Table 1. Symptomatic Treatments in ALS Patients
 
SymptomMedication
CrampsCarbamazepine
Phenytoin
SpasticityBaclofen
Tizanidine
Dantrolene
Botulinum toxin type A
Excessive droolingAtropine
Hyoscine hydrobromide
Hyoscine butylbromide
Hyoscine scopoderm
Glycopyrronium
Amitriptyline
Botulinum toxin injection to parotid glands
Irradiation of the salivary glands
Persistent saliva and bronchial secretionsCarbocisteine
Propranolol
Metoprolol
Cought assist machine
Excessive or violent yawningBaclofen
LaryngospasmLorazepam
PainSimple analgesics
Non-steroidal anti-inflammatory drugs
Opioids
Emotional liabilityTricyclic antidepressant
Selective serotonin-reuptake inhibitors
Levodopa
Dextrometorphan and quinidine
ConstipationLactulose
Senna
Depression, anxietyAmitriptyline
Citalopram
Psychological support and counseling
Lorazepam
InsomniaAmitriptyline
Zolpidem
FatigueModafinil
Psychological support and counseling

 

Table 2. Main Previous Linked Studies Between Disease Progression and Lipid, Urate and Ferritin in Sera or Plasma of ALS Patients
 
Authors. Year [*]CountrySamplesResults
*Reference number; †Univariate analysis; §Multivariate analysis.
Lipid levels
  Dupuis et al. 2008 [38]Francen = 369Long survival in patients with higher LDL-C/HDL-C ratio. §
  Chio’et al. 2009 [39]Italian = 658FCV < 70% in patients with lower levels of TC, HDL-C, LDL-C/HDL-C and TG. §
  Dorst et al. 2011 [40]Germanyn = 488Long survival in patients with higher levels of TC and TG by Kaplan-Meier method.
  Sutedja et al. 2011 [41]Netherlandsn = 303FCV < 70% in patients with lower levels of TC and LDL-C. †
Long survival in patients with higher LDL-C/HDL-C ratio. †
  Paganoni et al. 2011 [43]USAn = 427No relationship between LDL-C/HDL-C ratio and survival. §
  Ikeda et al. 2012 [42]Japann = 92Inverse association between TC and LDL-C levels and rapid worsening of ALS-FRS and FVC. §
  Dedic et al. 2012 [44]Serbian = 82No long survival in patients with hyperlipidemia. †
Urate levels
  Keizman et al. 2009 [48]Israeln = 86Significant relationship between relative decline of urate levels and ALS-FRS. †
  Zoccolella et al. 2011 [49]Italian = 132Long disease duration or bulbar onset in patients with lower urate levels. †
  Ikeda et al. 2012 [42]Japann = 92Inverse association between urate levels and rapid worsening of ALS-FRS and FVC. §
Ferritin levels
  Qureshi et al. 2009 [50]USAn = 90, 99Inverse association between serum ferritin levels and disease progression. §
  Ikeda et al. 2012 [42]Japann = 92Association between ferritin levels and rapid worsening of ALS-FRS and FVC. §

 

Table 3. Main Previous Clinical Trials of Phase II or III in ALS Patients
 
Therapeutic agentAuthors. Year [reference number]
Neurotrophic factors
  Ciliary neurotrophic factorMiller et al. 1996 [51]; ALS CNTF Treatment Study Group. 1996 [52]
  Brain derived neurotrophic factorOchs et al. 2000 [53]
  XaliprodenMeininger et al. 2004 [54]
  Insulin-like growth factorMitchell et al. 2007 [55]
MethylcobalaminKaji et al. 1998 [56]
Vitamin EDesnuelle et al. 2001 [57]; Graf et al. 2005 [58]
CreatineGroeneveld et al. 2003 [59]; Shefner et al. 2004 [60]
CeftriaxoneRothstein et al. 2005 [61]
ONO 2506Traynor et al. 2006 [62]
CelecoxibCudkowicz et al. 2006 [63]
EdaravoneYoshino et al. 2006 [64]
MinocyclineGordon et al. 2007 [65]
TCH346Miller et al. 2007 [66]
KNS-760704 (dexpramipexole)Gribkoff et al. 2008 [67], Cudkowicz et al. 2011 [68]
ArimoclomolLanka et al. 2009 [69]
TalampanelPascuzzi et al. 2010 [70]
LithiumChio et all. 2010 [71]
Tamoxifen[72]
Coenzyme Q10[73]
Copaxone[74]
CK-2017357Shefner et al. 2012 [75]