‘Table 3 Diseases that ean masquerade as ALS. “Table 3 (Conttmied) ‘Anatomical abmormatascompresion syndromes "Ammold-Chiai type {and oer hindbrain malformations Cervical, framan mage oF posterior fosa region tumours Cervical dsc berniaton with osteochondrosis Cereal meningzoma Retropharyngeal tumour Spinal cpidural cyst Spondylotc myelopathy and/or motor radiculopathy Syringomyeta Acquired enzyme defects ‘Adult GM, pangosdoss Denosaminidse A or B deny) Polyghcoxin body dese Pompe's Disease (Glyengen Storage Disease type 1) Auicimmine gndromes ‘Monacional gammopathy with motor neuropathy “Muifocal motor neuropathy witjwithoutconducion lock ‘Dysinmure lower motor neuron syndromes (with GM, GDiy abd alo-GM, antBodies) Otter dysimmmane lower motor neuron syndromes, ncuding CIDP Mulipe sees Myasthenia grave in parbenlar the ant-mutcle- specie receptor tyrosine kinace porte variant) Endoctine abmormaiies ‘Aligow syndrome Diabetic ‘amyotrophy’ Insuinoma causing nowropathy Hyperthyroidism with meopathy ‘Hypothyroidism with myopathy Hyperparathy roi (prima) Hyperparatiyroiisn Geeondary duc to vitamin D defen) Hypokalemia (Cona's syndrome) Bxagenoos toxins ‘Len! @), merry C,cadrium, alumina, asec than, ‘manganese, orginie pesticides neurokhyrsm, kono Iections Aeate potemyetie Pos palomyelisprogssive muscla aophy syndrome val (with vacaclar myelopathy) HTLV-1-associaied myelopathy (tropical spastic paraplegia) ‘Netroborrebods Sophie hypertrophic pachymeningte Spinal encephaieletargics, varcela-zoser Trehinoss ncelodts, at-srath deste Pron deordere Myopathiee Cachecuc myopathy Carcinoid myopathy Dystophin-dsicint myopathy Inclusion body myosits Inlaramatory myopathies Nemaine yopathy Polymyesiis Sarcid ayo Neoplastic syndromes Chronic Iyphocytc Feukeemia Tnvramealary gore {Lgmphopmoiferative disorders with paraprotinemia andor “igoclonal bands in the cerebrospinal Hid Panos! nour syndrome Paraneoplastic encephalomycits with anterior horn cell involvement © 2011 The Auris) “Stiff person plus” syndromes Physical injury “Hectic shock neuronopathy ‘Radiation-induced radieulo-plexopathies and/myclopathy Vasc disorders ‘Anteriovencous malformation ‘Defernc's anterior bulbar artery syndrome Stroke Vases Other neurological conditions ‘Western Pacific atypia forms of MNDJALS (Guam, New Guinea, Kit Penisul of Fapan) CCaniean stypicel forms of MIND-ementia-PSP (Guadeloupe) Madras-form of faverile onset MND/ALS (South India) ‘Frontotemporal dementia with MIND/ALS (nchiding Picks disease with amyotrophy) Mobipl sytem strophy ‘Obvo-ponto cerebelar atrophy syndromes ‘Primary latsral sclerosis (some subtypes not related to ALS) Progresive encephalomyelitis with rigidity PsP Hereditary spastic parapegis (any variants, some subtypes with distal amyotrophy) Prograsive spinal muscular atropby (some subtypes act related toALS) Spincbuibar museuierstrophy with/without dymacin or androgen reccptormutation Spinal musculr trophy IV Brown-Viaktto-van Lacresyndseme (cty-onst bulbar and spinal ALS with sensorineural deafnes) FFasio-Londe syndrome (infantile progresive bulbar paby) Hiarper-Young syndrome (rgngeal and distal spinal mascular atrophy) ‘Monomete sporadic spina! muscu atroghy (benign focal amyotrophy, including Hirayama syndrome) Polyneuropathies with dominating motor symptoms (ike heraitary ‘motor and sensory eeuropathy type 2, ereitary maor neuropathy ype 5) Familial amyloid polyneuropathy ‘Benign fasiculations Myokymia ALS, amyotrophic lateral scleresi; MND, motor ncuron disease; PSP, Progressive supranaclar palsy. 50% of the cases [16-19] (class IV). An evolution of atypical symptoms and a lack of progression of typical symptoms are the most important ‘red flags’ suggesting an alternative diagnosis [16], The diagnosis should be regularly reviewed [18,19]. The revised El Escorial eri- teria (20, summarized in Table 4] are excessively restrictive and are not designed for use in routine clin- ical practice [21]. The new Awaji clectrodiagnostic algorithm [22] added to the El Escorial criteria improves diagnostic sensitivity with no loss in specific- ity [23] and improves early diagnosis as shown in sev- eral class IV studies [24-27]. The clinician must decide, ‘on the balance of probability, whether or not the patient has ALS, even in the absence of unequivocal European Journal of Neurclogy © 2011 EFNS Europaan Joumel of Nouriogy