Table 1

Clinical features

Patient
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SexMMMMMMMMMV
Age of onset (y)54454439312355305753
Family history----------
Duration in years681873510611818
First diagnosisALSCMLSMSALSLSParkMSALSLS
Diagnostic delay (y)520628021030
Weakness----+-+---
Site of onsetL legL legLegsLegsR armLegsR legLegsSpeechL leg
Spasticity/Ashworth323222NT222
LMN signs---------+
Sensory deficit----------
Pseudobulbar affect++----+-++
Urinary incontinence++-------+
Barthel/Rankin scale16/316/318/220/24/520/211/418/217/317/3
Medical aidsWCHWCHW-WCH-WCHWWWCH
  • +=Present; -=absent/negative; NT=not tested; ALS=amyotrophic lateral sclerosis; CM=cervical myelopathy; MS=multiple sclerosis; LS=lateral sclerosis; Park=Parkinson; Ashworth scale 0–4; LMN=lower motor neuron; WCH=wheel chair; W=walker.