TableĀ 1

Transcranial magnetic stimulation (TMS) techniques in amyotrophic lateral sclerosis (ALS) mimic disorders

Mimic disorderRMTMEP amplitudeCMCTCSP durationSICIICFTST
Kennedy's diseaseNormalNormalNormalNormalNormalNormalNormal and abnormal
Acquired neuromyotoniaNormalNormalNormalNormalNormalNormalNot done
DHMNPNormalNormalProlongedNormalNormalNormalNot done
SMANormalIncreasedNormalNormalNormalNormalNot done
FOSMN syndromeNormalNormalNormalNormalNormalNormalNot done
Neuromuscular disorders*NormalNormalNormalNormalNormalNormalNot done
  • Single-pulse TMS studies have established a normal resting motor threshold (RMT) and cortical silent period (CSP) duration in all ALS mimic disorders. The motor evoked potential (MEP) amplitude was reported to be increased in spinal muscular atrophy (SMA), a finding attributed to greater corticomotoneuronal projections onto the surviving motor neurons. In addition, the central motor conduction time (CMCT) was reportedly prolonged in distal hereditary motor neuronopathy with pyramidal features (DHMNP). Short interval intracortical inhibition (SICI) and intracortical facilitation (ICF), assessed by the paired-pulse TMS technique, have been universally normal in ALS mimic disorders. In contrast, triple stimulation techniques (TST) have been reportedly abnormal in Kennedy's disease, suggesting subclinical upper motor neuron dysfunction, although a recent study has reaffirmed functional integrity of corticomotoneuronal tracts in Kennedy's disease (see Utility of peristimulus time histograms section). Single- and paired-pulse techniques have also been normal in facial onset sensory motor neuronopathy (FOSMN) syndrome.

  • *Neuromuscular disorders include demyelinating neuropathy, myasthenia gravis, lead toxicity and Hirayama's disease.