Case Report
Copyright ©The Author(s) 2022.
World J Clin Cases. Jul 6, 2022; 10(19): 6664-6671
Published online Jul 6, 2022. doi: 10.12998/wjcc.v10.i19.6664
Table 1 Patient clinical characteristics
Case 1
Case 2
Case 3
Age at onset, yr657154
Duration of disease from onset, mo3667
Site of disease onsetLLLRLLLLL
Evolution of symptomsLLL-LUL-RLLRLL-RUL-LLL-LUL-bulbar symptomsLLL-LUL-RLL-RUL-bulbar symptoms
Bulbar symptomsNoneYesYes
Sensory symptoms NoneNoneNone
Tone Hypertonia in left sideHypertonia in right sideHypertonia in left side
Power (MRC grade)Left side (grade 3/5)Right side (grade 4/5)LLL (grade 1/5); LUL (grade 4/5)
ReflexesHyperreflexia in left sideHyperreflexia in all limbs right ankle clonusHyperreflexia in left side, left ankle clonus
Babinski’s signNegativePositive in right sideNegative
Laboratory testNormalNormalNormal
EMGIncreased polyphonic motor unit potentialsUnremarkableMinor chronic denervation
Follow-upMobility with the help of a caneDied from respiratory failure 32 mo after the onset of symptomsDied from respiratory failure 44 mo after the onset of symptoms
Table 2 Differential diagnosis of Mills’ syndrome from amyotrophic lateral sclerosis and primary lateral sclerosis

Mills’ syndrome
Upper motor neuron signsPositivePositivePositive
Lower motor neuron signsNegativePositiveNegative
Initial site of disease onsetUnilateral lower limbCommonly bilateralCommonly bilateral
Progression mannerOne side lower limb - the same side upper limb - contralateral lower limb - contralateral upper limbFrom one segment to the others (cranial, cervical, thoracic, and lumbosacral)Usually ascending
Bulbar involvementLate stageMiddle or late stageLate stage
Symmetry of the symptomSignificant asymmetryCould be symmetric or asymmetricCommonly symmetric
Electrophysiological examinationNon-specialPositiveNon-special
PrognosisUncertain, probably rapid progressionRapid progression, poor prognosisRelatively benign