Table 3

Reports on idiopathic head drop

ReportSexOnset ageAt onsetPatternCK IU/lParaspinal mus EMGLimb EMGCx (cervical) paraspinal bxLimb mus bxRadiological examination Cx (cervical spine)Outcome
N, normal; ↑, increased; ↓, decreased; CK, creatine kinase; mus, muscle; bx, biopsy; EMG, electromyogram; PSW, positive sharp waves; Fibs, fibrillation; amp, amplitude; MUAP, motor unit action potential; rec, recruitment; regen, regenerative changes; degen, degenerative changes; Cx, cervical segment; Th, thoracic segment; NADH, nicotinamide adenosine dehydrogenase; GT, gomori-trichome; *site not indicated; “–”, not done/information not available; JHH, Johns Hopkins Hospital.
Lange1 (3 patients)*Non-diagnostic*Non-diagnosticUnchanged 3–5 y
Suarez31 case 1M712 monthsMild shoulder weaknessNFibs, small amp, short duration MUAP; early rec. at lower CxNBiceps: ↑ fibre size variabilityDegenUnchanged 5 months
2F813 monthsMild deltoid, neck flexor weaknessNFibs, small amp, short duration polyphasic MUAP at CxDeltoid: type II atrophyN
3F683 monthsMild weaknessNFibs, small MUAP; early rec at lower CxEarly rec, sternomastoid musFibre size variability; NADH: moth-eaten; GT: 2 fibres with vacuolesN
4F63several monthsMild deltoid weakness192Fibs, short duration MUAP; early rec at Cx and ThBiceps: type II atrophyN
Katz2 case 1F856 weeksMild neck flexion and deltoid weaknessNFibs, short duration MUAP; early rec at lower Cx to mid-ThNFibre size variability, ↑ internal nuclei and fibrosisDeltoid: NMild spondyosis, no neural impingementUnchanged 6 months
2M641 weekNilNFibs, short duration MUAP; early rec. at lower Cx to Th 1NFibre size variability, split fibres; ↑ internal nuclei and fibrosis, myofibrillar disarray in hypertrophic fibresBiceps: NUnchanged 20 months
3F843 monthsNilNNBiceps: NUnchanged 54 months
4M652 monthsNilNNAs above and ↓ central NADH staining.Deltoid: NUnchanged 48 months
Khella34F84NilNShort duration, polyphasic MUAP, early recStable after 5 y
Jaster32M694 hNilNSmall amp, short duration MUAP at lower CxNDegen, regen, and necrotic fibresNImproves after 6 months
JHH, case 1M803 weeksNil362PSW in Cx 5 and Cx 8NNeurogenic and some myopathic changesNImproves after 12 months
2M791–2 daysMild weakness of neck flexion and shoulder abductionNSmall amp, short duration MUAP, early rec at Cx 7–Cx 8Small amp, short duration MUAPNeurogenic changesBiceps: neurogenic changesMultilevel degenImproves after 12 months; has some features of MSA
3F81“Days”Mild ptosisN1+ fibs, PSWNDeltoid: NMild degenRemains the same 4 y later
4F70“Days”; pain at onsetMild proximal weaknessNNFew small amp, short duration MUAPNon-specific myopathic changesQuadriceps: mild neurogenic changes, type II atrophyNRemains the same 4 y later
5F73“Weeks”NilNSmall amp, short duration MUAP; early recNTrapezius: type II atrophyRemains the same 4 y later