Reports on idiopathic head drop
| Weakness | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Report | Sex | Onset age | At onset | Pattern | CK IU/l | Paraspinal mus EMG | Limb EMG | Cx (cervical) paraspinal bx | Limb mus bx | Radiological 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-diagnostic | – | Unchanged 3–5 y |
| Suarez31 case 1 | M | 71 | 2 months | Mild shoulder weakness | N | Fibs, small amp, short duration MUAP; early rec. at lower Cx | – | N | Biceps: ↑ fibre size variability | Degen | Unchanged 5 months |
| 2 | F | 81 | 3 months | Mild deltoid, neck flexor weakness | N | Fibs, small amp, short duration polyphasic MUAP at Cx | – | – | Deltoid: type II atrophy | N | |
| 3 | F | 68 | 3 months | Mild weakness | N | Fibs, small MUAP; early rec at lower Cx | Early rec, sternomastoid mus | Fibre size variability; NADH: moth-eaten; GT: 2 fibres with vacuoles | – | N | |
| 4 | F | 63 | several months | Mild deltoid weakness | 192 | Fibs, short duration MUAP; early rec at Cx and Th | – | – | Biceps: type II atrophy | N | |
| Katz2 case 1 | F | 85 | 6 weeks | Mild neck flexion and deltoid weakness | N | Fibs, short duration MUAP; early rec at lower Cx to mid-Th | N | Fibre size variability, ↑ internal nuclei and fibrosis | Deltoid: N | Mild spondyosis, no neural impingement | Unchanged 6 months |
| 2 | M | 64 | 1 week | Nil | N | Fibs, short duration MUAP; early rec. at lower Cx to Th 1 | N | Fibre size variability, split fibres; ↑ internal nuclei and fibrosis, myofibrillar disarray in hypertrophic fibres | Biceps: N | ” | Unchanged 20 months |
| 3 | F | 84 | 3 months | Nil | N | N | Biceps: N | ” | Unchanged 54 months | ||
| 4 | M | 65 | 2 months | Nil | N | N | As above and ↓ central NADH staining. | Deltoid: N | ” | Unchanged 48 months | |
| Khella34 | F | 84 | – | Nil | N | Short duration, polyphasic MUAP, early rec | – | – | – | – | Stable after 5 y |
| Jaster32 | M | 69 | 4 h | Nil | N | Small amp, short duration MUAP at lower Cx | N | Degen, regen, and necrotic fibres | – | N | Improves after 6 months |
| JHH, case 1 | M | 80 | 3 weeks | Nil | 362 | PSW in Cx 5 and Cx 8 | N | Neurogenic and some myopathic changes | – | N | Improves after 12 months |
| 2 | M | 79 | 1–2 days | Mild weakness of neck flexion and shoulder abduction | N | Small amp, short duration MUAP, early rec at Cx 7–Cx 8 | Small amp, short duration MUAP | Neurogenic changes | Biceps: neurogenic changes | Multilevel degen | Improves after 12 months; has some features of MSA |
| 3 | F | 81 | “Days” | Mild ptosis | N | 1+ fibs, PSW | N | – | Deltoid: N | Mild degen | Remains the same 4 y later |
| 4 | F | 70 | “Days”; pain at onset | Mild proximal weakness | N | N | Few small amp, short duration MUAP | Non-specific myopathic changes | Quadriceps: mild neurogenic changes, type II atrophy | N | Remains the same 4 y later |
| 5 | F | 73 | “Weeks” | Nil | N | Small amp, short duration MUAP; early rec | N | – | Trapezius: type II atrophy | – | Remains the same 4 y later |