Table 2.
Neurophysiological (NCS/EMG) Findings in Allgrove (Triple A) Syndrome Patients.
| Ref: | N | Age(s) | Sensory NCS | Motor NCS | Interpretation: |
|---|---|---|---|---|---|
| Goizet 7 | 1 | 33 years | Normal | Normal a | Motor neuron disease |
| Miyazawa 9 | 1 | 38 years | Normal | Low CMAP amplitudes to ulnar and tibial nerves | Motor neuron disease |
| Strauss 8 | 1 | 22 years | Normal | Low CMAP amplitudes to median, ulnar, tibial nerves; CV = normal. | Motor neuropathy with axonal features |
| Messina 15 | 1 | 10 years | Normal | Low CMAP amplitudes to median, ulnar, peroneal. Peroneal CV = 32 m/s (80% LLN) | Motor neuropathy with axonal features |
| Dixit 16 | 1 | 11 years | Normal | Low CMAP amplitude to median, peroneal. | Motor neuropathy with axonal features |
| Koehler 10 | 1 | 14 years | Normal | Normal at 3 yo; developed low CMAP amplitude to median, ulnar & peroneal nerves by age 9 yo. CV = normal | Motor neuropathy with axonal features |
| Houlden 2 | 6 | 18-48 years | Absent/low SNAP amplitude to median (3/6); ulnar (4/6); sural (3/6) b | Low CMAP amplitude to median (3/6); peroneal (5/6) b | Sensorimotor polyneuropathy (motor predominant) with axonal features |
| Kimber 17 | 3 | 40-60 years | Absent/low SNAP amplitude to median (3/3); ulnar (3/3); sural (3/3) | Absent/low CMAP amplitude to median (1/3); ulnar (1/3); peroneal (1/3); tibial (1/2); CV = >80% LLN | Sensorimotor polyneuropathy with axonal features |
| Vishnu 18 | 1 | 36 years | Low SNAP amplitude to median, ulnar, radial SNAP amplitude; Absent sural response. | Low CMAP amplitudes to median, peroneal CV = >74% LLN. | Sensorimotor polyneuropathy with axonal features |
| Vallet 3 | 8 | 21-59 years | Low SNAP amplitudes to median (6/8 patients) and ulnar (5/7 patients) | Low CMAP amplitudes to median (3/8); ulnar (8/8); tibial (8/8). CV = within range of axonal loss | Sensorimotor polyneuropathy with axonal features |
| Gebril 19 | 2 | 13, 15 years | Mixed sensory and motor axonal neuropathy with decreased amplitudes | Sensorimotor polyneuropathy with axonal features | |
| Nakamura 20 | 1 | 60 years | Low median SNAP amplitude; absent sural response | Low median & tibial CMAP amplitudes. CV = 62 = 67% LLN. | Sensorimotor polyneuropathy with axonal and demyelinating features |
| Dumic 1 | 2 | 3-5 years | Mixed sensory and motor demyelinating neuropathy. CV = 67-76% LLN | Sensorimotor polyneuropathy with demyelinating features | |
| Current | 1 | 11 years | Absent sural, peroneal response; low median, ulnar SNAP amplitude; | Low CMAP amplitude to ulnar, peronal, tibial. CV 52 to 86% LLN | Sensorimotor polyneuropathy with axonal and demyelinating features |
Abbreviations: EMG, electromyography; SNAP, sensory nerve action potential; CMAP, compound motor; action potential; CV, conduction velocity; LLN, lower limit of normal; yo, years old.
a
EMG noted diffuse denervation.
b
Progressive decline in amplitude with serial studies performed.