Fig. 27.1
The patient’s hands showing wasting of small muscles, contraction of finger flexors and a purplish discolouration
There was marked distal weakness in the upper and lower limbs bilaterally. Upper limb reflexes were present, knee and ankle jerks absent and both plantar reflexes down-going. Vibration sense was impaired to both ankles, to the right wrist and was normal in the left upper limb. Proprioception was impaired in the fingers and to the right ankle and left knee. Pinprick was reduced up to the elbows and to the top of the thighs. Romberg’s sign was positive, and his gait was ataxic.
Investigations
Blood tests including fasting glucose, HBA1C, vitamin B12, methylmalonic acid, homocysteine, vitamin B6, vitamin B1, folate and ANA were all normal. No paraprotein was detected.
Neurophysiology (Tables 27.1 and 27.2)
Table 27.1
Sensory nerve conduction studies
μV | |
---|---|
Right Median (F3-wrist) | Absent |
Right Ulnar (F5-wrist) | Absent |
Right Radial (forearm-wrist) | Absent |
Left Radial (forearm-wrist) | Absent |
Table 27.2
Motor nerve conduction studies
Right | Left | |
---|---|---|
Median (SE on APB) | ||
DML | 5.8 ms | 5.9 ms |
CV(wrist-elbow) | 31 m/s | 24 m/s |
CMAP(wrist) | 0.7 mV | 1.1 mV |
CMAP(elbow) | 0.5 mV | 1.1 mV
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