Table 1

 Clinical details for the 11 patients with flail arm syndrome

Patient NoAge (y)/sexDisease duration (months)ALSFRS-RTriggs hand scoreMRC UL proximal scoreMRC UL distal scoreUMN score
ALSFRS-R, Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised; MRC, Medical Research Council; UL, upper limbs; UMN, upper motor neuron.
†Deceased patients.
Patients with flail arm syndrome had increased survival, with 8 of the 11 patients from the present series still being alive.
Mean age refers to patient age at the time of examination.
Cortical excitability was undertaken in 8 flail arm patients (*), while peripheral nerve excitability was completed in 9 flail arm patients (‡).
Disease duration refers to the period from symptom onset to the date of testing or last review in the ALS clinic.
Patients were clinically graded using the ALSFRS-R, with a maximum score of 48 when there is no disability.
Muscle strength in the proximal upper limbs was graded by summating the MRC score for 12 different movements (ie, shoulder abduction, adduction, internal and external rotation, and elbow flexion and extension bilaterally) for a maximum score of 60, while strength in the distal upper limbs was also assessed for 12 different movements (ie, wrist flexion and extension, finger and thumb abduction, finger extension and flexion bilaterally) for a maximum score of 60.
The UMN score comprised a sum of pathologically brisk reflexes that included the assessment of biceps, supinator, triceps, finger, knee and ankle reflexes, with plantar responses, facial and jaw jerks, all bilaterally, for a maximum possible score of 16. UMN involvement was graded according to the UMN score, with a maximum score of 16. Four patients were graded as having a UMN score of 6 because of hyperreflexia in the lower limbs. Patient No 11 had longstanding hyperreflexia due to a prior closed head injury at age 7 years.
1†*‡59, M5730224380
2*‡50, M2442040606
3*‡71, M9638040520
4†*‡67, M4840248460
5*‡69, M1042240406
6*52, M18044140480
7*‡68, M7415236360
873, M2432224426
9†67, F9628236380
10*‡42, M3146030580
11*‡56, M3640246606