engleski

Flail arm syndrome, a form of ALS with slow progression

ALS is a fatal neurodegenerative motor neuron disease (MND). Most frequently, ALS begins with weakness and atrophy of the upper limb muscles (classic ALS), less frequently with bulbar muscle weakness. Flail arm syndrome (FAS) is thought to be a different form of MND or ALS with slow progression. Primary muscle atrophy (PMA) is the form of MND which clinically resembles FAS. In the last 10 years, we have had three male patients with symptoms suggestive of FAS. All of them presented with progressive proximal weakness of the upper limbs. In the further course of the disease, the first patient (62 years) after 28 months had no signs of progression of muscle weakness, and soon after he died of intracerebral hemorrhage ; in the second patient (69 years) we saw a progression of arm weakness and the development of leg weakness after 19 months, after a further 14 months bulbar symptoms appeared and he died 6 years after the onset of the disease. In the third patient (57 years), after 25 months, the weakness spread to the forearm and hand, but did not affect other parts of the body. He can still walk, has no bulbar symptoms. In the patients with symptoms restricted to the upper limbs weakness, the possible diagnoses include classic ALS and FAS. ALS mostly involves distal upper limb muscles, while FAS mostly involves proximal upper limb muscles. To the best of our knowledge, FAS has a better natural history than classic ALS, due to slower spread to other parts of the body. Due to the onset and the progression of muscle weakness, we believe that our patients met the criteria for FAS.

Flair arm syndrome, ALS, neurodegenerative diseases

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