Bilateral brachial neuropathy and acute aortic dissection due to giant cell arteritis

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Sirs, A previously well 59-year-old male presented to the neurology services with progressive bilateral proximal upper limb weakness and bilateral shoulder pain. Weakness and pain started on the right-side 2 weeks prior to presentation and developed on the left-side 1 week later. Erythrocyte sedimentation rate (ESR) performed by the general practitioner at symptom onset was normal (12 mm/h). Muscle strength testing revealed asymmetrical (left [ right) bilateral proximal upper limb weakness; left shoulder abduction (MRC grade 3), right shoulder abduction (grade 4), left elbow flexion (grade 2), right elbow flexion (grade 3), left elbow extension (grade 3), right elbow extension (grade 4). Biceps and brachioradialis reflexes were absent bilaterally. Pin prick sensation was reduced at both shoulder tips. Lower limb examination was normal. Nerve conduction studies demonstrated bilateral reduced radial and median sensory action potentials. Ulnar and median motor action potentials were normal. Electromyography showed fibrillation potentials in biceps and deltoid muscles bilaterally, consistent with acute denervation. Lower limb electrophysiology was normal. These findings implicated a postganglionic process and were most consistent with bilateral brachial neuropathy. Abnormal blood tests included elevated ESR (63 mm/h) and reduced haemoglobin (10 g/dL). Autoantibody titres, including anti-neuronal antibodies, were normal. HIV antigen, VDRL and lyme serology were negative. MRI of cervical spine and brachial plexuses, and CT of thorax, abdomen and pelvis were normal. Lumbar puncture revealed normal routine cerebrospinal fluid constituents. ESR remained persistently elevated, peaking at 102 mm/h. One week following admission severe retrosternal and epigastric pain radiating to the interscapular region developed. Repeat CT thorax and abdomen revealed a new dissection of the descending and abdominal aorta, distal to the origin of the left subclavian artery. The cardiothoracic team managed this conservatively. Five days later a dull right sided headache developed with associated tenderness of the right temporal artery. A right temporal artery biopsy was performed, demonstrating necrosis (Fig. 1a), disruption of internal elastic lamina (Fig. 1b), panarteritis (Fig. 1c) and multinucleated giant cells (Fig. 1d), consistent with active giant cell arteritis. The patient had an excellent response to oral prednisolone (60 mg daily). At 6 weeks, power had fully recovered, headache resolved and ESR normalised (23 mm/h). Prednisolone dose was subsequently tapered to 10 mg daily and his neurological and cardiovascular status remained stable. This patient had bilateral brachial neuropathy and AAD due to GCA. Eleven cases of GCA-related brachial neuropathy have been previously reported (bilateral in 4) [1–9]. Unlike this case, fever, malaise, diaphoresis and/or fatigue (constitutional symptoms) were present in all cases at presentation. Apart from these constitutional symptoms, brachial neuropathy symptoms were the initial manifestation of GCA in each case. In all reported cases, patients were over 60-years-old and presentation ESR was elevated. In ten cases clinical recovery occurred with prednisolone therapy, and, as in this patient, response to therapy S. Saidha (&) T. H. Mok M. Butler H. Harrington Department of Neurology, Mercy University Hospital, Cork, Ireland e-mail: shivsaidha@hotmail.com; shivsaidha@physicians.i.e