Postgrad Med J: first published as 10.1136/pgmj.58.679.311 on 1 May 1982. Downloaded from

Postgraduate Medical Journal (May 1982) 58, 311-313

Painful brachial plexopathy: an unusual presentation of polyarteritis nodosa S. G. ALLAN* H. M. A. TOWLA* M.R.C.P. B. Med. Biol., M.B. C. C. SMITH* A. W. DOWNIE* F.R.C.P. F.R.C.P. J. C. CLARKt M.B., Ch.B. *Department ofMedicine, The Royal Infirmary, Foresterhill, Aberdeen tDepartment ofPathology, University ofAberdeen, Foresterhill

Summary intact save for evidence of a right recurrent laryn- An elderly man was admitted to hospital with an geal palsy on indirect laryngoscopy. There unusual painful bilateral brachial neuropathy, which was a flicker of left biceps contraction and grade 2/5 progressed in spite ofhigh dose corticosteroid therapy. power in the deltoids and adductors of the shoulders, Polyarteritis nodosa, characterized by widespread but otherwise total paralysis of the upper limbs. arteriolar involvement, was shown at post-mortem. Marked tenderness was noted over each brachial copyright. plexus, but there was neither muscle tenderness nor fasciculation. 'Glove' loss of all sensory modalities Introduction was present up to the elbows bilaterally, with hypo- The clinical presentations of polyarteritis nodosa tonicity and areflexia. There was minimal hip flexor are classically diverse, reflecting variable vascular weakness and absent left knee and both ankle involvement of many organs and tissues. Mono- reflexes without sensory abnormality or calf tender- multiplex is the most common neurological ness. Bladder and bowel functions were unaffected sequel (Ford and Siekert, 1965), but asymmetrical and examination otherwise unremarkable. His blood http://pmj.bmj.com/ , predominantly involving the lower pressure was 150/90 mmHg, and fundi normal. No limbs, are well recognized. Central nervous system skin lesions were apparent. involvement is described, but unusual. The syn- Investigations: The ESR was 30 mm in the first drome of a progressive, painful, bilateral brachial hour; Hb 14-1 g/dl; WBC 13 8x109/l (2% eosino- neuropathy with paralysed upper limbs, and hoarse- phils); repeated urinalyses proved negative; urea ness due to recurrent laryngeal nerve involvement is 12-0 unusual and reported now. mmol/l, creatinine 86 ,umol/l, with normal electrolytes; bilirubin 11 Fmol/l, alkaline phos- on September 26, 2021 by guest. Protected phatase 147 u./l, y-glutamyl transpeptidase 270 i.u./l. Case report Anti-nuclear factor, anti-smooth muscle antibody A 78-year-old retired farm worker was admitted and rheumatoid factor were negative, as were the with a one week history of progressive bilateral arm VDRL and TPHA in blood and cerebro-spinal fluid. weakness, numbness below the elbows and pain in (CSF) which was clear and under normal pressure, his shoulder muscles. He had felt generally unwell without a pleocytosis or increased protein concen- for 6 weeks and had noticed discomfort in his neck tration. His initial chest X-ray was normal, but films and several ribs. For 2 days he had been hoarse, but of cervical spine showed disc space narrowing at the denied symptoms of recent intercurrent C3/4 and C4/5 levels. Cervical myelography was although he had been breathless and wheezy for 10 normal. An isotope bone scan revealed several areas months. He had not smoked for many years, and had of increased uptake throughout the ribs. Hb5Ag was received only bronchodilator and diuretic therapy. not detected by radio-immunoassay. Nerve conduc- On examination he was lucid, apyrexial and tion studies showed an absence of nerve action hoarse. His arms dangled helplessly by his sides, but potentials from the median at wrist and he was able to walk unaided. His cranial nerves were elbow, with diminished amplitude in the ulnar nerves 0032-5473/82/0500-0311 $02.00 ©) 1982 The Fellowship of Postgraduate Medicine Postgrad Med J: first published as 10.1136/pgmj.58.679.311 on 1 May 1982. Downloaded from

312 Clinical reports

and slowing of conduction. was to 50 % of patients with polyarteritis nodosa (Goetz, unremarkable. Re-examination one month later 1980), often appearing early in the course of the ill- showed profuse fibrillation in the forearm muscles ness and dominating the clinical picture. Neuro- with complete inexcitability of ulnar and median logical symptoms and signs may indeed provide the nerves bilaterally. major clinical evidence for the eventual diagnosis. A diagnosis of atypical polymyalgia rheumatica In one series of 114 patients with proved polyar- had been made by his family doctor before admission teritis nodosa (Ford and Siekert, 1965), 68% had a and therapy commenced with prednisolone 40 mg , of which group 58% had daily. This dose was increased to 80 mg daily follow- mononeuritis multiplex, 16% a and ing admission to hospital. In spite of this, his condi- 26% a mixed pattern. Symmetrical polyneuropathy tion progressively deteriorated with increasing predominantly affecting the lower limbs, most paralysis and sensory loss in the upper limbs and the marked distally and associated with muscle pain and development of progressive weakness in the lower tenderness is well described (Goetz, 1980). Brachial limbs, initially proximal but spreading distally. and lumbo-sacral plexopathies have been reported, Breathing and swallowing difficulties arose, necessi- but are so rare (Goetz, 1980) that it seemed worth- tating tracheostomy and intra-gastric feeding. while describing a further case. Repeated chest radiography showed variable pneu- This patient's progressive symmetrical bilateral monic shadowing, predominantly in the lower zones, brachial plexopathy, with involvement of the right unresponsive to antimicrobial therapy. Transient recurrent largyneal nerve, raised the possibility of an visual loss in his left eye occurred and was thought atypical Guillain-Barre syndrome which may present to be ischaemic or embolic in origin. He complained with upper limb motor involvement and loss of of increasingly severe pain in his thoracic cage in deep touch, vibration and proprioception before spite of analgesics and, as his bone scan was sugges- spreading to involve laryngeal, palatal and pharyn- tive of metastatic disease, the unusual neuropathy geal functions or, later, the lower limbs. The course was regarded as carcinomatous, predominantly of this patient's illness and his normal CSF bio- the upper limbs and recurrent chemistry were against this diagnosis. A pure affecting right laryn- copyright. geal nerve, but with increasing involvement of the ascending motor polyneuropathy, progressing to lower limbs. Corticosteroid therapy was withdrawn respiratory and swallowing difficulties, with pre- and he died under sedation of a bronchopneumonia. sumed but mistaken diagnosis of Guillain-Barre Post-mortem revealed widespread arteritis pre- syndrome, has been described with polyarteritis dominantly affecting medium and small arteries and nodosa (Goetz, 1980) but is a rare presentation. arterioles. The lesions varied from those that were Sensorimotor polyneuropathy is one of the recog- chronic in a healing phase to others that were acute, nized carcinomatous neuropathies and may be

with fibrinoid necrosis of the media, polymorph infil- indistinguishable from the Guillain-Barre syndrome, http://pmj.bmj.com/ tration and intraluminal thrombus formation. The save that sensory symptoms usually dominate the lungs were conspicuously involved, showing exten- clinical picture (Bruyn, 1979). In view of this sive areas of necrosis with superadded broncho- patient's radio-isotope bone scan report, and faced pneumonia, and associated venous involvement. with progressive clinical deterioration in spite of Granulomata were not present in the broncho- adequate corticosteroid therapy, the authors re- pulmonary tree or elsewhere. The , garded carcinomatous polyneuropathy as a likely median, ulnar, sciatic and vagus nerves bilaterally explanation of the picture and withdrew therapy. In and the right recurrent laryngeal nerve showed retrospect, his broncho-pulmonary symptoms and on September 26, 2021 by guest. Protected arteritis of the epineurial vessels. Several posterior radiographic signs, together with the painful root ganglia were involved but the central nervous peripheral neuropathic involvement and retinal system was unaffected. Scattered arteritic lesions ischaemic episode were attributable to the inexorable were present in the kidneys, gut, spleen and adrenals. progress of his polyarteritis nodosa. There was pronounced fatty change in the liver with The post-mortem findings of severe pulmonary large numbers of polymorphs within the sinusoids, involvement raise the possibility of Wegener's consistent with a bacteraemia, but no arteritic granulomatosis, but the absence of respiratory tract lesions were identified. The appearances were granulomata and minimal renal involvement with- regarded as typical of polyarteritis nodosa. The out granuloma formation is against this diagnosis. bony lesions seen on isotope scanning represented Attempts have been made artificially to distinguish fibrous dysplasia. No evidence of primary or 'classic' polyarteritis nodosa, in which there is spar- metastatic tumour was found. ing of the lungs, from allergic angiitis granulomatosis where broncho-pulmonary involvement with pre- Discussion ceding asthma or bronchitis and a blood eosino- Peripheral neuropathic involvement occurs in up philia dominate the picture and granulomata are Postgrad Med J: first published as 10.1136/pgmj.58.679.311 on 1 May 1982. Downloaded from

Clinical reports 313 found in relation to the arteritic lesions (Austen, biopsy should be considered at an early stage in the 1979; Churg and Strauss, 1951). This patient had investigation of the patient. intermittent wheeze and breathlessness for some months before admission, but the absence of a blood References with the AUSTEN, K.F. (1979) CECIL Textbook of Medicine 15th edn, eosinophilia, together demonstration of p. 180. Beeson, McDermott and Wyngaarden; W.B. Saun- widespread typical polyarteric lesions at post- ders Co., Philadelphia, London, Toronto. mortem, particularly in the lungs, highlights the BRUYN, G.W. (1979) Carcinomatous polyneuropathy. In: difficulty in attempting artificially to differentiate Handbook of Clinical , Vol. 38, p. 679. (Ed. by in this rare and Vinken, P.J. & Bruyn, G.W.) Elsevier, Amsterdam. ends of a spectrum confusing disease. CHURG, J. & STRAUSS, L. (1951) Allergic granulomatosis, The unpredictable response of polyarteritis nodosa Allergic angiitis and peri-arteritis nodosa. American to corticosteroid therapy and its occurrence without Journal of Pathology, 27, 277. obvious precipitant in the elderly is re-emphasized. FORD, R.G. & SIEKERT, R.G. (1965) Central nervous system The diagnosis should be manifestations of peri-arteritis nodosa. Neurology, 15, 114. suspected where atypical GOETZ, C.G. (1980) Polyarteritis nodosa. In: Handbook of neurological syndromes arise in the absence of Clinical Neurology, Vol. 39. (Ed. by Vinken, P.J. & tumour or other obvious association and nerve Bruyn, G.W.) p. 295. Elsevier, Amsterdam. copyright. http://pmj.bmj.com/ on September 26, 2021 by guest. Protected