| OCR Text |
Show Ii) 1986 Raven Press, New York Chronic Inflammatory Polyradiculoneuropathy with Ophthalmoplegia Antonia C. Chalmers, LCDR., M.e, U.S.N.R., and Robert G. Miller, M.D. We report a case of chronic progressive inflammatory polyradiculoneuropathy with a subacute exacerbation that included ataxia, tendon areflexia, ptosis, and ophthalmoplegia. Spinal fluid protein was elevated and electrophysiological studies revealed a demyelinating neuropathy. This case suggests a continuum between chronic inflammatory polyradiculoneuropathy and the Miller Fisher variant of acute inflammatory polyneuropathy. From the Division of Neurology, Naval Hospital. Oakland. California (A.ee). and the Departments of Neurology, Children's Hospital and the University of California, San Francisco, California (R.C.M.). /66 The association of ophthalmoplegia, ataxia, and tendon areflexia as a variant of acute inflammatory polyneuropathy is well known (Miller Fisher syndrome) (1-3). We wish to report a patient who developed this syndrome as part of chronic progressive inflammatory polyradiculoneuropathy, an association that has not been described previously. CASE REPORT A 59-year-old male presented in August 1982 with a 3-year history of numbness and tingling of both feet and a I-year history of proximal upper extremity weakness. A right middle lung mass was explored with thoracotomy and benign pleural thickening was found. In December 1983, he complained of burning feet and tightness in his legs. Shoulder girdle weakness again was noted, and tendon reflexes were hypoactive. Electromyography showed moderate numbers of positive sharp waves and fibrillations in proximal and distal muscle groups of the right upper and lower extremities. In March 1984, without any history of antecedent illness, he noted sudden progression. He began having difficulty using his hands; his walking became unsteady and he developed impotence. In April 1984, his cerebrospinal fluid contained no cells, but his protein was 177 mg/dl. A sural nerve biopsy disclosed no definite abnormality, but insufficient tissue was obtained. In mid-May, the patient developed intermittent diplopia in all directions of gaze. One month later, the diplopia became persistent and fluctuating ptosis was noted. Ptosis became prominent and weakness of the upper extremities progressed; by end of June, the patient could not open doors or turn keys and writing became impossible. His walking became more unsteady and he had to hold the walls to ambulate. POLYRADICULONEUROPATHY /67 His only other medical problt'm was hypertension, diagnosed in 1977. His blood pressure was stable until May 1984, when it became labile and difficult to control. Medications were furost'mide and hydralazine. On examination in July 1984, ptosis was complete on the left and partial on the right. On voluntar~' lid elevation, his left lid did not move, while the right t'levated minimally. Thert' was complete extt'rnal ophthalmoplegia, except for a slight upward e'cursion of the Idt eye. Bell's phenomenon was abst'nt and head turning did not induce eye movement. Pupils were normal and there was no f.Kial paresis. There was diffuse weakness of both upper extremities involving the proximal and distal muscle groups equally. There was minimal weakness of both lower extremities. Sensory examination revealed a glove-andstocking moderate sensory loss of sensitivity to pinprick and temperature'. There was profo~nd loss of both vibration and joint position senses in the lower extremities and a sensory gait ataxia. Tendon reflexes were absent and there was a mild left-arm tremor. Clinical laboratory tests revealed an elevated sedimentation rate of 57 mm/h. Serum creatinine kinase was elevated at 3,041 U (20-220 LU.). Normal tests included the following: complete blood count; metabolic panel, including fasting blood sugar; urine porphobilinogen; serum and urine protein electrophoresis; serum vitamin B)2 and folate; thyroid profile; acetylcholine receptor antibody; serum lipoprotein electrophoresis; serum and urine levels of lead, mercury, and arsenic; ANA; RH screen; and RPR. Muscle biOpsy of the deltoid showed neurogenic atrophy. Electrodiagnostic studies revealed markedly prolonged distal motor and F response latencies. Electromyography showed a moderate number of fibrillations and positive sharp waves with decreased recruitment patterns. Results of repetitive stimulation of the left ulnar, spinal accessory, and facial nerves were normal. Brainstem auditory evoked potentials were normal. Treatment was started on July 20, 1984, with prednisone 80 mg daily. After 8 weeks of treatment, there was nearly complete resolution of ophthalmoplegia and marked improvement in upper extremity strength. The patient continues to improve on tapering doses of prednisone, although lower extremity sensory symptoms persist. DISCUSSION Extraocular muscle involvement in chronic in-f1ammatory polyradiculoneuropathy is rare (1-4). In Dyck et al.'s series of 53 cases of chronic inflammatory polyradiculoneuropathy, only four reported diplopia and none had clinical ophthalmoparesis (2,3). Similarly, Dalakas and Engel reviewed 25 cases of chronic relapsing demyelinating polyneuropathy and found two with oculomotor disturbance, but details were not provided (1). On the other hand, acute ophthalmoparesis, with ataxia and areflexia (Miller Fisher syndrome) as a variant of acute inflammatory pOlyneuritis, is well known (5-8). In typical cases of Miller Fisher syndrome, signs develop rapidly (within days) and complete, spontaneous recovery is the rule. Past and recent reports document relapsing forms of Miller Fisher syndrome (5,7-9) as well as longer intervals between the onset of ophthalmoparesis and the development of limb symptoms (10). This patient had a chronic progressive demyelinating neuropathy for 2 years before the subacute development of ataxia, areflexia, and ophthalmoplegia. His clinical course represents a transition between chronic demyelinating neuropathy and Miller Fisher syndrome, two entities which hitherto have been considered quite distinct. Virtually identical pathological and electrophysiological findings have been reported in chronic inflammatory pOlyneuropathy and acute inflammatory polyneuropathy (1-4,11), and cases in which acute inflammatory polyneuropathy and Miller Fisher syndrome overlap have been described (12,13). Thus, in spite of different clinical features in chronic inflammatory polyneuropathy and Miller Fisher syndrome, our patient's situation raises the possibility of related pathophysiologic mechanisms in these two conditions. REFERENCES \. Dalakas. MC, and Engel. WK: Chwnic relapsing (dvsimmune) polyneuropathy: pathogenesis and treatment. All/I NCllro/1981;9(suppl):134-145. 2. Dyck. P). Lais. AC, Ohta. M. Bastron. IA. Okazaki. H. and Gwover. RV: Chwnic intlammatory pl>lvneuropathv. Maw C/ill Prot' 1975;50:621-b37. 3. Dyck. P). Thomas. PK. Lamb.'rt. EH. and Bunge. R: Pcril'ht'rlll N"IIY1'I'"lhy. \',,1 2. Philadelphia: WB Saunders. 19!14:2101-2114. 4. Prim·as. JW. and McLt'od, JG: Chronic relapsing polym'uritis. I Nellr,,/ S.-i 1976;27:427-45/1. 5. Elizan, TS. Spire. )P. Andiman. RM. Baughman, FA. and Lloyd-Smith. DL: Syndrome of acute idiopathic ophthalmoplegia with ,1t,lXi,1 and aretlexia. Nellrology 1971;21:281292. 6. Fisher. M: An unusual variant of acute idiopathic polyneuritis (syndroml' of ophthalmoplegia. ataxia and areflexia). N Ellgi , Med 1956;255:57-65. 7. Van Allen. MW. ,md MacQueen. jC: Ophthalmoplegia. J Clill N.'lIw-cJl'1Jt'wllllO/. Vol. 6. No.3. 1986 /68 II. C. CHALMERS AND R. G. MILLER ,1t,l\i,1 'llld till' svndnlllll' ot L'llIndry-ClIilldin B,HH'. {mils 1\/11 N,,",.,J/ 1\';""" I'lM;Hl):l)H- 1lI.1. H. Cibbl'rd, I'll: l )phthdlJ11opll'gi.l In dClItl' polvll"lIritis. 11rth N,'II""/ IlJ7();~.llhl-lh4. l). K.lpl.lll, IC, SdldllJ11bllrg, 1111, dnd SlIJ11Il11'r, i\: Rl'I.lpsing ophthd InlllpiL-gi.l-sl'nSt Jrv lH'lIl"llpd th v svnd n JIlIL'. N"II ""/".\.1/ Il)H'i;.l'i:'illC;-C;l)h. Ill. Don,lghv, M, ,HId Folll CI: lkllldr pdlsy pn'n'ding duonil n'l,lpsing polVlH'lIropdthv bv St'vl'rdl Wl'l'ks. /\/111 N<'III'lJ/ I'lH'i; 17:4l)- 'ill. I elJII Nt'JI,o-0l'hllwllJlol. Vol. b, No . .J, J9/ib II. Miller, RG: The Guillain-Barre syndrome. Current methods of diagnosis and treatment. Postxrad Med 1985;15:62-64. 12. Phillips, MS, Stewart, S, and Anderson, JR: Neuropathological findings in Miller Fisher syndrome. / Neurol Neuro,," rg Psychiatry 1984;87:492-495. 1.1. Sduron, !:l, !:louchl', P, Calhala, HP, Chain, F, and Castdigne, P: Miller Fisher syndrome: clinical and electrophysiologic evidl'nce of peripheral origin in 10 cases. Neurology 14H4;.14:4'i.1-456. |
