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Show ORIGINAL CONTRIBUTION Isolated Unilateral Adduction Deficit and Ptosis as the Presenting Features of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Christina Pieh, Beatrice Rossillion, Anne C. Heritier- B arras, Michel Chofflon, Theodor Landis, and Avinoam B. Safran A patient with chronic inflammatory demyelinating polyneuropathy ( CIDP) presented with an isolated unilateral adduction deficit and ptosis. Investigations were negative until the onset of limb weakness and fatigue 2 years later. At that time, electroneuromyography, cerebrospinal fluid examination, and magnetic resonance imaging confirmed the diagnosis of CIDP. Thus, ophthalmic signs can precede extremity and bulbar signs with a long latency in CIDP. ( JNeuro- Ophthalmol 2002; 22: 92- 94) Chronic inflammatory demyelinating polyneuropathy ( CIDP) is a symmetric polyradiculoneuropathy that affects motor and sensory fibers of proximal and distal limbs. Unlike the acute onset characteristic of Guillain- Barre syndrome and its Miller- Fisher variant, the course of CIDP may evolve over weeks to years. Typical manifestations are weakness, reduced sensation, and paresthesias. Recovery is usually incomplete even years later. Predominant involvement of ocular motor nerves is rare ( 1- 5). We report a case of CIDP that initially presented with an isolated ptosis and adduction deficit. Two years later, more characteristic clinical features appeared. CASE REPORT A 3 5- year- old man experienced sudden onset of horizontal diplopia in left gaze and ptosis of the OD. A history of infectious mononucleosis was present. Ophthalmologic examination confirmed the right upper lid ptosis and disclosed a nearly complete adduction deficit of the OD. There were no other abnormalities. Over the next 2 years, multiple neurologic examinations failed to disclose any additional abnormalities. Cranial magnetic resonance imaging ( MRI) Ophthalmology Clinic ( CP, BR, ABS) and Neurology Clinic ( ACH- B, MC, TL), Department of Clinical Neurosciences, Geneva University Hospitals, Switzerland. Address correspondence to Avinoam B. Safran, Ophthalmology Clinic, Department of Neurosciences, Geneva University Hospitals, 22, rue Alcide Jentzer, 1211 Geneve 14, Switzerland and magnetic resonance angiography were unremarkable, as were cerebrospinal examination, serum protein electro- FIC. 1. A: Axial T1 enhanced magnetic resonance imaging ( MRI) shows enhanced and thickened third cranial nerves as they exit the midbrain ( arrows). B: Coronal T1 enhanced MRI shows enhanced third division of both fifth cranial nerves ( arrows). ^ 92 . Dpi: 10.1Q97/ Ql. WNO. QQ0QP18222.83526, F2. . J Neuro- Ophthalmol. Vol. 22, No. 2,. 2Q02 , Copyright © Lippincott Williams & Wifkins. Unauthorized reproduction oftnis article is prohibited. PlEH ETAL. JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 phoresis, Immunoelectrophoresis, and several standard batteries of chemistries and hematologic tests. At onset of general fatigue and limb weakness, the patient was referred to our clinic. Visual acuity was 20/ 20 in both eyes. Biomicroscopy of the anterior segment, fundus and automated visual field examinations, electroretinogra-phy, and pattern visually evoked potentials were normal. The pupils showed equal size in dim illumination, brisk symmetric light reactions, and no afferent pupillary defect. OD ptosis of 3 mm was noted. He had an exotropia of more than 40 prism diopters in left gaze due to a nearly complete deficit of adduction of the OD. Neurological examination showed normal muscle tone and strength, diminished deep tendon reflexes, and diminished vibratory sensation of the lower limbs. Pinprick sensation in the region of the first trigeminal nerve division was diminished bilaterally, but the patient did not complain about facial numbness. Hematochemical routine parameters, serum protein electrophoresis, and immunoelectrophoresis were again normal. Epstein- Barr virus ( EBV) IgG antibodies were positive. Antiganglioside antibodies ( GMlb IgM, GQlb IgG, GDlb), antimyelin- associated glycoprotein, and acetylcholine receptor antibodies were negative. MRI demonstrated bilateral enhancement of cranial nerves III ( Fig. \ A) and V ( Fig. IB). CSF showed a cytoalbuminologic dissociation with 0 red blood cells, 7 white cells per mm3 ( 92% lymphocytes, 8% monocytes, rare plasma cells), and a protein of 90 mg/ dL. Electroneuromyography showed reduced conduction velocity and desynchronization of potentials with bilateral and symmetrical sensory and motor involvement. These results led to a diagnosis of CIDP. After treatment with oral methylprednisolone ( 1 mg/ kg/ day) and five daily infusions of high- dose intravenous human immunoglobulin ( 0.4 g/ kg), ptosis and limb weakness improved, but the adduction deficit was unchanged ( Fig. 2). DISCUSSION In this patient who fulfilled the diagnostic criteria of CIDP( 6), 2 years elapsed between the appearance of ophthalmic and extremity manifestations. Ptosis and ophthalmoplegia have generally not preceded extremity signs and symptoms in CIDP by such a long interval ( 1- 4). In a case similar to ours, transient unilateral ptosis occurred before diplopia, and upper and lower limb muscle weakness developed 6 years later ( 5). However, in contrast to our patient, the CSF protein level was already elevated at the onset of ophthalmic manifestations. When ocular motor manifestations occur in CIDP, they are usually symmetric ( 5). Interestingly, the adduction deficit was strictly unilateral in our patient, whereas MRI enhancement of the third and fifth cranial nerves was bilateral. Enhancement of cranial nerves on MRI, which has only rarely has been described in CIDP ( 5), evidently need not cause clinical manifestations. The role of infectious mononucleosis in the patient's history is unclear. The influence of lifelong persistence of EBV on the development of an autoimmune disorder is known ( 7). IgG anti- GQlb gangliosides, which play a role in the pathogenesis of ophthalmoplegia in Guillain- Barre syndrome with ophthalmoplegia and in the Miller- Fisher syndrome ( 8), were absent in our patient, which showed that other antibodies may be involved in ocular motor palsies. Also, anti- GMl and anti- GDlb antibodies, whichhave been present in other cases of CIDP with ocular involvement ( 1), were absent. Antimyelin- associated glycoprotein antibodies, which are described primarily in sensory polyneuropathy, were also negative. Cellular or humoral sensi- FIC. 2. After treatment with methylprednisolone and 5 days of high- dose intravenous immunoglobulin, a large OD adduction deficit persists. 93 Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited JNeuro- Ophthalmol, Vol. 22, No. 2, 2002 CHRONIC INFLAMMATORY DEMYELINATING POLYRADICULONEUROPATHY tivity to a myelin antigen was not evident in our case, as none of the antibodies tested was elevated. The partial recovery after intravenous human immunoglobulin and corticosteroid treatment suggests a role of humoral mediators but does not clarify their nature. REFERENCES 1. Serratrice G, Azulay JP, Pouget J, et al. Ptosis et ophtalmoplegies variables et recidivants au cours des polyradiculonevrites chro-niques. Rev Neurol 1997; 153: 197- 200. 2. Barohn RJ, Kissel JT, Warmolts JR, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: a study of proposed elec-trodiagnostic and histologic criteria. Arch Neurol 1989; 46: 878- 84. 3. McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and electrophysiological study of 92 cases. Brain 1987; 110: 1617- 30. 4. Maisonobe T, Chassande B, Verin M, et al. Chronic dysimmune demyelinating polyneuropathy: a clinical and electrophysiological study of 93 patients. J Neurol Neurosurg Psychiatry 1996; 61: 36- 42. 5. RottaFT, Sussman AT, Bradley WG, etal. The spectrum of chronic inflammatory demyelinating polyneuropathy. J Neurol Sci 2000; 173: 129- 39. 6. Ad Hoc Subcommitee of the American Academy of Neurology AIDS Task Force. Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy ( CIDP). Neurology 1991; 41: 617- 8. 7. Wandinger K, Jabs W, Siekhaus A, et al. Association between clinical disease activity and Epstein- Barr virus reactivation in MS. Neurology 2000; 55: 178- 84. 8. Chiba A, Kusunoki S, Obata H, et al. Serum anti- GQlb IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain- Barre syndrome: clinical and immunohistochemical studies. Neurology 1993; 43: 1911- 7. „ 94 . „ „ . , „ . . © 2002 Lippincott Williams & WUkins , Copyright © Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited. |
