Amyloid neuropathy in a younger domino liver transplanted recipient

Cranial nerve palsies (CNP) are a part of the clinical spectrum of chronic inflammatory demyelinating polyneuropathy (CIDP), but they are commonly associated with a more diffuse pattern. In a cohort of 146 CIDP patients, 2% presented isolated CNP and 17% had CNP associated with limb weakness or sensory involvement. Antiganglioside antibodies can occasionally be detected in the sera of patients with CIDP. In a cohort of 20 patients with CIDP, only 1 had anti-GD1a antibodies. These anti-GD1a antibodies have been identified in cases of acute motor axonal neuropathy (AMAN). We report the case of a 71-year-old woman who presented with recurrent multiple CNP of unknown etiology but with anti-GD1a antibodies. A 58-year-old woman was admitted for left facial weakness. The patient had no personal or familial neurological history and no history of headache. At 57 years of age she experienced right hemifacial pain for several months, which spontaneously regressed. At the age of 58, she developed left Bell’s palsy associated with dysgeusia and left hemitongue anesthesia. She recovered fully with a 15-day course of 80 mg/day of prednisone. At age 61, she developed diplopia for a few days but did not seek medical advice. At age 64, she again developed diplopia with paresis of the left abducens nerve preceded by right hemicranial pain. She had no sequelae after treatment with corticosteroids. At 71 years of age, she progressively developed diplopia and dizziness 2 weeks after influenza vaccination. We observed mild ataxia with moderate hypopallesthesia of the lower limbs, left ptosis, and limitation of abduction and vertical movements of the left eye. Tendon reflexes were all present, and there was no motor deficit or dysautonomia. Periorbital pain appeared the following day, suggesting a left orbital nerve injury. Cerebrospinal fluid analysis showed no evidence of hypercellularity or elevated protein levels. Nerve conduction studies and electromyography were normal. Cerebral magnetic resonance imaging revealed no contrast enhancement of the cranial nerves or lesion of the cavernous sinus. No evidence of a systemic disorder was found. Schirmer’s test was negative, and a minor salivary gland biopsy revealed a grade 3 inflammatory infiltration according to the Chisholm classification. Tests for antiSSa and anti-SSb antibodies were negative. Blood sedimentation rate and glucose were normal, and there was no monoclonal gammopathy. The angiotensin-converting enzyme level was slightly elevated. Radiography of the chest showed no abnormality. We tested antiganglioside antibodies by immunostaining after thin layer chromatography and found anti-GD1a IgG antibody positivity (1:100). Initial steroid therapy at 1 mg/kg/day followed by progressive tapering resulted in regression of symptoms. Four weeks later there was only left ptosis on neurological examination. This is a case of recurrent multiple CNP without peripheral involvement. Extensive follow-up over a period of 14 years did not reveal any associated systemic disease. In view of the relapsing course over more than a decade and the corticosteroid responsiveness, we diagnosed CIDP despite the normal peripheral electrophysiological evaluation. However, the triggering after influenza vaccination, the acute onset of each relapse, and the absence of progression of disability between relapses could argue for recurrent Guillain–Barré syndrome (GBS). Anti-GD1a antibody positivity is compatible with both etiologies. A recent study has identified clinical and paraclinical characteristics that help to distinguish acuteonset CIDP from fluctuating GBS within the first year of the disease course. Nevertheless, there are no available criteria to discriminate recurrent GBS from CIDP in a longer follow-up. Few cases of multiple isolated CNP with antiganglioside antibody positivity have been described. These cases of polyneuritis cranialis associated with antiganglioside antibodies were considered to be variants of GBS, and there was no recurrence of CNP. Studies done on patients with recurrent multiple CNP, published in 1958 and 1970, were considered to be idiopathic cases. It could be hypothesized that some of these cases were inflammatory neuropathies, but the limited paraclinical work-up prevents any definite conclusions from being drawn. VC 2011 Wiley Periodicals, Inc.