Parvovirus B19 infection antedating Guillain–Barre’ syndrome variant with prominent facial diplegia

Abstract

The clinical spectrum of Guillain–Barre syndrome (GBS) has expanded due to variants accounting for benign forms without progression [1–4]. Patients with acute onset of facial diplegia, with or without limb weakness, acral paraesthesias, or areflexia are affected by a regional GBS variant ‘‘facial diplegia and paraesthesias’’ (FDP) [1, 3]. This 36 year old man, 3 weeks after high fever with myalgias, experienced facial pain, difficulty in closing eyes, and pursuing lips. Taste, lacrimation, swallowing, and hearing were normal. On admission (day 2), neurological examination showed facial diplegia and areflexia. Eye movements, strength, coordination, and sensibility were unaffected. By day 4, the patient reported hand numbness. Normal laboratory tests included screening for autoimmune-rheumatic diseases. Virological, microbiological studies excluded a variety of infections (Borrelia burgdorferi, HSV-1/2, VZV, EBV, CMV, HHV-6). Serum IgG and IgM antibodies to gangliosides GM1, GM2, GM1b, GD1a, GD1b, GalNAc-GD1a, GT1a, and GQ1b were investigated (day 3) by enzyme-linked immunosorbent assay (ELISA) as described elsewhere [2, 3]. The antiganglioside antibody assays were negative. CSF (day 5) showed increased protein content (68 mg/dl, normal \ 45). ELISA revealed anti HPV-B19 IgM and IgG (titer above 5.99). Real Time PCR detected a viral load of 28,794 copies/ml. N-PCR showed HPV-B19 DNA in CSF. Electrophysiological study (day 8) showed temporally dispersed peroneal, tibial compound muscle action potentials (1.5–2 mV in amplitude, normal [ 4), border-line slowing in motor conduction (40 m/sec normal [ 45), and delayed peroneal, tibial F-waves (65 ms, normal \ 58). There were proximal conduction blocks in median and ulnar nerves [5]. Sensory fibers were spared. Brain MRI, auditory, and brain stem evoked responses were normal. Intravenous immunoglobulin was started on day 5 (IVIg 0.4 g/kg/daily) and completed over the next five days. By 3 weeks, bifacial weakness and distal numbness had recovered; on day 122, there were neither residual clinical nor electrophysiological abnormalities. ELISA (day 127) showed anti B19 IgM titer lowered to 0.79, whereas IgG remained 5.99 (Biotrin international ltd, Dublin, Ireland).Our patient presented with a post-infectious FDP variant [3]. Major features were albumino-cytologic dissociation and presence of HPV-B19 DNA in serum and CSF during the acute phase. Susuki et al. [3] reported 22 cases with a disease compatible with Ropper’s original description [1]; among those patients, marginal cases were identified without limb paraesthesias or areflexia [3]. Infection serologies during the acute phase evidenced anti-cytomegalovirus, Campylobacter jejuni, Epstein-Barr, Borrelia burgdorferi, and Mycoplasma pneumoniae IgM antibodies in 14 subjects; 13 experienced upper respiratory tract infection. Anti-ganglioside IgM antibodies were detected in five patients. Motor conduction study suggested demyelination in 14 cases and was normal in 7 [3]. HPV-B19 causes erythema infectiosum, aplastic crisis, fetal hydrops, arthritis, vasculitis, and neurological syndromes [6–9]. Skin rashes or arthralgias associated with positive rheumatoid factor and antinuclear antibodies F. Barbi A. Ariatti G. Galassi (&) Department of Neurosciences, University of Modena and Reggio Emilia, Via P. Giardini, 1350, 41010 Modena, Italy e-mail: giulianagalassi@aliceposta.it