P29.7 Phrenic neuropathy in systemic lupus erythematosus

P29.7 Phrenic neuropathy in systemic lupus erythematosus

S246 Posters / Clinical Neurophysiology 117 (2006) S121–S336 not regressed. She will be treated with ciclofosfamide in the future. Conclusion: NT ar...

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S246

Posters / Clinical Neurophysiology 117 (2006) S121–S336

not regressed. She will be treated with ciclofosfamide in the future. Conclusion: NT are essential in the diagnosis and follow up of the mononeuritis multiplex. We emphasize that hepatitis C should be included in causes of mononeuritis multiplex, and not always associated with crioglobulinaemia. doi:10.1016/j.clinph.2006.06.484

P29.7 Phrenic neuropathy in systemic lupus erythematosus P.M. Roos Ullevaal University Hospital, Department of Clinical Neurophysiology, Norway Background: Diaphragmatic weakness has been noted in patients with systemic lupus erythematosus (SLE). Aim: To investigate the cause of respiratory weakness in a SLE case. The origin of this weakness is unknown. Neuropathic and myopathic causes have been discussed. The shrinking lung syndrome (SLS) with elevated hemidiaphragm on chest X-rays is a rare respiratory manifestation of SLE. We present a lupus case where sudden respiratory arrest was diagnosed as SLS. Methods/Results: Nerve conduction studies of the phrenic nerve disclosed severely reduced motor amplitudes bilaterally. Artificial ventilation proved life saving but weaning off the ventilator was not possible. Concentric EMG of the diaphragm showed fibrillation potentials and positive sharp waves. An axonal phrenic neuropathy could be suspected to cause SLS in this SLE case. The patient slowly recovered on a cyclophosphamide plus prednisolone regimen. Conclusion: Phrenic electrophysiologic studies may be considered in lupus cases with respiratory problems. doi:10.1016/j.clinph.2006.06.485

P29.8 Study of the course of the Guillain–Barre-Syndrome on the basis of a dynamographic functional analysis C. Mueller, P. Baum, D. Boeckler, A. Wagner Department of Neurology, University, Germany Objective: Studies of the course of the Guillain–BarreSyndrome (GBS) are essential to evaluate the prognosis as well as effectivity of medical treatment and physical therapy. This study analyses how the significance of such an evaluation may be increased by gathering paretic data based on a dynamographic functional analysis. Method: With a system for dynamography (Biodex, USA) the isometric maximum strength (Fmax) of the dorsiflexion and plantarflexion at the ankle was measured six times (week 0, 1, 4, 13, 26 and 52) at 18 GBS patients (42 y +/ 14, 8 female, 10 male). The data was then com-

pared to gender and age specific normal values as well as initial values. Results: Overall, Fmax changed from severe (14.9% dorsiflexion and 34.7% plantarflexion, respectively) to light constraints (88.1% and 80.4%, respectively) (each relating to the normal value = 100%) after 52 weeks. The latter does not differ significantly from the normal value. In terms of the initial values a significant dysbalance is to be noticed to the disadvantage of the dorsiflexion. Regarding the course of disease, there appear to be different types. Group 1, complete remission of both directions of movement (n = 7); group 2, divergent recovery with a remaining constraint of the dorsiflexion (n = 8); group 3, incomplete remission of both directions of movement (n = 3). An incomplete remission exists if there is either no significant dysbalance after 13 weeks or if both directions of movement do not show a significant difference compared to initial values. Risk factors for this group are high age, severe cases (e.g., compulsory ventilation) as well as concomitant diseases (e.g., diabetes m.). There is no influence by the ganglioside-ab, the evidence of campylobacter j. and cytomegalovirus as well as the form of therapy (IVIG vs. Plasmapheresis). Conclusion: By using dynamography it was possible to not only improve the evaluation of prognosis, but it also showed risk factors that influence the course of GBS. doi:10.1016/j.clinph.2006.06.486

P29.9 Evolving pattern of Guilliane–Barre syndrome in Israel M. Kushnir 1, C. Klein 1, L. Polak 1, J.M. Rabey 2 1 2

Assaf Harofeh Medical Center, Neurology, Italy Assaf Harofeh Medical Center, Israel

Background: Acute axonal polyradiculopathy is one of the subtypes of Guillain–Barre syndrome (GBS) not frequent observed in Western countries. In contrast, in China and Japan this form is more prevalent than demyelinating disease. Objective: To investigate the frequency of axonal GBS in Israel patients. Methods: Clinical and electrophysiological finding of 40 patients admitted between 1999–2005, with abnormalities compatible with AMAN (acute motor axonal neuropathy), AMSAN (acute motor sensory axonal neuropathy) or AIDP (acute inflammatory demyelinating neuropathy) were reviewed. Results: Electrophysiological finding showed that 25 (62.5%) patients had AIDP, 9 (22.5%) AMAN, 6 (15%) AMSAN. There were significant differences in severity of disease. Most patients (87%) with axonal type were hospitalized with moderate or severe symptoms (3–4 Hughes grade score) and progressed to severe grade (4–6) in comparison with AIDP patients, in whom 64% were hospital-