Poster Abstracts Wednesday, November 9, 2005 $331

He was a known hypertensive on irregular treatment and follow-up. He did not adhere to a proper physiotherapy regimen and was left with a spastic left hemiparesis. There was no clinical evidence of myasthenia or neuropathy. The patient was not on anffnoglycosides and there was no other known contraindication to botulinmn toxin injection. Methods: A detailed clinical assessment was done and the possible benefits of botulinum toxin therapy were discussed with him and the family members. After their consent, it was decided to inject botulinum toxin into the spastic left upper limb alone due to finandal constraints. Doses administered into each muscle were as follows: 40 U into biceps brachii,15 U into pronator teres, 15 U into .extensor carpi radialis, 15 U into extensor carpiulnaris, 15 U into long flexors of the fingers. Results: About a week later the patient reported with drooping of the left upper eyelid with classical diurnal variation. A Repetitive nerve stimulation test showed significant decrement in the left orbicularis oculi. There was no significant decremental or incremental response in the trapezius or the abductor pollicis brevis. Neostigmine test was positive. Ach receptor antibodies were negative in the blood. Video clippings of the patient pre-and post-injection show the significant drooping. A diagnosis o f ocular myasthenia was made and the patient started on pyridostig~nine. Patient improved significantly and has since then been on pyridostig~nine.

Assessments on day 21 and day 90 showed good reduction in the spasticity. In view of the unexpected complication, plans of further administration were dropped. Conclusions: Ocular myasthenia following or unmasked by botulinum toxin injection at a distant site has not been reported in the literature. Tiffs case poses an important question as to whether it is necessary to do a repetitive nerve stimulation in every case before injecting botulinum toxin.

0906 Natriuretie peptide mediated cgmp synthesis and phosphodiesterase activity in the rat spinal cord slice preparation

Vies, J~, Markerink-van, [~, De Vente, j2. 1Department of Neurology, University Hospital Maastricht, The Netherlands.." 2European Graduate School of Neuroscience (EURON), Maastricht University, Dept. of Psychiatry and Neuropsychology, Maastricht, The Netherlands

The presence of natriuretic peptides (NP) has been described in the spinal cord almost 20 years ago. However, little is known about the role of these peptides in the spinal cord. Recently it has become clear that NPs function as growth factors during development. We described the ANP-responsive, cGMP-synthesizing structures in lanffna 1 and 2 of the rat spinal cord (Vles et al., Brain Res. 857, 219-234 2000). The intracellular cGMP concentration is determined by phosphodiesterase (PDE) activity. Presently 11 subfamilies of PDE's are known. Selective PDE inhibitors are known for number of these enzymes. We prepared rat spinal cord slices and we studied the effect of IBMX (nonselective), EHNA and BAY 60-7550 (PDE2 selective), sildenafil (PDE5 selective), dipyridamole (PDE5 and PDE10), and SCH81566 (PDE9) on the localisation of cGMP-immunoreactivity after incubation in vitro in the presence of ANP, BNP, or CNP. cGMP-innnunoreactivity was observed in fibers, running mainly longitudinal through lamina 1 and 2. In addition, a weak cGMP-immunostaining was observed in all gray matter layers localized in cells tentatively characterized as astrocytes. All PDE inlffbitors had qualitatively a similar effect, although there were quantitative differences. Only in the presence of sildenafil or IBMX we noted NP-stimulated cGMP synthesis in ependymal cells.

We conclude that NPs have a signaling role in the spinal cord, which might be modulated by GABA, as baclofen inlffbits NP-stimulated cGMP synthesis (De Louw et al., Neuroscie Lett. 321, 120 122 2002).

0907 Pereutaneous ratUofrequency lreatinent adjacent to the dorsal root ganglion as a treahnent modality for spaslieity in elfldxen

Vies, J*, de Louw, A ~, van Kleef M ~. 1Department of Neurology, Academic hospital Maastricht, The Netherlands.." ~Department of Anaesthesiology, Academic hospital Maastricht, The Netherlands

The treatment of spasticity in children with cerebral palsy remains to be a problem. Selective dorsal rtffzotomy (SRD) was shown to be effective, although major surgery is involved and adverse events have been reported. Percutaneous radiofrequency treatment of the dorsal root ganglion (RF-DRG) could be a simple and safe alternative treatment. In tiffs study we investigated the clinical effects of RF-DRG in the treatment of spasticity. In 20 children with care-giving problems, siccering and/or pain due to adductor spasm of the hip a RF-DRG treatment was performed. Individual treatment goals were evaluated using a VAS based questionnaire. In 6 severely affected spastic children the effects were systematically evaluated using the Gross Motor Function Measure (GMFM), the modified Ashworth scale, the Pediatric Evaluation of Disability Inventory (PEDI) and a care givers questiomtaire. We observed a slight improvement in muscle tone after treatment while no improvement was detected using the G M F M or PEDI. Therefore these assessment methods proved to be ineffective, while the individual treatment goals evaluated using the VAS based questiomtaire, were easily met according to the care givers.

We believe that R F-DR G is one of the possible options to treat spasticity in children and could be considered as an alternative in the range of treatment options available nowadays.

0908 Myodonus due to Subacute Sderosing Encephalitis in an Eighteen Years-Ohl Man

Wong, H 1, Kwan, p 1 Hui, A 1, Wong K 1 . 1Division of Neurology', Department of Medicine and Therapeutics, Prince of Wales Hospital, Hong R~ng SAR

Background: Subacute sclerosing encephalitis (SSPE) is a rare neurodegenerative disease with poor long-term prognosis. Four cases of SSPE were previously reported in Hong Kong due to the 1988 measles outbreak. Method: Review of a case with clinical and serological evidence of SSPE. Results: An 18-years old man presented with 5 months history of pro- gressive myoclonus disorder. He had measles infection in 1988 when he was one-year old. Initial symptoms were atonic attacks leading to stuttering of gait and falls. Deterioration in school performance was also noted. It progressed to myoclonus episodes of sudden extension of neck, arms and legs associated with tongue protrusion and speech arrest. The frequency of the myoclonus episodes was erratic, initially. It later became regular at two to three episodes per minutes but subsides during sleep. Video-electroencephalogram demonstrated periodic lffgh- voltage generalized slow waves complexes occurring at sixteen to twenty seconds-interval. Cerebrospinal fluid analysis from lmnbar puncture revealed measles antibody titre at 1:64 and the presence of IgG oligoclonal band. Magnetic. resonance imaging of the brain was unremarkable. Extensive work-up to rule out other conditions was negative. He fulfilled Dyken's criteria of SSPE and is currently at stage I1- of the disease. He has been treated with 1-soprinosine, clonazepam and piracetam with minimal improvement of his symptoms. Conclusion: This case highlights the long incubation period of 17 years between measles infection and the onset of SSPE. It hopefully will be the last case of this devastating disease originating from the 1988 measles outbreak in Hong Kong.

0909 Delayed Continuous Bruxism with Olivaxy Hyperthrophy after Top of Basflar Syndrome

Minn, y1, Cho, S 1, Kwon, K 1. ~Department of Neurology, Hallym University college of Medicine, Seoul, Korea