Bj Sweeney

The opinion of the general practitioner toward clinical management of patients with psychogenic nonepileptic seizures

OBJECTIVE The purpose of this work was to assess the opinion of general practitioners (GPs) regarding the diagnosis of psychogenic nonepileptic seizures (PNES) and the role they feel they should play in the management of the disorder. METHODS Patients with PNES were identified from hospital records. Seizure and patient characteristics were recorded. Their GPs were surveyed regarding their understanding of the diagnosis and ongoing management of PNES. RESULTS Twenty-three patients were identified over a 3-year period as having been diagnosed with PNES. Sixty-five percent of GPs agreed with the diagnosis, and when asked to grade their understanding of the diagnosis (poor = 1, excellent = 10), the mean score was 5.7 (+/-SD 2.3). Thirty-five percent of GPs felt psychological input was of benefit to their patients. Fifty-two percent of GPs felt comfortable following up these patients, either with or without neurology outpatient services. CONCLUSIONS PNES remains a difficult disease to manage. There is a high level of uncertainty regarding the optimum management of PNES among primary care physicians, for which further education is needed.

Vacuolar leucoencephalopathy and pulvinar sign in association with coeliac disease

Several neurological disorders have been described in association with coeliac disease, including epilepsy, myoclonus, ataxia, myelopathy and peripheral neuropathy. Disorders of white matter have been reported but are rare.1 We report the clinical, radiological and pathological findings of a man with coeliac disease, progressive neurological decline and spongiform white matter degeneration. A 55-year-old man presented with anxiety, headache and left upper limb jerking. Coeliac disease had been diagnosed 6 months previously after small bowel biopsy showing positive tissue transglutaminase antibodies. There was no medical history. The patient was not taking any drugs and there was no history of substance misuse. His daughter also has coeliac disease but is neurologically normal. Magnetic resonance imaging (MRI) of the brain and electroencephalogram (EEG) were normal and the patient was started on oral anticonvulsants. He re-presented 2 months later, with increasing anxiety, tremulousness, ataxia, confusion and focal onset seizures becoming secondarily generalised. On examination, he was agitated and was tremulous at rest. He looked about vaguely but was capable of following a 1-step command. Extraocular movements were full, with no nystagmus. Reflexes and plantar responses were normal. He had ataxia and dysmetria on finger-nose-finger testing and a broad-based gait. Sensation was intact. He deteriorated and developed tonic–clonic seizures, requiring intensive care unit admission. Repeat MRI of the brain showed diffuse abnormality of the white matter. Brain biopsy …

Vacuolar leucoencephalopathy and pulvinar sign in association with coeliac disease.

Several neurological disorders have been associated with coeliac disease, including epilepsy, ataxia and neuropathy. Here we report a rare case of white matter disease in a 55-year-old man with coeliac disease. He presented with anxiety, headache and left upper limb jerking. He subsequently developed epilepsy and brain MRI revealed diffuse white matter abnormality. He died 6 months after presentation due to status epilepticus and sepsis. Brain biopsy demonstrated vacuolar leucoencephalopathy with no evidence of vCJD. An extensive clinical screen excluded infectious, inflammatory and para-neoplastic causes for this condition. Coeliac disease may be causally associated with vacuolar leucoencephalopathy in this case.

Clinical neurophysiology referral patterns to a tertiary hospital : a prospective audit

AbstractBackground Cork University Hospital (CUH) provides a tertiary service for all neurophysiology referrals in the Southern Health Board region. Aim To ascertain the number, source, symptoms and diagnosis of neurophysiology referrals at CUH. Methods We did a prospective audit of the referral patterns to the neurophysiology department over a 12-week period. Results Of 635 referrals, 254 had electromyograms (EMG), 359 had electro-encephalograms (EEG), 18 had visual evoked potentials (VEP), three had somato-sensory evoked potentials (SSEP) and one had multiple sleep latency tests (MSLT). We analysed the demographic pattern, reason for referrals, the average waiting time for neurophysiology tests and the patterns of diagnosis in this audit. Conclusions Patients from County Cork are making more use of the neurophysiology services than patients from other counties within the Southern Health Board. The average waiting time for an EEG was 32 days and for an EMG was 74 days. However, more than 35% of those patients waiting for an EEG or an EMG had their tests done within four weeks of referral. The appointments of EEG and EMG were assigned on the basis of clinical need.

11. Nephrotic syndrome and Guillain-Barré syndrome – is there an association?

Introduction: To the authors’ knowledge, this is the first report of a pure sensory neuropathy seen in association with peripheral nerve hyperexcitability and positive serum voltage gated potassium channel (VGKC) antibodies. Case report: A 55-year-old woman presented in 1998 with fatigue, twitching in calves, and leg cramps of 4 years duration. Examination showed myokymia of calf and impaired proprioception in upper and lower limbs. Electrophysiology showed pure sensory neuropathy (absent SNAPs), fasciculations and medium rate continuous motor activity. FBC, Vitamin B12, Folate, metabolic profile, and thyroid function were normal. Antiganglioside antibodies, anti-Ro and anti-La/Sm/RNP antibodies, anti-Hu antibody were negative. Cerebrospinal fluid profile was normal. Serum was strongly positive for serum VGKC antibodies. Serum HLA typing showed autoimmune pattern. Chest and brain imaging were normal. She found a dramatic improvement of cramps following plasmapheresis. Serum VGKC antibodies became negative. She was maintained on steroids and azathioprine. Her symptoms gradually worsened. Examination showed marked sensory ataxia. She underwent plasmapheresis with minimal improvement. Electrophysiology showed significant improvement in membrane excitability with no effect on sensory neuropathy. Her condition plateaued over 5 years; electrophysiology did not change. Recently, her symptoms had worsened. Electrophysiology shows sensory neuropathy unchanged with the return of spontaneous motor activity. She has been treated with intravenous immunoglobulin. Conclusion: Pure sensory neuropathy can be a feature of peripheral nerve hyperexcitability associated with positive VGKC antibodies. An extensive search for a paraneoplastic cause was negative. An antibody mediated dorsal root ganglionopathy may play a part in its pathogenesis.

P05.4 Inclusion body myositis, the great mimicker! – Lessons from 2 cases

tool pointing to abnormalities in muscle contractile properties in myopathies. Objects and methods: We investigated the clinical value of the MMG in determining muscle contractility in patients with myopathies. Five percutaneous supramaximal electrical stimulations were delivered to the biceps brachii muscle while simultaneously recording baseline MMG peak to peak (p–p) amplitudes. Subjects were then asked to perform isometric maximum voluntary contraction (MVC) for 10 s after which another 5 supramaximal percutaneous stimulations were delivered. MMG-PAP was determined by getting the ratio of the MMG p–p amplitude immediately after MVC to the mean of the baseline MMG recordings. We did preliminary antibody staining for NOS in the muscle biopsies of some muscles since the abnormal contractile property was reported to be correlated with NOS production. Results: Mean PAP in myopathies (66 + 53%) was significantly different from disease (128 + 34%; p < 0.005) and normal controls (102 + 43%; P < 0.05). When myopathic patients were segregated into dystrophic and nondystrophic groups, the mean PAP of the non-dystrophic group (38 + 20%) was much lower than the disease and normal control groups (p < 0.0001 and p < 0.001, respectively). The mean PAP of the dystrophic group (148 + 23%) did not differ from the disease and normal controls but differed significantly from the non-dystrophic group (p < 0.005). The patients with inflammatory myopathies and low PAP had increased staining for iNOS in the muscle nuclei. In the patient with mitochondrial myopathy and low PAP, and in the patient with OPMD, no significant staining can be found. Discussion: MMG determination of PAP may be a useful tool in quantifying changes in muscle contractility. However, clearly, further investigations regarding the effect of iNOS on muscle contractility need to be done.

P35.23 Ross syndrome – 1st documented case in Ireland

complication of surgical treatment of bilateral chemodectoma, and the usefulness of this method to assess patients with autonomic storm. Patient and methods: A 27-year-old man had surgical resection of bilateral giant carotid body chemodectomas. Immediately after the second procedure he developed paroxysmal hypertension, severe headaches, vomiting and dysphagia. Baroreceptor sensitivity was measured with a computer system during continuous non-invasive blood pressure monitoring. Results: The methods were well tolerated during autonomic storm. Baroreflex sensitivity was 3.73 ± 2.31 ms/ mmHg (normal values: 8.41–10.62 in 70 controls). Conclusion: Baroreceptor sensitivity can be easily used in a patient during autonomic storm to assess carotid body denervation.