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1/13. Neuromyotonia with neuropathy and muscle hypertrophy: association or cause?

    Continuous muscle fibre activity in a patient with demyelinating neuropathy is rare. We report an 18 year old boy who presented with walking difficulty and continuous rippling in muscles of upper and lower limbs. He had dysarthric speech, hypertrophied arm and calf muscles with normal power, tone, reflexes and sensations. Myokymic discharges were seen in deltoid, biceps, quadriceps and calf muscles. His blood counts, chemistry, thyroid profile, dna, Rh factor were normal and CPK was raised. CSF showed protein 50 mg/dl and 4 lymphocyte/mm3. Nerve conduction study revealed conduction block and absence of peroneal F wave. EMG showed neuromyotonic discharges which disappeared on regional neuromuscular blocker but not on nerve block or general anaesthesia. He responded partially to prednisolone. Acquired demyelinating neuropathy may result in neuromyotonia and muscle hypertrophy which may partially respond to prednisolone.
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2/13. Neurological complications of systemic sclerosis--a report of three cases and review of the literature.

    We report three cases of systemic sclerosis demonstrating four different neurological complications: trigeminal neuropathy, peripheral neuropathy, carpal-tunnel syndrome and prolonged response to local anaesthesia. A review of the literature reveals a wide range of neurological abnormalities associated with systemic sclerosis. When they occur, these are often presenting features.
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3/13. Anaesthetic management of a patient with familial amyloid polyneuropathy of the Portuguese type.

    Although familial amyloid polyneuropathy of the Portuguese type (FAP-PT) was first described in 1952, there is little in the medical literature detailing the anaesthetic management of such patients. FAP-PT is a disease with multiple clinical manifestations which include disturbances of sensibility, progressive paresis starting in the lower extremities, autonomic dysfunction, cardiac conduction disturbances, gastro-intestinal disorders, nephrotic syndrome, sexual and sphincter disorders, extreme emotionalism and apprehension. Several intermingling problems have to be considered in the anaesthetic management of each individual case. In our patient a sinus dysrhythmia resolved after isoflurane and this seems to be a good choice for general anaesthesia in patients with FAP-PT, if they are in an early stage of heart involvement.
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4/13. Peripheral neuropathy associated with the sicca syndrome.

    Three patients with the sicca syndrome and chronic sensory neuropathy are described; in two of them neuropathy was the presenting feature of the disease. The sicca syndrome can give rise to a characteristic neurological syndrome comprising areflexia and asymmetrical sensory loss, particularly of proprioception, in the limbs. This is often associated with tonic pupils and trigeminal anaesthesia.
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5/13. Congenital trigeminal neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome).

    A 2 1/2 year old child with clinical features of Goldenhar-Gorlin syndrome showed diminished pinprick sensation over the right half of the face. After surgery for the cleft lip, the child died. Neuropathological investigations showed agenesis of the right trigeminal nerve and hypoplasia of the right trigeminal brain-stem nuclei. Nosological aspects of the Goldenhar-Gorlin syndrome and previously reported cases of congenital trigeminal anaesthesia in this disorder are discussed. It is suggested that the hypoplasia of the trigeminal nerve is responsible for the diminished facial sensation seen in some patients with this craniofacial syndrome.
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6/13. femoral neuropathy: a complication of lithotomy position under spinal anaesthesia. Report of three cases.

    Three patients developed solitary unilateral peripheral femoral neuropathy after vaginal hysterectomy. All were operated under subarachnoid analgesia in the lithotomy position. Straight rod leg supports with swing stirrups were used and the procedures lasted for two and one-half hours. The complication is thought due to the extreme abduction of thighs with external rotation at the hip causing ischaemia of the femoral nerve as it is kinked beneath the tough inguinal ligament. The prognosis was found to be excellent with complete recovery within eight to ten weeks. The complication is preventable by using lateral thigh supports limiting the degree of abduction.
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7/13. Postoperative bilateral diaphragmatic paralysis and subsequent anaesthesia.

    A case is described of a 56-year-old man who developed bilateral diaphragmatic paralysis following surgery remote from the course of the phrenic nerves. This rare complication is reviewed and the management of subsequent anaesthesia described.
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8/13. Transient radicular irritation after single subarachnoid injection of isobaric 2% lignocaine for spinal anaesthesia.

    Several cases have been reported recently in which symptoms suggestive of transient radicular irritation occurred following the use of hyperbaric 5% lignocaine for spinal anaesthesia. We report on three patients in whom we observed similar symptoms attributable to this kind of radicular irritation following uneventful spinal anaesthesia using isobaric 2% lignocaine. All three patients underwent minor gynaecological procedures and developed burning pains in the buttocks within 24 h of surgery. The long-term outcome was not clear for all the patients, but in at least one the pain disappeared.
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ranking = 6
keywords = anaesthesia
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9/13. sciatic nerve palsy following childbirth.

    Two cases are reported of sciatic nerve palsy after delivery by Caesarean section in primigravidae. One mother was slender and had an emergency Caesarean section for failure to progress with a breech presentation. Epidural analgesia during labour was extended for operative delivery. The other mother was obese, mildly hypertensive, had a large baby with a high head and was delivered by elective Caesarean section under epidural anaesthesia. She experienced severe intrapartum hypotension. Both patients suffered right sided sciatic nerve palsy. The aetiologies of obstetric palsies and those following regional block are reviewed and the importance of careful diagnosis and of avoiding peripheral nerve compression during regional block are emphasised.
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10/13. Spinal anaesthesia and spina-bifida occulta.

    We describe a patient with unexpected spina bifida who underwent spinal anaesthesia for trans-urethral resection of prostate and developed serious neurological signs. An unexpected spinal tumour was removed two weeks later. This report demonstrates that not all neurological problems associated with spinal anaesthesia should be blamed on the technique.
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ranking = 6
keywords = anaesthesia
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