Clinical features and disease progression of L-2-hydroxyglutaric aciduria in 27 Staffordshire bull terriers.

To describe the development of clinical signs (CS) and outcome of L-2-hydroxyglutaric aciduria (L-2-HGA), owners of 119 Staffordshire bull terriers positive for the known L-2-hydroxyglutarate dehydrogenase autosomal-recessive mutations were requested to complete a questionnaire regarding their pet's CS. Questionnaires were returned for 27 dogs, all with neurological abnormalities-not all questions were answered in all cases. The mean age of CS onset was 12 months (range 2.5-60). Gait dysfunction was reported in 26/26 dogs, with stiffness of all four limbs the most common (24/26) and earliest recognised abnormality. Kyphosis (19/26), body and/or head tremors (19/26) and hypermetria (15/26) were frequent. Behavioural changes were present in 24/27 dogs; most commonly staring into space (21/24), signs of dementia (17/24) and loss of training (15/24). Eighteen dogs demonstrated paroxysmal seizure-like/dyskinetic episodes. Nineteen (70 per cent) dogs were alive at a mean survival time of 76.6 months (12-170) after onset of CS. L-2-HGA was the cause of euthanasia in six dogs. Euthanasia occurred at a mean survival time of 44 months (8.5-93) after onset of CS, with 2/8 dogs euthanased within 12 months. L-2-HGA is considered a progressive neurological disease; however, CS can be successfully managed with affected dogs potentially living a normal lifespan.

Hypnotherapy for irritable bowel syndrome: an audit of one thousand adult patients.

BACKGROUND: Gut-focused hypnotherapy improves the symptoms of irritable bowel syndrome (IBS) with benefits being sustained for many years. Despite this, the technique has not been widely adopted by healthcare systems, possibly due to relatively small numbers in published studies and uncertainty about how it should be provided. AIM: To review the effect of hypnotherapy in a large cohort of refractory IBS patients. METHODS: One thousand IBS patients fulfilling Rome II criteria, mean age 51.6 years (range 17-91 years), 80% female, receiving 12 sessions of hypnotherapy over 3 months, were studied. The primary outcome was a 50 point reduction in the IBS Symptom Severity Score. The fall in scores for Noncolonic Symptoms, Quality of Life and Anxiety or Depression, were secondary outcomes. The Federal Drug Administration's recommended outcome of a 30% or more reduction in abdominal pain was also recorded. RESULTS: Overall, 76% met the primary outcome which was higher in females (females: 80%, males: 62%, P < 0.001) and those with anxiety (anxious: 79%, non-anxious: 71%, P = 0.010). The mean reduction in other scores was: IBS Symptom Severity Score, 129 points (P < 0.001), Noncolonic Symptom Score, 65 (P < 0.001) and Quality of Life Score, 66 (P < 0.001). Sixty-seven per cent reported a 30% or more reduction in abdominal pain scores. Pain days fell from 18 to 9 per month. Patients with anxiety and depression fell from 63% to 34% and 25% to 12% respectively (P < 0.001). Outcome was unaffected by bowel habit subtype. CONCLUSION: These results provide further evidence that gut-focused hypnotherapy is an effective intervention for refractory IBS.

Urine concentrations of xanthine, hypoxanthine and uric acid in UK Cavalier King Charles spaniels.

OBJECTIVES: Xanthine urolithiasis and asymptomatic xanthinuria have been diagnosed in Cavalier King Charles spaniel dogs suggesting that primary xanthinuria may be a breed-related disorder, although its prevalence remains unclear. The hypothesis of this study was that asymptomatic xanthinuria is common in Cavalier King Charles spaniel dogs. METHODS: Free catch urine samples were collected from 35 client-owned Cavalier King Charles spaniel dogs and from 24 dogs of other breeds. The purine metabolites were measured by high-performance liquid chromatography. The urine ratios of xanthine/creatinine and hypoxanthine/creatinine were calculated and compared between the two groups of dogs. RESULTS: The urine concentrations of purine metabolites were not significantly different between the two groups and were very low in both. The urine concentrations of xanthine in all 35 Cavalier King Charles spaniel were markedly lower than in the previously reported case of xanthine urolithiasis in a UK Cavalier King Charles spaniel dog. CLINICAL SIGNIFICANCE: Asymptomatic xanthinuria was not detected in this UK Cavalier King Charles spaniel population. This data may be used as a reference for urinary purine metabolite concentrations in the dog.

Reactivity to images in health and irritable bowel syndrome.

BACKGROUND: We have been using a medical artist to record and paint the images patients have of their irritable bowel syndrome (IBS) and have hypothesized that the reaction to such images might differ in health and IBS, which could have practical implications for future research. AIM: To examine reactivity to images in health and IBS. METHODS: Twelve paintings of IBS were shown to 70 patients to determine the four most evocative images. The spontaneous response to these images and four 'non-IBS painful' and four neutral paintings was assessed in another 100 IBS patients and 100 controls. The prompted reaction in terms of whether an image evoked the notion of pain, bloating or discomfort and to what degree was also recorded. RESULTS: Four images depicting bloating and pain scored the highest. These IBS images triggered significantly different reactivity between patients and controls in terms of their spontaneous and prompted responses. Even 'non-IBS painful' and neutral images resulted in exaggerated and frequently significantly different responses in patients than in controls. CONCLUSIONS: Visual hypersensitivity appears to be another manifestation of the tendency of IBS patients to react adversely to a variety of endogenous and exogenous stimuli. Identifying how individuals relate to different images might also give useful insights into understanding gastrointestinal symptoms.

Association between cervical and intracranial dimensions and syringomyelia in the cavalier King Charles spaniel.

OBJECTIVES: To investigate the possible association between caudal fossa area and cervical vertebral dimensions and the presence of syringomyelia in cavalier King Charles spaniels. METHODS: From magnetic resonance imaging scans of 78 cavalier King Charles spaniels, measurements were made of the widest vertical spinal width at C1/C2, C2, C2/C3 and C3; angulation of the C2/C3 spine; and estimated caudal fossa area. A correlation between these measurements and syringomyelia was sought. RESULTS: A total of 59 dogs with and 19 without syringomyelia were compared. Older dogs had a significantly higher incidence of syringomyelia. No difference in incidence was noted between genders. There was no significant difference in vertebral canal width at C1/C2 and C2, or angulation of C2/C3 between syringomyelia and non-syringomyelia groups. The width of the canal at C2/C3 and C3 was significantly increased in syringomyelia dogs. There was no significant difference in the caudal fossa area between groups. CLINICAL SIGNIFICANCE: Although syringomyelia was shown to be more prevalent in older dogs, the age beyond which dogs were considered at greater risk was not deducible from the dataset. The association identified between wider spinal canal at C3, and C2/C3 and syringomyelia presence is of questionable clinical significance, as the difference between syringomyelia and non-syringomyelia groups is too small to be measured in a clinical setting.

Syringomyelia in cavalier King Charles spaniels: the relationship between syrinx dimensions and pain.

OBJECTIVES: This study was designed to test the hypothesis that pain associated with syringomyelia in dogs is dependent upon size and involvement of the dorsal part of the spinal cord. METHODS: Masked observers determined syrinx dimensions and precise location within the spinal cord on magnetic resonance images of 55 cavalier King Charles spaniels with syringomyelia. After removal of masking, syrinx size and location were compared between the cohorts of dogs that exhibited pain with those that did not. RESULTS: Maximum syrinx width was the strongest predictor of pain, scratching behaviour and scoliosis in dogs with syringomyelia. Both pain and syrinx size were positively correlated with syrinxes located in the dorsal half of the spinal cord. CLINICAL SIGNIFICANCE: Large syrinxes associated with damage to the dorsal part of the spinal cord are associated with persistent pain suggesting that the pain behaviour expressed by this group of patients is likely to be "neuropathic pain," resulting from disordered neural processing in the damaged dorsal horn. As such it is likely that conventional analgesic medication may be ineffective.

An algorithmic approach to the management of cutaneous burns.

An algorithm of fluid resuscitation for cutaneous burns is presented. The management of these cases entails an initial process of evaluation followed, when appropriate, by the administration of intravenous fluids. The fluid requirements are prospectively calculated and subsequently modified by using closed loop feedback with monitoring of urine output, plasma deficit, osmolality and vital signs. Three 'problem boxes' have been incorporated into the algorithm to outline the management of: 1. fluid deficit and excess; 2. acute renal failure; 3. the critically ill patient. An algorithm such as this can never be complete, but in its present form it provides both a teaching tool for junior staff as well as a valuable clinical aid for those involved in the acute management of burns patients.