Factors related to frequency,type, and outcome of anal fistulas in Crohn's disease

PURPOSE: Relation of clinical factors to frequency, type, and, in particular, outcome of anal fistulas in Crohn's disease was studied. METHODS: One hundred twelve patients seen in this hospital between January 1972 and June 1993 who suffered from Crohn's disease were included in the study. Those 35 (31 percent) with anal fistulas were reexamined or interviewed and asked about their perianal symptoms and anal control. RESULTS: Rectal involvement of Crohn's disease was associated with an increased incidence of anal fistula (49 vs. 17 percent;P <0.01), especially high ones (82 vs. 17 percent;P <0.01). Ten of 18 patients with low fistulas underwent fistulotomy; all 10 fistulas healed, but slowly (mean healing time, 7.5 months), and 4 of them recurred. Of eight low fistulas managed by drainage alone, four healed. Finally, 11 of 18 patients with low fistulas had their fistulas healed. Fourteen of 17 patients with high fistulas were primarily treated by drainage and 3 by local surgery. Finally, only three patients had healed fistulas—two after simple drainage and one after local surgery, and seven patients had to undergo proctectomy. Only two patients with low fistulas required proctectomy. Eight patients (33 percent) of those 24 with fistulas in whom anal continence could be assessed, 5 with local surgery and 3 with drainage alone, reported minor defects in anal control. CONCLUSIONS: Fistulotomy is a justifiable option with satisfactory results for low symptomatic anal fistulas associated with Crohn's disease, although healing may be delayed and some fistulas will recur. Outcome of high fistulas is less satisfactory, and proctectomy is ultimately required in a number of patients; therefore, for high fistulas a conservative approach is primarily recommended.     

Sensory neuropathy in infantile onset spinocerebellar ataxia (IOSCA)

Infantile onset spinocerebellar ataxia with sensory neuropathy is a new, inherited multisystem disorder discovered in 19 Finnish patients. In order to define the neuropathy of the disease, we measured sensory nerve action potentials and nerve conduction velocities in 18 patients, and recorded somatosensory evoked potentials (SEP) in 10 patients and performed a sural nerve biopsy in 13 patients. The fixed and teased nerve fascicles were examined by light and electron microscopy, and the whole transverse section of a nerve fascicle was photographed and enlarged for morphometric measurements. Our investigation revealed an early onset, rapidly progressive axonal neuropathy: the sensory action potentials were decreased after the age of 2 and a severe loss of mainly large myelinated fibers was found. © 1994 John & Sons, Inc.     

Viral encephalitis: a review of diagnostic methods and guidelines for management

Viral encephalitis is a medical emergency. The spectrum of brain involvement and the prognosis are dependent mainly on the specific pathogen and the immunological state of the host. Although specific therapy is limited to only several viral agents, correct immediate diagnosis and introduction of symptomatic and specific therapy has a dramatic influence upon survival and reduces the extent of permanent brain injury in survivors. We searched MEDLINE (National Library of Medicine) for relevant literature from 1966 to May 2004. Review articles and book chapters were also included. Recommendations are based on this literature based on our judgment of the relevance of the references to the subject. Recommendations were reached by consensus. Where there was lack of evidence but consensus was clear we have stated our opinion as good practice points. Diagnosis should be based on medical history, examination followed by analysis of cerebrospinal fluid for protein and glucose contents, cellular analysis and identification of the pathogen by polymerase chain reaction (PCR) amplification (recommendation level A) and serology (recommendation level B). Neuroimaging, preferably by magnetic resonance imaging, is an essential aspect of evaluation (recommendation level B). Lumbar puncture can follow neuroimaging when immediately available, but if this cannot be obtained at the shortest span of time it should be delayed only in the presence of strict contraindications. Brain biopsy should be reserved only for unusual and diagnostically difficult cases. All encephalitis cases must be hospitalized with an access to intensive care units. Supportive therapy is an important basis of management. Specific, evidence-based, anti-viral therapy, acyclovir, is available for herpes encephalitis (recommendation level A). Acyclovir might also be effective for varicella-zoster virus encephalitis, gancyclovir and foscarnet for cytomegalovirus encephalitis and pleconaril for enterovirus encephalitis (IV class of evidence). Corticosteroids as an adjunct treatment for acute viral encephalitis are not generally considered to be effective and their use is controversial. Surgical decompression is indicated for impending uncal herniation or increased intracranial pressure refractory to medical management.     

Erratum to: Long-term results of surgery for lumbar spinal stenosis: a randomised controlled trial

European Spine Journal -     

Localization of Glial Cell Line-derived Neurotrophic Factor (GDNF) mRNA in Embryonic Rat by In Situ Hybridization

The localization of glial cell line-derived neurotrophic factor (GDNF) mRNA was studied by in situ hybridization in rat from embryonic (E) day E10 to E15. At E10, GDNF mRNA is found in the urogenital field and the cranial part of the gut. At E11, the most abundant expression of GDNF mRNA is seen in the epithelial cells of the second, third and fourth pharyngeal pouches, the third and fourth pharyngeal arches and pharynx. Also mesenchymal cells of the gut and mesonephric tubules contain GDNF mRNA. At E13, expression is observed in the mesenchymal cell layers of the oesophagus, intestine and stomach, the mesenchymal cells around the condensing cartilages and metanephric kidney mesenchyme. Also, the epithelia of Rathke's pouch and pharynx are intensely labelled. High expression of GDNF mRNA continues at El5 in kidney, gastrointestinal tract and cartilage. At that stage, GDNF mRNA is seen also in whisker pad and skeletal muscles. The distribution of GDNF mRNA in embryonic rat suggests important roles for GDNF in the early differentiation of the kidney tubules, the innervation of the gastrointestinal tract and the differentiation process of the cartilage and muscle. Our results indicate novel functions for GDNF outside the nervous system.     

Stability of vocational outcome in adulthood after moderate to severe preschool brain injury.

We studied how moderate to severe childhood traumatic brain injury (TBI) affects vocational outcome with time. This is the second follow-up of patients who were injured as preschoolers in traffic accidents. In the first follow-up the mean age was 23 years and in the present study the average age of the 27-patient cohort was 40 years. Twenty-two patients were assessed clinically by a neuropsychologist, neurologist and a social worker. Five patients, although not able or willing to participate in the clinical study, were contacted by telephone and interviewed on their vocational outcome. Compared to the first follow-up, 20/27 patients in total had no change in their vocational status. Nine out of the 27 patients were working full-time, two had subsidized jobs and 16 were not working. Twenty-four of 27 patients were independent in daily living. In the neuropsychological tests of executive functions, preserved flexibility associated with full-time work status. In conclusion, 1/3 of the patients were still employed full-time over 30 years after the TBI. This suggests that favorable vocational outcome, reached by young adulthood, is maintained at least until middle age.     

Ultrastructural studies of the retina in infantile neuronal ceroid-lipofuscinosis

A 9-year-old boy who had died of infantile neuronal ceroid-lipofuscinosis had experienced retina-derived visual failure. Ophthalmologically and morphologically, his retina was severely atrophic and scarred by a dense fibrillary gliosis while photoreceptor cells had completely disappeared, cells of the bipolar layer had decreased in number and had become atrophic beyond cytologic recognition. Retinal pigment epithelial cells had undergone either atrophy or proliferation. Disease-specific granular lipopigments had accumulated in perikarya and processes of remaining cells and were infrequently associated with melanin within huge melanolipofuscin bodies and RPE cells of sessile and migrating nature, and within cells of the atrophic retina, among them glial cells. The ubiquitous lipopigment accumulation in the retina of this patient was identical to that seen in other childhood forms of NCL and to that observed in canine NCL while the retinopathy likewise resembled that encountered in childhood NCL, and in primary human and canine retinopathia pigmentosa.     

The Association Between Visual Acuity and Functional Limitations: Findings from a Nationally Representative Population Survey

Purpose: To determine the independent effect of visual acuity on individual activities of daily living (ADL), instrumental activities of daily living (IADL) and mobility. Methods: Cross-sectional survey on a sample representing the Finnish population aged 55 years and above. Of the 3392 eligible people, 3185 (93.9%) were interviewed, 2870 (84.6%) attended a comprehensive health examination, and 2781 (82.0%) had distance visual acuity (VA) assessed. A home interview included assessment of ADL, IADL and mobility, demographic variables and chronic conditions. Mobility measurements and binocular VA were assessed during the examination. Results: Prevalence of ADL, IADL, and mobility limitations increased with decreasing VA (p < 0.001). Visually impaired persons (VA ≤ 0.25) had ADL disabilities four times more likely than those with good VA (VA ≥ 0.8) after adjustment for socio-demographic and behavioral factors, and chronic conditions (OR 4.36, 95%CI 2.44–7.78). Limitations in IADL and measured mobility were five times as likely (OR 4.82, 95%CI 2.38–9.76 and OR 5.37, 95%CI 2.44–7.78, respectively), and self-reported mobility limitations were three times as likely (OR 3.07, 95%CI 1.67–9.63) as in persons with good VA. Conclusions: Decreased VA is strongly associated with functional limitations, and even a slight decrease in VA was found to be associated with limitations in functioning.