Follow-up of patients with one kidney.
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Follow-Up Studies↗
Biomedical subjects
P J Shaw
Publications and source records attributed to P J Shaw.
The quantitative autoradiographic distribution of [3H]MK-801 binding sites in the normal human brainstem in relation to motor neuron disease.
The distribution of N-methyl-D-aspartate (NMDA) receptors in the normal human brainstem has been investigated using MK-801. Specific [3H]MK-801 binding showed a heterogeneous distribution, the greatest density of binding sites being found in the substantia nigra, locus coeruleus, and the hypoglossal and inferior olivary nuclei. Brainstem motor nuclei subserving eye movements, which tend to be spared in motor neuron disease (MND), had significantly lower densities of binding compared to other cranial nerve motor nuclei (V, VII, X, XII) which tend to be affected. The anatomical distribution of NMDA receptors may be one factor determining selective vulnerability to excitotoxic injury.
Adult↗
Neuroblastoma with intracranial involvement: an ENSG Study.
We report the experience of the European Neuroblastoma Study Group (ENSG) with central nervous system (CNS) involvement of neuroblastoma. Among this series of intensively treated patients, CNS neuroblastoma was diagnosed by computerised tomography (CT) scanning, rather than by autopsy. Cranial disease occurred in 5% of ENSG patients. Of 11 patients with intracranial disease, 4 had disease in the posterior fossa, a site rarely reported previously. Furthermore, 5 cases had CNS metastases at a time when there was no detectable disease elsewhere, rather than as part of extensive relapse. The pattern of disease we observed, at least for those with parenchymal disease, is in keeping with arterial spread. Although CT scanning is the optimal modality for identifying CNS disease, 2 cases had normal head CT scans prior to the onset of CNS disease. As most patients had symptoms of raised intracranial pressure (RICP) at the time the CNS disease was diagnosed, there does not seem to be any indication for routine CT scanning of the head at diagnosis, but this should be performed as soon as any symptoms or signs appear. With patients living longer with their disease, vigilance must be maintained during follow-up.
Antineoplastic Combined Chemotherapy Protocols↗
Intracranial extension of orbital pseudotumour.
Radiologically demonstrable intracranial extension of orbital pseudotumour is rare, and only four cases appear to have been reported previously in the literature. A review of the computed tomographic (CT) images of 90 biopsy proven cases of orbital pseudotumour seen over a 10 year period revealed eight cases with intracranial meningeal spread.
Adult↗
Re: The necessity of contralateral surgical exploration in Wilms tumor with modern noninvasive imaging technique: a reassessment.
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Humans↗
Correlation of bacteriological flora of the urethra, glans and perineum with organisms causing urinary tract infection in the spinal injured male patient.
Positive urine cultures are common and often asymptomatic in the male spinal injured patient performing self clean intermittent catheterisation. It is possible that the positive urine cultures result from contamination from the colonised urethra at the time of catheterisation. This contamination could result in true infection of the bladder urine or yield false positive results, explaining the frequently seen asymptomatic cases. In a prospective study positive urine cultures were found on 58 occasions (74%) in 10 asymptomatic patients studied. In 19% of screenings, with positive urine cultures, an identical organism was cultured from the catheter specimen of urine, the perineum and the urethra. The flora of the anterior urethra is strongly correlated to that of the perineum (37.1%), as well as that of the bladder (52.6%). In 4 patients a correlation also existed between the urethra and fingers, and the perineum and fingers. This was associated with an increased incidence of positive urine culture in these patients. Suprapubic aspirates of urine before and after catheterisation cultured the same organisms. However, quantitative culture revealed colony counts that approached a 10-fold increase following catheterisation in one patient. This suggests that catheterisation is at least partially responsible for ascending infection in this group of patients. Catheter specimens were found to be a good representation of the bladder urine, with an 87.5% correlation.
Adult↗
Complicated migraine resulting in blindness due to bilateral retinal infarction.
Retinal migraine is not uncommon, but permanent sequelae in the anterior visual pathway are rare. We describe the case of a young woman in whom blindness developed over a six-year period due to recurrent episodes of migraine-related occlusions of a branch retinal artery.
Adult↗
Adult-onset motor neuron disease and infantile Werdnig-Hoffmann disease (spinal muscular atrophy type 1) in the same family.
We describe a family in which infantile Werdnig-Hoffmann disease and adult-onset progressive muscular atrophy both occurred. The possibility of these two diseases developing within the same family by chance is unlikely, and several genetic hypotheses may be put forward to explain the association. We suggest that the molecular pathogenesis of these two subtypes of lower motor neuron degeneration may be linked. The genetic defect in the childhood spinal muscular atrophies has been mapped to chromosome 5q in close proximity to the microtubule-associated protein 1B locus. The association of diseases within this family suggests that chromosome 5q should also be studied in relation to adult-onset familial motor neuron disease.
Aged↗
Excitatory amino acid neurotransmission, excitotoxicity and excitotoxins.
Exciting recent developments have begun to define the molecular basis for excitatory amino acid (EAA) receptor diversity and function. Clarification of the roles of these receptors will require identification of the entire repertoire of EAA subunit genes, the subunit composition of each receptor subtype and the mapping of subunits within the central nervous system (CNS). This may allow the development of selective receptor targeting by therapeutic agents. Further evidence is emerging about the molecular processes underlying excitotoxic injury and the importance of free radical formation acting in concert with calcium-dependent processes is being increasingly recognized. There are many clues indicating that primary or secondary excitotoxic mechanisms may play a part in the pathogenesis of some chronic human neurodegenerative disorders. Further work is needed to clarify the mechanisms of selective vulnerability of particular neuronal types given the widespread distribution of EAA receptors within the CNS.
Alzheimer Disease↗
The quantitative autoradiographic distribution of [3H]MK-801 binding sites in the normal human spinal cord.
The distribution of NMDA receptors in the normal human spinal cord has been investigated using the non-competitive channel blocking agent MK-801. Specific [3H]MK-801 binding was present throughout the spinal grey matter at all segmental levels, the greatest density of binding being found in the substantia gelatinosa. Focal areas of high binding were also found in a distribution corresponding to lower motor neurones in the ventral horns. This study provides anatomical evidence that NMDA receptors are likely to be important in motor as well as sensory spinal synaptic transmission. The anatomical distribution of NMDA receptors in relation to motor neurone somata may have important implications in selective vulnerability to excitotoxic injury.
Aged↗
Bulbar and pseudobulbar palsy complicating therapy with high-dose cytosine arabinoside in children with leukemia.
Three of 38 children given high-dose cytosine arabinoside therapy developed a previously undescribed complication. Neurological problems are a frequent occurrence in patients given this therapy, particularly cerebellar ataxia, but the development of bulbar and pseudobulbar palsy has not been reported. In two of these cases, it was sufficiently marked for the course of treatment to be curtailed and occurred at a relatively low cumulative dose of the drug. Neurotoxicity can occur at any time using high-dose cytosine therapy.
Adolescent↗
Microtubules rich in post-translationally modified alpha-tubulin form distinct arrays in frog lens epithelial cells.
Isolated frog lens epithelia were stained with antibodies against tyrosinated, detyrosinated or acetylated alpha-tubulin and observed by several means including a scanning confocal microscope. The most prominent feature of Rana pipiens lens cells was a primary cilium close to the apical surface of the cells above the centrosome. This structure was associated with microtubules rich in modified alpha-tubulin. The cilium was less pronounced but still discernible in the cells of another species R. ridibunda. In both species, the modified (acetylated or detyrosinated) microtubules formed arrays spatially distinct from the unmodified (tyrosinated) microtubules. The modified microtubules formed a basket of microtubules with a curly distribution around the nucleus while the tyrosinated array consisted predominantly of rather straighter microtubules running from the apical centrosome to the cell periphery, down the lateral sides of the cells and across the basal surface adjacent to the lens capsule and basement membrane. It is concluded that the organization of modified microtubules previously described for several types of cultured cells may represent a remnant of the three-dimensional perinuclear array of such microtubules described here for the cells of an intact epithelium.
Animals↗
Three-dimensional fluorescence microscopy.
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Microscopy, Fluorescence↗
Does the Kaufman prosthesis still have a place? Review of thirteen years' experience.
The cases of 57 patients who underwent implantation of a Kaufman prosthesis for urinary incontinence at this center are reviewed. All except one of the patients were incontinent following prostatic surgery. Although many of the patients required revision or adjustments after the initial implantation, 55 percent of the cases eventually had a satisfactory outcome. Patients rendered incontinent after retropubic prostatectomy had a higher success rate with the Kaufman prosthesis than patients after other types of prostatectomy. This simple and relatively inexpensive device can still play a useful role in the treatment of patients with mild and moderate stress incontinence following prostatectomy.
Aged↗
A consumer's guide to commercially available urodynamic equipment.
Eight commercially available urodynamic machines from 6 major manufacturers (Aspen Medical, Dantec, Electro-Medical Supplies (EMS), Uro-gyn, Ormed and Wiest) were assessed for accuracy, construction and user-friendliness. There was less than 10% inaccuracy with regard to fill volume, void rate and pressure measurements. The Wiest 6000 plus system was between 11 and 14% inaccurate during voided volume measurement and other systems were less than 10% inaccurate. User-friendliness was assessed during routine cystometry and is mainly dependent on the software supplied with the equipment. The merits and faults of each urodynamic system with regard to performing a urodynamic investigation, running a urodynamic service and data management are compared with an "ideal" urodynamic system.
Diagnostic Errors↗
Lower motor neuron degeneration and familial predisposition to colonic neoplasia in two adult siblings.
A previously unreported association between a familial predisposition to colonic neoplasia and familial adult onset lower motor neuron (LMN) degeneration is reported. Two brothers presented at the ages of 53 and 44 years with multiple colonic adenomata and invasive colonic carcinoma respectively. Subsequently both developed a virtually identical pattern of motor neuron disease of progressive muscular atrophy type. At presentation both had LMN weakness affecting predominantly the upper limb and neck muscles. The disease progressed rapidly to involve the lower limb and bulbar musculature and both brothers died after a 15 month course. Necropsy was performed on one brother and showed pathological changes confined to the LMNs with no evidence of involvement of the pyramidal tracts or motor cortex. The combination of these diseases in two brothers may be of importance in the search for genes responsible for familial motor neuron disorders. It is suggested that a genomic search should be directed initially to the vicinity of known colon neoplasia genes, particularly 5q, 17q and 18q.
Adenocarcinoma↗
Too stringent patient selection criteria in a clinical trial of alpha-blockers reduce the value of the results.
The patient selection criteria in a clinical trial of an alpha-blocker for the treatment of benign prostatic hypertrophy were such that 85% of the patients on the waiting lists of 4 hospitals had to be excluded. The value of such a trial is discussed. Trial protocols should be designed to allow the enrollment of patients truly representative of the patients for which the treatment is intended.
Adrenergic alpha-Antagonists↗
Hashimoto's encephalopathy: a steroid-responsive disorder associated with high anti-thyroid antibody titers--report of 5 cases.
We describe 5 patients with a relapsing encephalopathy in association with Hashimoto's disease and high titers of anti-thyroid antibodies. The presentation is usually with a subacute onset of confusion, alteration in conscious level, and focal or generalized seizures. The relapsing course, association with myoclonus or tremulousness, and episodes of stroke-like deterioration are characteristic features. The long-term prognosis is favorable with steroid therapy, though additional immunosuppressive therapy may be required. Neurologic investigation typically shows a diffusely abnormal EEG, high CSF protein level without pleocytosis, and normal brain CT and cerebral angiogram. Isotope brain scan may show patchy abnormal uptake. Hashimoto's encephalopathy should be recognized as a definite neurologic entity and added to the list of CNS complications of thyroid disease.
Adolescent↗