Surgical aspects of pediatric urinary incontinence

This thesis tries to bridge between functional and structural non-neuro- genic incontinence and to give insight in the surgical options. Children with anatomically based bladder neck and urethral insufficiency often present with the same symptoms as children with genuine non-neurogenic functional incontinence. Bladder neck insufficiency causes urine to leak into the proximal urethra and urine in the proximal urethra is one of the triggers of the micturition reflex with detrusor contraction and sphinc- teric relaxation as a result. Children with an insufficient bladder neck are constantly counteracting this micturition reflex by pelvic floor contraction and can present with typical symptoms of true dysfunctional voiding. Constant awareness of these facts is mandatory for all the members of the team. The urologist should be very meticulous in the endoscopic and ultrasound judgement of the anatomy of the bladder, the bladder neck and the urethra. Video-urodynamic studies must be interpreted with the assumption that derailment of the Wolffian duct can be a cause of the incontinence. The urotherapist must know that some children cannot be cured by biofeedback treatment and eventually will need an operation to get rid of the urinary incontinence. The physical therapist is the only per- son that can specifically teach the child to use the puborectalis muscle instead of all the muscles around. This is an underestimated task because many physical therapists do not realise that this result can only be obtained by invasive treatment with rectal examination and biofeedback training by anal balloon expulsion. Meanwhile, the physical therapist must be aware of the rare neurogenic anomalies that sometimes can be over- looked by urodynamic studies. The psychologists and all the members of the team should realise the psychological impact of incontinence, some- times combined with malformations of the external genitalia, on the behaviour of a child. Any child with a handicap copes with the disability by eleminating the concerning part of the body from the self-image. The resulting ignoring behaviour of the child that should be interpreted as a natural defence mechanism to cope with the disability is commonly mis- interpreted as a behavioural disorder by parents and therapists. Finally one must realise that also children with structural anatomically based inconti- nence as in bladder extrophy and epispadias as well as children with ambiguous genitalia often need to be treated by the team of urotherapists and physical therapists to accomodate the function of the restored anato- my to the daily life situation.. Moreover, in many cases these children need specific guidance for gender identity and psychosexual development by a team that is specialised in dealing with ambiguous genitalia in childhood. The chapters in this thesis describe functional incontinence and many aspects of structural incontinence in childhood. The main objective in the treatment of the different patient groups has been to minimise morbidity and health care consumption in patient groups that historically present with chronic lower urinary tract problems. Our approach to ectopic urete- roceles, in our opinion, reduces the morbidity as much as possible. More research in this field by other groups will be neccessary to prove this fact. The Otis-urethrotomy victims, when untreated, are bound to life-long uri- nary incontinence with many frustrating events by failing therapies. Our approach for girls with ambiguous genitalia has been able to reduce the work-load of the urotherapists in this group of patients to zero, in con- trast to their work load in the relatively large group of patients that has been treated by historic schedules resulting in non-neurogenic bladder/sphincter dysfunction and/or urinary tract infections in the majority of the patients. The treatment of female epispadias as presented in this thesis appears to reduce the morbidity and the number of surgical procedures for the patients. Further an extensive overview of the treatment of extrophia vesicae (open bladder) and of epispadias is presented

Induction of c-Jun immunoreactivity in spinal cord and brainstem neurons in a transgenic mouse model for amyotrophic lateral sclerosis

Transgenic mice carrying amyotrophic lateral sclerosis (ALS)-linked superoxide dismutase 1 (SOD1) mutations develop a motoneuron disease resembling human ALS. c-Jun is a transcription factor frequently induced in injured neurons. In this study we have examined the distribution of c-Jun-immunoreactivity in the brainstem and spinal cord of transgenic SOD1 mice with a glycine 93 alanine (G93A) mutation. In non-transgenic littermates c-Jun immunostaining was predominantly situated in motoneurons. The number of c-Jun immunoreactive motoneuron was reduced in SOD1(G93A) mice due to pronounced loss of motoneurons. In SOD1(G93A) mice, however, c-Jun-immunoreactivity was strongly induced in neurons in the intermediate zone (Rexed's laminae V-VIII and X) of the spinal cord and throughout the brainstem reticular formation. These findings are of interest since increased levels of c-jun also have been found in the intermediate zone of the spinal cord of ALS patients. Thus c-Jun may be involved in the neurodegenerative processes both in ALS and in motoneuron disease in SOD1(G93A) mice

Differential diagnosis in spinal and bulbar muscular atrophy clinical and molecular aspects

Kennedy disease is caused by an enlarged trinucleotide repeat sequence within the androgen receptor gene. We report here seven male patients with a benign motor neuron syndrome highly analogous to Kennedy disease but with a normal trinucleotide repeat

A descent into African psychiatry

Dertien jaar geleden werkte ik als tropenarts in Zuid-Senegal en Guinee Bissau. Na mijn terugkeer in Nederland volgde ik een psychiatrie-opleiding en vertrok vervolgens weer naar Afrika, ditmaal op verzoek van de Guineese regering om de geestelijke gezondheidszorg in Guinee Bissau op te zetten. De daaruit voortgekomen wisselwerking tussen de Guineese cultuur en de westerse psychiatrie vormt het hoofdtema van dit boek. ... Zie: Samenvatting

Immunohistochemical characterization of the inflammatory infiltrate in amyotrophic lateral sclerosis

In order to test the hypothesis that the immune system plays a role in the pathogenesis of amyotrophic lateral sclerosis (ALS), the cellular composition of the spinal cord inflammatory infiltrate was analysed in eight cases of sporadic ALS by a panel of monoclonal antibodies. The majority of the many diffusely scattered lymphocytes seen in the anterior and lateral corticospinal tracts and anterior horns belonged to the suppressor/cytotoxicity T-cell subset and were admixed with variable numbers of macrophages. Helper-inducer T-cells were rare and B-cells were conspicuously absent. Compared to controls, ALS specimens exhibited an increase in major histocompatibility complex (MHC) products or human leucocyte antigens (HLA) in the corticospinal tracts and anterior horns. HLA-ABC antigens were expressed in the honeycomb pattern of the glial matrix of the spinal cord, and HLA-DR antigens were strongly expressed by large dendritic cells. In addition, macrophages and endothelial cells were labelled by HLA-DR. These findings suggest that an autoimmune process or infectious agent may play a role in AL

Cervically induced ocular torsion: Physiological and clinical aspects

Ocular torsion was measured in five subjects during sinusoidal lateral tilt (amplitude 25°, 0.2 Hz). The cervical contribution to ocular torsion was best visible as the difference between the signals obtained in conditions with only head tilt and conditions with whole body tilt. Contribution of the neck did not affect the slow component, but produced an anticompensatory modulation of the beating field offset by means of saccades (analogous to gaze shift). Static tilt conditions (25°tilt) of the trunk only, the head only or the whole body showed similar data, although of smaller amplitude. The results from patients suffering from post-whiplash syndrome were similar to those of healthy subjects, showing large intersubject variability. The reduced tolerance to head tilt of whiplash patients restricts useful implementation of this sort of test in the clinic