The temporal variant of frontotemporal dementia.

Frontotemporal dementia is a dementia syndrome with diverse clinical characteristics. Based upon clinical parameters and single photon emission computed tomography, we identified 47 frontotemporal dementia subjects. In 10 of these 47 the primary site of brain dysfunction was anterior temporal and orbital-frontal with other frontal regions relatively spared. In this temporal lobe variant (TLV) of frontotemporal dementia, five of the subjects had more severe left-sided, and five had more right-sided, hypoperfusion. The clinical, neuropsychological and neuropsychiatric features of predominantly left-sided (LTLV) and right-sided (RTLV) TLV subjects are discussed and contrasted with more frontal presentations of frontotemporal dementia. In LTLV, aphasia was usually the first and most severe clinical abnormality RTLV patients presented with behavioural disorders characterized by irritability, impulsiveness, bizarre alterations in dress, limited and fixed ideas, decreased facial expression and increased visual alertness. These findings suggest that: (i) frontotemporal dementia is clinically heterogeneous with bitemporal and inferior frontal lobe dysfunction contributing to the clinical presentation; (ii) behavioural disturbance and aphasia are the most prominent features of predominantly temporal subtypes of frontotemporal dementia; (iii) the right and left anterior temporal regions may mediate different behavioural functions. The results of this study suggests that TLV offers a valuable source of information concerning the behavioural disorders seen with combined anterior temporal and inferior frontal lobe dysfunction.

Clam ileocystoplasty.

Clam enterocystoplasty has been used successfully in the treatment of refractory urge incontinence. We report 31 patients who underwent ileocystoplasty between 1982 and 1989. The majority of patients were pleased with the outcome of the operation. The main post-operative complication was voiding dysfunction due to relative bladder outflow obstruction. Urinary tract infection and mucus production were significant long-term problems.

The changing practice of percutaneous stone surgery. Review of 1000 cases 1981-1988.

A review was carried out on 1000 cases of percutaneous nephrolithotomy (PCNL), Group 1 (500), 1981-1985, being compared with Group 2 (500), 1985-1988. Previous renal surgery had been performed in 17.4% of patients in Group 1 and 36% in Group 2. There were 17.2% complicated patients in Group 1 and 51% in Group 2. The stone burden included 30.2% multiple, partial staghorn and staghorn calculi in Group 1 and 47.2% in Group 2. The use of in situ stone disintegration increased from 22.2% in Group 1 to 73.4% in Group 2 and nephrostomy drainage was necessary in 29.6% compared with 75%. Post-operative complications increased from 13.6 to 24%. Stone-free rates decreased from 92 to 51%, but the addition of other methods of treatment and the inclusion of patients with stone fragments of 2 mm or less increased these figures to 98% in Group 1 and 83% in Group 2. More complicated patients with complex stones are now being referred for PCNL. These patients require multiple treatments, including extracorporeal shock wave lithotripsy (ESWL) and percutaneous surgery, in combination with other endoscopic and radiological procedures.

Wilms' tumour: a 30 year experience at Wellington Hospital.

Over the 30 year period from 1958-88, 20 cases of Wilms' tumour have been treated at Wellington Hospital. All eight patients presenting prior to 1976 have died. The 12 patients presenting after 1976 were treated according to the guidelines of the National Wilms' Tumour Study Group and ten of these are alive (mean follow up seven years). Four of the patients received preoperative chemotherapy and this was found to facilitate surgical removal of the tumour. The concept of partial nephrectomy in the management of Wilms' tumour is discussed. Retrospective analysis of this series has shown that this would have been a feasible management option in four of the cases reviewed.

Urethral syndrome (abacterial cystitis)--search for a pathogen.

Thirty-one women with the recurrent urethral syndrome (abacterial cystitis) had extensive microbiological, cytological and histological investigations. A Streptococcus species was isolated from the bladder aspirate of one patient, a wall-deficient Streptococcus species from two others and both a Lactobacillus species and Ureaplasma urealyticum from another. A possible microbiological cause was therefore identified in the bladder in only 4 of the 31 patients. A Lactobacillus species was isolated from the bladder aspirate of one control subject. Lactobacilli were grown in the voided urine of the majority of patients and in seven of the eight control subjects. Chlamydia trachomatis and Herpes simplex were isolated from the cervix and the urethra of one patient. Urethral cytology was normal in all patients. Trigonitis was noted at cystoscopy in 26 of the 31 patients. Bladder biopsies showed squamous metaplasia in 15 and lymphocytic infiltration of the lamina propria in 29, giving support to an inflammatory aetiology of this enigmatic syndrome.