Frontotemporal dementia and parkinsonism linked to chromosome 17 with the N279K tau mutation

We present a case of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP‐17) harboring the N279K mutation in the MAPT gene from the family known as pallido‐ponto‐nigral degeneration (PPND). This 49‐year‐old man was followed for 17 years. He presented at age 41 years with left leg stiffness and en‐bloc turning. During the course of his illness he developed a constellation of symptoms including parkinsonism, pyramidal signs, vertical gaze palsy, dysphagia, dystonia, personality and cognitive dysfunction, weight loss and mutism. Gross neuropathological examination showed mild atrophy of the cerebral cortex, hippocampal formation, amygdala, thalamus, subthalamic nucleus and depigmentation of the substantia nigra. Microscopy revealed neuronal loss and gliosis in the same regions. Tau immunohistochemistry showed pretangles, numerous threads, grain‐like structures and oligodendroglial tau‐positive inclusions (“coiled bodies”). In the spinal cord the tau pathology was more abundant in gray than white matter. Pretangles and threads were present in the anterior and, to a lesser extent, in the posterior horns. FTDP‐17 should be suspected in patients with a history of familial parkinsonism combined with behavioral and cognitive changes, onset before age 65 years and an aggressive clinical course.     

Estimation of gestational age by last menstrual period, by ultrasound scan and by SP1 concentration: comparisons with date of delivery

The date of delivery in 62 normal pregnant women was predicted by last menstrual period, by ultrasound scan and by the serum concentration of Schwangerschaftsprotein 1 (SP1). It was found that SP1 concentration in the first trimester gave as reliable an indication of when labour would occur as did the last menstrual period or ultrasound scan.     

Benign polyps with prostatic-type epithelium of the urethra and the urinary bladder

The clinico-pathological features of nine urethral and urinary bladder polyps with prostate-type epithelium are described. The average age of the patients was 46 years. Three patients previously had cystoscopy and the lesion was not noticed on the initial examination. The commonest presentation in this series was haematuria, dysuria and frequency of micturition. One patient presented with postmicturition dribble and another with haemospermia. The polyps contained acini and papillae lined by prostate-type epithelium which was confirmed by immunohistochemical tests for prostate specific antigen and prostate acid phosphatase. In this series no age versus location relationship could be established. Symptoms resolved following resection or initial biopsy followed by fulguration. Recurrence is extremely rare.     

Neurotoxic effects of MDMA (“ecstasy”) administration to neonatal rats

3,4-Methylenedioxymethamphetamine damages fine serotonergic fibers and nerve terminals in adult organisms. Developing animals seem to be less susceptible to this effect, possibly due to a lack of drug-induced hyperthermia. We tested this hypothesis by producing hyperthermia in neonatal rats for 2 h after each of twice-daily MDMA (10 mg/kg s.c.) or saline injections administered from postnatal days 1–4. Other drug-treated and control litters were maintained at normothermic temperatures following injection. Changes in forebrain serotonergic innervation were assessed at postnatal day 25 (serotonin transporter binding and serotonin levels), postnatal day 60 (serotonin transporter binding), and 9 months of age (serotonin transporter immunohistochemistry). We also determined the influence of MDMA treatment on apoptotic activity by means of immunohistochemistry for cleaved caspase-3 at postnatal day 5. The hippocampus showed significant MDMA-related reductions in serotonergic markers at postnatal day 25 and postnatal day 60. At 9 months, there was no effect of prior MDMA exposure on serotonin transporter-immunoreactive fiber density in the hippocampus; however, significant reductions in fiber density were observed in two neocortical areas and a hyperinnervation was found in the caudate-putamen and nucleus accumbens shell. MDMA treatment also produced a two-fold increase in the number of cleaved caspase-3-immunoreactive cells in the rostral forebrain and hippocampus. All of these effects were completely independent of pup body temperature. These findings demonstrate that neonatal MDMA administration exposure stimulates apoptotic cell death in various forebrain areas and also leads to a long-term reorganization of the forebrain serotonergic innervation. Consequently, offspring of MDMA-using women may be at heightened risk for abnormal neural and behavioral development.     

Opioid peptides and receptors in joint tissues: study in the rat.

Using immunohistochemical and biochemical techniques, the occurrence of endogenous opioid peptides and their receptors in normal rat bone and joint tissues was investigated. Opioid receptors were detected, quantified, and characterized in homogenates from capsule/synovium and periosteum using radioligand binding assays. Receptor binding of the nonselective opioid [3H]naloxone to tissue homogenates was stereospecific and saturable, showing similar characteristics to that of brain tissue, although with lower binding capacities. By immunohistochemistry, the neuronal occurrence of four different enkephalins was demonstrated in synovium, bone marrow, periosteum, and juxta-articular bone, whereas no neuronal dynorphin immunoreactivity was detected. Double-staining studies disclosed that enkephalins coexisted with substance P in primary afferent fibers. The applied techniques can be used to assess changes in the distribution of endogenous opioids and their receptors in joint tissues in conditions associated with pain and inflammation. The endogenous opioid system now demonstrated might be targeted and exploited therapeutically to obtain peripheral control of symptoms in joint disorders.     

Hydatid cyst of gall bladder.

Gall bladder hydatid cyst is a rare entity. Concurrent occurrence of gall blader hydatid cysts along with liver cysts, especially with the biliary channels clear of cysts, is very rare. We report a 27-year-old man with a gall bladder hydatid cyst that was diagnosed only after opening the resected specimen of the gall bladder.     

Brain abscess in Saudi Arabia

There are significant variations among countries in the incidence of brain abscess. We report here 26 cases of brain abscess treated at the Neurosurgery Department of King Faisal University and Dammam Central Hospital Saudi Arabia over a six year period (1982–1988). This is 2.3% of total admissions to the two neurosurgery departments serving a population of approximately 1.2 million in the same period.Young males were most often affected (M/F ratio 3.3:1; 31% were less than 15 years old, 46% aged between 15–39 years, and 23% older than 40 years). Streptococcus was found to be the most common microorganism (38.4%). Mixed infection was seen in 15.3%, and sterile abscesses were found in 11.5% of the patients after aerobic and anaerobic cultures of the pus. Chronic otitis media and paranasal sinusitis predisposed the patients to abscess formation in 57.6% of the cases. The temporo-parietal area was the commonest site. Epilepsy was a complication in 30.7% of our patients, and the mortality rate was 15.3%.