11C-methionine PET aids localization of microprolactinomas in patients with intolerance or resistance to dopamine agonist therapy.

PURPOSE: To assess the potential for 11C-methionine PET (Met-PET) coregistered with volumetric magnetic resonance imaging (Met-PET/MRCR) to inform clinical decision making in patients with poorly visualized or occult microprolactinomas and dopamine agonist intolerance or resistance. PATIENTS AND METHODS: Thirteen patients with pituitary microprolactinomas, and who were intolerant (n = 11) or resistant (n = 2) to dopamine agonist therapy, were referred to our specialist pituitary centre for Met-PET/MRCR between 2016 and 2020. All patients had persistent hyperprolactinemia and were being considered for surgical intervention, but standard clinical MRI had shown either no visible adenoma or equivocal appearances. RESULTS: In all 13 patients Met-PET/MRCR demonstrated a single focus of avid tracer uptake. This was localized either to the right or left side of the sella in 12 subjects. In one patient, who had previously undergone surgery for a left-sided adenoma, recurrent tumor was unexpectedly identified in the left cavernous sinus. Five patients underwent endoscopic transsphenoidal selective adenomectomy, with subsequent complete remission of hyperprolactinaemia and normalization of other pituitary function; three patients are awaiting surgery. In the patient with inoperable cavernous sinus disease PET-guided stereotactic radiosurgery (SRS) was performed with subsequent near-normalization of serum prolactin. Two patients elected for a further trial of medical therapy, while two declined surgery or radiotherapy and chose to remain off medical treatment. CONCLUSIONS: In patients with dopamine agonist intolerance or resistance, and indeterminate pituitary MRI, molecular (functional) imaging with Met-PET/MRCR can allow precise localization of a microprolactinoma to facilitate selective surgical adenomectomy or SRS.

Colloid cyst of the third cerebral ventricle with an embryological remnant consistent with paraphysis cerebri in an adult human.

The histogenesis of colloid cysts of the third ventricle remains unsettled. Initial theories favored a neuroepithelial (paraphysis, ependyma, choroid plexus) origin and some investigators based on morphologic analysis have offered an alternative endodermal source. We report a case of colloid cyst of the third ventricle arising in association with a remnant which we believe corresponds to the paraphysis cerebri in man.

Spinal metastasis from renal cell carcinoma, 31 years following nephrectomy--case report.

Most renal cell carcinomas recur or metastasize within 2 years. We present a 62-year-old lady with metastatic renal cell carcinoma in the spine, 31 years following nephrectomy of the primary tumor. This is the longest interval between diagnosis of primary renal cell carcinoma and metastasis reported in the literature.

Proximal myotonic myopathy (PROMM) presenting as myotonia during pregnancy.

Proximal myotonic myopathy is a recently described autosomal dominant condition characterized by proximal myopathy, cataracts, intermittent myotonia, and myalgia. We report a further family with this condition. The proband and her two sisters presented with myotonia during pregnancy which resolved after each delivery. Two sisters experienced myalgia between each pregnancy. This relationship between pregnancy and symptom exacerbation suggests an intriguing hormonal influence in PROMM.

Effect of the motilin agonist KC 11458 on gastric emptying in diabetic gastroparesis.

BACKGROUND: KC 11458, a motilin agonist without antibiotic properties, accelerates gastric emptying in animals and healthy humans. AIM: To evaluate the acute effects of KC 11458 on gastric emptying in diabetic gastroparesis. METHODS: Twenty-nine patients (6 type 1 and 23 type 2) with gastroparesis underwent assessments of: (i) gastric emptying of a solid/liquid meal using scintigraphy, (ii) glycaemic control (blood glucose at 0, 30, 60, 90 and 120 min during the gastric emptying measurement) and (iii) upper gastrointestinal and 'meal-related' symptoms (questionnaire), at baseline and after treatment with KC 11458 in a dose of 8 mg t.d.s., or placebo for 8 days. RESULTS: KC 11458 had no statistically significant or clinically relevant effect on gastric emptying of either the solid intragastric retention at 100 min (T100) (P = 0.87) or liquid 50% emptying time (T50) (P = 0.17) components of the meal. KC 11458 slightly worsened (P = 0.04) upper gastrointestinal symptoms when compared with placebo. The magnitude of the change in solid gastric emptying correlated with the change in the blood glucose concentration (r = 0.49; P < 0.05). CONCLUSIONS: KC 11458, in a dose of 8 mg t.d.s. for 8 days, does not accelerate gastric emptying in patients with diabetic gastroparesis. The absence of efficacy may relate to an effect of hyperglycaemia.

Human duodenal phase III migrating motor complex activity is predominantly antegrade, as revealed by high-resolution manometry and colour pressure plots.

Abstract Late phase III migrating motor complex activity has been said to be primarily retroperistaltic but has not been assessed with high resolution manometry or three-dimensional colour pressure plots (pressure/time/distance). Duodenal phase III was examined in healthy young volunteers (seven male, two female) with a 20-lumen assembly. With the most proximal sidehole in the distal antrum, after a 4.5-cm interval 18 sideholes at 1.5-cm intervals spanned the duodenum with a final sidehole 3 cm beyond. Fasting pressures were recorded until phase III occurred. Comparisons were made between proximal (P) and distal (D) duodenum during early (E) (first 0.5-1 min) and late (L) (last 0.5-1 min) phase III. With colour pressure analysis, 121 of 180 pressure wave (PW) sequences were purely antegrade, two purely retrograde and 57 bidirectional. Ten of fifty-seven bidirectional PW sequences were complex, branching to become two separate sequences. Bidirectional sequences occurred more frequently in late than early phase III (L 43 vs. E 14 of 57), but their occurrence did not differ between proximal and distal duodenum (P31 vs. D 24 of 57). Antegrade propagation velocity was faster in late compared with early phase III (L 28.50 vs. E 17.05 mm s(-1); P = 0.006), but did not differ between proximal and distal duodenum. Colour pressure analysis also indicated an intermittent segmental pattern to phase III, with each subject exhibiting a change in velocity or direction, or a relative failure of peristalsis somewhere along the duodenum during part of phase III. Duodenal phase III is not homogenous and, in contrast with previous studies, does not primarily constitute a retroperistaltic pump. Colour pressure analysis is useful in interpreting intraluminal pressure profiles and may improve the sensitivity and specificity of clinical studies.

Arachnoid cyst of the craniocervical junction: case report.

OBJECTIVE AND IMPORTANCE: An arachnoid cyst at the craniocervical junction presenting with obstructive hydrocephalus as a result of blockage of the outflow of the fourth ventricle is described. This is a very rare anatomic site, with only five other cases described in the literature. CLINICAL PRESENTATION: A 37-year-old woman presented with a 9-month history of severe neck pain, persistent vomiting, visual disturbances, and numbness of the nose, cheek, and lips. She had severe bilateral papilledema on ophthalmoscopy. Magnetic resonance imaging revealed a midline cystic lesion extending down to C2. INTERVENTION: The patient underwent posterior fossa craniectomy and excision of the arachnoid cyst. She made a full recovery and was asymptomatic at follow-up examination. CONCLUSION: The symptomatology of these rare craniocervical arachnoid cysts and their development are discussed.

Post-partum retreatment with iridium-192 wire brachytherapy for meningioma recurring in pregnancy.

We report the case history of a female patient who had received radical radiotherapy for a malignant meningioma at the age of 11 years. Thirteen years later, during her first pregnancy, she presented with a recurrence. The tumour was surgically debulked, but complications related to postoperative sepsis, the location of the tumour, and the extent of her previous treatment made the delivery of adjuvant radiotherapy problematic. The tumour bed was treated using an interstitial implant of 192Ir wires to a dose of 60 Gy in 100 hours. The patient remains well with no evidence of tumour recurrence or brain necrosis 2 years later. We discuss the role of female sex hormones in meningioma and the difficulties of radical retreatment of tumours in the central nervous system. The various techniques of brachytherapy in the brain are highlighted. The specific advantages of 192Ir in this patient are discussed.

Schwannomatosis in a child--or early neurofibromatosis type 2.

A case of multiple cervical schwannomas in a five-year-old boy, without other evidence of neurofibromatosis type 2, is described. Schwannomatosis is a disorder characterized by the presence of multiple schwannomas in the absence of neurofibromatosis type 2 that has only been recognized in the last 15 years. The clinical and genetic features of neurofibromatosis types 1 and 2 and schwannomatosis are compared and contrasted. This patient with possible schwannomatosis is presented to illustrate the potential pitfalls of making this diagnosis in the paediatric age group and to increase awareness of the debate on whether this is a distinct entity or a form fruste of neurofibromatosis type 2.

Prospective evaluation of the utilization of aspirin and non-steroidal anti-inflammatory drugs in acute medical admissions.

The aim of this study was to analyse the frequency of aspirin and NSAID usage in 400 unselected patients admitted to the general medical wards through the Accident and Emergency Department. One hundred and twenty patients (30%) reported using NSAIDs (n = 27) or aspirin (n = 99) prior to admission. The median age was 70.5 years (IQR 54-80). Most aspirin use was low dose for cardiovascular prophylaxis and headache. The reported indications for NSAID use were osteoarthritis (n = 12), rheumatoid arthritis (n = 9), gout (n = 3) and psoriatic arthritis (n = 2) and headache (n = 1). Only 23 (19%) patients were aware of the potential side effects of these agents. Co-prescribing with an H2 antagonist (n = 10), proton pump inhibitor (n = 11) or misoprostol (n = 5) was noted in 21.6%. Approximately one third of patients admitted to general medical wards in this study were receiving NSAIDs or Aspirin. The indications for prescribing were appropriate for aspirin. NSAID use was more symptom based and may have been better managed using an analgesic in some cases. Despite the high prevalence of upper gastrointestinal symptoms, co-prescribing of ulcer healing drugs was relatively uncommon.

Papillary tumour of the pineal region.

Papillary tumour of the pineal region (PTPR) is a relatively new and rare pathological entity, which appears to run a spectrum of clinical courses. We add another case with detailed description of the clinical course documented with serial imaging over the total of 7 years. In accordance with previous reports we recommend total surgical resection with subsequent focal radiotherapy. Clinical and radiological follow up of the entire cerebrospinal axis is mandatory.

Basal cell adenocarcinoma of the ethmoid sinuses.

Basal cell adenocarcinoma is a rare and relatively recently characterised malignant salivary gland tumour. It accounts for 5 per cent of parotid gland tumours and 1 per cent of salivary gland malignancies. It is very rarely documented in anatomical sites other than the major salivary glands. Basal cell adenocarcinoma has only been described once before in the ethmoid sinus. We report a case of basal cell adenocarcinoma in the ethmoid sinus, extending into the right orbit and anterior cranial fossa. We describe the clinical aspects of the patient's management and detail the histopathological features of this very rare diagnosis.

Primary cerebral gliosarcoma: report of 17 cases.

A retrospective study of 17 cases of primary cerebral gliosarcoma is presented. These uncommon highly aggressive intracranial neoplasms were seen at the Royal Preston Hospital, between 1973 and 1992. The patients' ages ranged from 21 to 73 years (mean 52), nine were males and eight were females. They presented with signs and symptoms of a rapidly expanding brain tumour. The diagnosis was suspected on radiological findings and confirmed by histological examination. Treatment involved surgical excision in 15 cases and biopsy in two followed by radiotherapy. Chemotherapy was given in three cases. Despite active management, median survival was only 9 months. The clinical, radiological and pathological features of these lesions are highlighted with emphasis on combined histochemistry and immunohistochemistry. The features of gliosarcoma and glioblastoma are compared and contrasted.

Intracranial angiolipoma: report of two cases.

Intracranial tumours composed of adipose tissue and abnormal blood vessels are rarely recognized. We report two such cases; one showed the radiological and histological appearances of a combined lipoma and an arteriovenous malformation, the other the appearances of a lipoma with a prominent capillary component. Computed tomography did not give any indication of their vascular nature, but surgery was complicated by intra- and postoperative haemorrhage.

Fibrous dysplasia of the cervical spine presenting as a pathological fracture.

Fibrous dysplasia (FD) is an uncommon benign fibro-osseous abnormality of bone, of unknown aetiology and equal sex incidence, which is most commonly restricted to one bone (monostotic FD: MFD, 70%). Less commonly multiple bones are affected (polyostotic FD: PFD, 27%). Vertebral involvement is uncommon (4%), but more common with PFD (7 - 24%) than MFD (1%). Of 20 cases of FD involving the cervical spine, only three have represented MFD. Unlike cases associated with PFD, all cases presented with acute neck pain without significant neurological impairment after minor trauma. We present the case of a 35-year-old male with MFD who developed a pathological fracture of C3 following minor trauma. Radiographs showed collapse and typical 'ground glass' lucency of C3. CT revealed replacement of C3 cancellous bone by hypodense tissue extending into the right lateral mass. The cortex was thinned and fractured, and encroached upon the right foramen transversarium and spinal canal. Magnetic resonance imaging demonstrated hypo-intensity on both T1 and T2, with uniform contrast enhancement. Subtotal excision was achieved via an anterior C3 corpectomy, with residual FD left within the right lateral mass. Stability was achieved utilizing an iliac crest strut autograft, C2-4 plate-and-screws, and mobilization in a halo frame for 3 months. At 18 months, he remained asymptomatic and without deficit. Radiography, CT and MRI confirmed graft fusion without FD invasion, but with residual right lateral mass FD unchanged in size.

Familial tumoral calcinosis: association with cerebral and peripheral aneurysm formation.

Two siblings with histologically and radiologically proven tumoral calcinosis presented with cerebral and peripheral aneurysms. The brother died of a ruptured subclavian artery aneurysm after surgical repair of brachial, iliofemoral and coeliac axis aneurysms. Magnetic resonance and catheter angiography in the sister demonstrated marked carotid dysplasia and a left ophthalmic segment aneurysm, not amenable to treatment. We believe this is the first reported case of familial aneurysms in association with tumoral calcinosis.