Peripheral Primitive Neuroectodermal Tumour (pPNET) in the cervical spine.

Primary spinal primitive neuroectodermal tumours are rare. We present a 45-year-old man with a peripheral primitive neuroectodermal tumour arising in the cervical spine. We believe this to be the first report of this type of tumour in the cervical spine.

Intracranial and spinal tuberculosis requiring neurosurgical intervention. The Wellington Hospital experience 1998-2001.

AIMS: To describe the Wellington Neurosurgical Unit's recent experience of managing tuberculosis between January 1998 and January 2001. METHODS: Patients with microbiologically confirmed tuberculosis of the central nervous system and whose management included surgery are described. Personal recall and review of the hospital records were used to extract relevant data. RESULTS: Five patients were identified. As well as involvement of the brain parenchyma, meninges, spinal cord or spinal column, all had evidence of tuberculosis elsewhere. All but one patient deteriorated neurologically after being started on antituberculous chemotherapy. CONCLUSIONS: The number of patients presenting with neurotuberculosis appears to have increased recently in the Wellington region. The high proportion of paradoxical progression in our series is unusual. Neurosurgical intervention may be required for diagnosis, to treat hydrocephalus, or to relieve mass effect. Management is prolonged and often complex, and close co-operation is required between the neurosurgical team and a physician experienced in the management of tuberculosis.

Gene structure, chromosomal localization, and expression pattern of Capn12, a new member of the calpain large subunit gene family.

We report the identification of mouse Capn12, a new member of the calpain large subunit gene family. It possesses potential protease and calcium-binding domains, features typical of the classical calpains. In situ hybridization and Northern blot analysis demonstrate that during the anagen phase of the hair cycle the cortex of the hair follicle is the major expression site of Capn12. The gene was sequenced in its entirety and consists of 21 exons spanning 13 kb with an exon-intron structure typical of the calpain gene family. The last exon of the mouse Actn4 gene overlaps the 3' end of Capn12 but in the opposite orientation. This overlap between the two genes is conserved in the human genome. Three versions of the Capn12 mRNA transcript were identified. They occur as a result of alternative splicing, and two of these encode a protein lacking the C-terminal calmodulin-like domain. Radiation hybrid mapping localized Capn12 to mouse chromosome 7, closely linked to a marker positioned at 10.4 cM. Refined mapping of Capn5, also previously localized to chromosome 7, indicated that it was not closely linked to Capn12, mapping tightly linked to a marker positioned at 48.5 cM.

Complications of trans-sphenoidal surgery: the Wellington experience.

BACKGROUND: All patients who underwent trans-sphenoidal surgery between January 1984 and December 1998 were reviewed to assess morbidity resulting from this operation. METHODS: There were 185 operations on 165 patients. The operative approach was sublabial in 80 cases and transnasal in 105. One surgeon (VB) performed the vast majority of operations. RESULTS: Complications included nasal perforation (7.6%), transient diabetes insipidus (4.9%), permanent diabetes insipidus (3.8%), cerebrospinal fluid fistula (4.3%), donor site haematoma (2.2%) and residual tumour haemorrhage (1.6%) causing ophthalmoplegia (1.1%) and loss of vision (1.1%). Other complications included epistaxis (1.1%), meningitis (0.5%) and sinusitis (0.5%). Injury to the anterior superior alveolar nerve also occurred in the sublabial approach in 6.3% of patients. There were no perioperative deaths. CONCLUSIONS: There is a small but significant risk of a number of complications that should be considered for informed consent of this procedure.

Non-functioning pituitary adenomas: indications for postoperative radiotherapy.

To determine the indications for postoperative radiotherapy after surgical resection of a nonfunctioning pituitary macroadenoma. A retrospective chart review of 72 patients with histologically proven chromophobe adenoma who presented for pituitary surgery between January 1985 and June 1998, with a minimum follow-up period of 12 months. The study endpoint was tumour recurrence or progression detected either by routine follow-up imaging or by clinical progression with subsequent confirmation by imaging. A proportional hazards model was used to determine independent prognostic factors. Mean follow-up was 64 months. In the radiotherapy group 13 of 50 recurred (or progressed) (26%), while in the nonradiotherapy group 10 of 22 recurred (46%), logrank test, P = 0.025. In patients assessed as having complete excision of tumour (n = 20) only two recurred (10%), both in patients without radiotherapy. No further treatment has been required in either case to date. In patients with residual tumour (n = 52), 41 had radiotherapy with 13 recurrences (32%), while 11 patients had no radiotherapy with eight subsequent recurrences (73%); logrank test, P = 0.007. Further treatment has been required in the majority of these cases. Cox's proportional hazards model analysis showed that only complete tumour removal and postoperative radiotherapy were independent favourable prognostic factors. The goal of surgery should be complete surgical excision where possible. The risk of recurrence in patients with no residual tumour on postoperative imaging is low enough to justify withholding routine postoperative radiotherapy in this group. In patients with residual tumour, conventional external beam radiotherapy administered within 12 months of surgery is effective at reducing recurrence or progression.

Persistent facial pain following microvascular decompression of the trigeminal nerve.

Pain in the face following microvascular decompression (MVD) can be due to persisting trigeminal neuralgia (TGN) or a variety of other facial pain syndromes. If magnetic resonance tomoangiography (MRTA) indicates continuing vascular compression and the patient has true persistent TGN, then the patient can be relieved of pain by repeating the MVD. When the MRTA is negative for continuing compression alternative techniques may be employed; section of the nerve at the pons may be the treatment of choice for true persistent TGN in the absence of neurovascular compression. In some cases the pain is dysaesthetic in nature and not persistent TGN. This is always associated with previous destructive lesions to the nerve, usually radio-frequency thermocoagulation. When this component to the pain is recognized pre-operatively the patient must be warned not to expect relief of this same component of the pain from MVD. When it is not possible to classify the facial pain clinically, improvement does not occur following MVD even when there is clear evidence of vascular compression on MRTA.

Haemangiopericytoma presenting as recurrent intracranial haemorrhage.

A 17-year-old girl presented with intracranial haemorrhage from a malignant haemangiopericytoma. During the course of treatment with surgery, radiotherapy and chemotherapy there were three further episodes of intracranial haemorrhage over a 9-month period, culminating in death from the effects of raised intracranial pressure. The pathology of haemangiopericytoma is reviewed with particular reference to factors predisposing to haemorrhage.

Cysteine-rich regions of protein kinase Cdelta are functionally non-equivalent. Differences between cysteine-rich regions of non-calcium-dependent protein kinase Cdelta and calcium-dependent protein kinase Cgamma.

Regulatory domain elements of the non-calcium-dependent protein kinase Cdelta (nPKCdelta), including either or both of the cysteine-rich regions Cys1(delta) and Cys2(delta), were expressed as fusion proteins with glutathione-S-transferase and characterized using liposomal or mixed micellar phorbol ester binding assays. Fusion proteins containing Cys2(delta) bound phorbol-12,13-dibutyrate (PDBu) efficiently in the assay employing phosphatidylserine (PS) vesicles, while no significant binding was seen for proteins containing only Cys1(delta). Likewise, in mixed micellar assays, fusion proteins with Cys2(delta) bound PDBu with high affinity (Kd: 14-37 nM) and to significant stoichiometric levels (0.23-0.66 mol/mol), but no binding could be detected for proteins with Cys1(delta) only. The PS dependence of PDBu binding to Cys2(delta) was highly cooperative with Hill numbers lying in the range of 2.5-5.2. These results demonstrate the presence of striking functional differences between the cysteine-rich regions of nPKCdelta and the calcium-dependent isoform, cPKCgamma, where both cysteine-rich regions represent functional PDBu binding elements.