Recurrent cryptic vascular malformation associated with a developmental venous anomaly.

Abnormal venous drainage patterns, such as developmental venous anomalies (DVAs), are frequent findings neighbouring cryptic vascular malformations (CVMs). Although the clinical relevance of DVAs remains controversial, increasing attention has been focused on the possible importance of venous outflow disturbance and venous hypertension in DVAs for the development of CVMs. We present the case of a 32-year-old man with dysphasic seizures symptomatic for recurrence and rebleeding of a CVM of the left angulare gyrus, which drained entirely into a large DVA. A cavernoma had been surgically removed 1 year before, while the associated DVA had been left in place. In the following second surgical procedure, the new malformation, which was histologically verified as arteriovenous angioma (AVM), was removed and the DVA was occluded for the length of the angioma. Postoperative course of the patient was unremarkable, the patient is seizure free and neurologically intact. This first report of such a sequence of events raises important questions regarding the association of various vascular malformations, regarding transitional and hybrid forms, as well as the role of anomalous venous drainage in their pathophysiology and probably in their development. Our case and selected cases in literature suggest that venous hypertension in a DVA might not be just a coincidential finding, but sometimes can induce or influence the development and recurrence of associated vascular lesions. The developmental interrelationship, the potential mechanisms for this association and the implications of similar cases reported in pertinent literature are discussed.

Advanced surgical approach for selective amygdalohippocampectomy through neuronavigation.

OBJECTIVE: Selective removal of the mesiobasal temporal structures through the transsylvian approach was introduced by Yasargil and Wieser in 1982. This alternative to standard temporal lobectomy provides excellent outcomes for seizure control. Basic actions in the transsylvian fissure exposure mainly serve to orient the surgeon, and they carry the risk of vasospasm and vessel damage. The aim of our study was to reduce landmark-guided surgery steps through neuronavigation. METHODS: During a 14-month period, 16 selective amygdalohippocampectomies were performed with the aid of the SMN (Carl Zeiss, Inc., Thornwood, NY) or StealthStation (Sofamor Danek, Memphis, TN) optically guided systems. We added safety procedures to the operation (including intraoperative rereferencing, obtaining additional bony reference points before craniotomy, performing a small craniotomy and making an accurate dural incision, and using contrast medium for vessel visualization) to develop a method that relies on navigational systems without further orientation by anatomic landmarks. RESULTS: Originally, performing an amygdalohippocampectomy required exposing the sylvian fissure from the carotid bifurcation to 2 cm beyond the middle cerebral artery bifurcation, which exposed one-third of the insula. By determining the entry point at the limen insulae and the target at the tip of the temporal horn, the mandatory extent of the opening to the sylvian fissure can be projected. Therefore, the exposure of the fissure can be limited to exactly the extent required for the transventricular approach through the uncinate fasciculus. CONCLUSION: Computer-assisted surgery is an effective tool in eliminating the exposure of anatomic landmarks in selective amygdalohippocampectomy. This modification combines the precision of targeting with minimal cortical and vessel traumatization.

Non-HTLV-I associated pleomorphic T-cell lymphoma of the brain mimicking post-vaccinal acute inflammatory demyelination.

Two weeks after vaccination against tick-borne encephalitis (TBE), a 57-year-old female suddenly developed mental confusion and hemiparesis of the left side. Cranial MRI demonstrated extensive bilateral lesions in the fronto-parietal white matter of both hemispheres, suggesting an acute inflammatory demyelinating disease following vaccination. Despite administration of high-dose corticosteroids, the patient died 3 weeks after onset of neurological symptoms. Autopsy revealed diffuse infiltrates of a primary cerebral pleomorphic T-cell lymphoma of medium and large cell type. PCR on brain tissue for HTLV-I and serology for anti-HTLV-I antibodies in CSF and serum were negative; immunocytochemistry on brain tissue did not detect EBV-related antigen. This is the first recorded observation of a diffusely infiltrating primary central nervous system T-cell lymphoma, clinically and radiologically mimicking a fatal acute inflammatory demyelinating complication after vaccination.

Lyme borreliosis in children. A controlled clinical study based on ELISA values.

A total of 27 children with clinical symptoms indicative of Lyme borreliosis are described, 21 of which were seropositive. CNS symptoms were found in 17 of the seropositive children (81%). Of these 21, 7 were CSF negative. Another 3 (with Bell's palsy and/or aseptic meningitis) were initially CSF negative but developed specific CSF titres 80, 65 and 120 days after the first lumbar puncture, respectively. Thus, seropositive children with aseptic meningitis and without initial signs of an infectious aetiology should be checked for a neuroborreliosis even when CSF negative in the first lumbar puncture. Antibiotic therapy undertaken in 26 children showed clinical recovery (Sodium penicillin, 300,000-500,000 units/kg per day for 14 days). One developed residual hypoacusis. Comparison of enzyme-linked immunosorbent assay (ELISA) IgG values from 27 cases with those of 30 healthy controls showed that elevated ELISA titres are a good indication of the disease. However, Lyme borreliosis can only be diagnosed correctly if the clinical symptoms conform with specific titres.