Drop attack as the only symptom of type 1 Chiari malformation. Illustration by a case

An unusual case of Chiari type I malformation is presented. The only symptom was a "drop attack" during sneezing or coughing. The possible pathogenetic mechanisms are briefly reviewed and the good results of the surgical therapy are stressed.     

Crossed aphasia. An update

The aim of this article is to present an update of a rare but interesting problem: crossed aphasia. This term indicates the presence of aphasia after unilateral cerebral lesion of the hemisphere ipsilateral to the patient's dominant hand. We report two cases, review the most relevant literature, and analyze clinical, neuroanatomical, and neurophysiological aspects, taking in consideration the various interpretations proposed to explain this unusual language disorder.     

Medulloblastoma in children and in adults: a comparative study

Summary We report on the clinical and pathological characters and factors influencing prognosis in a consecutive series of 20 cases of medulloblastoma presenting in childhood and of 20 cases of the tumour presenting in adulthood. The significant differences which emerged were compared with the findings of the largest published series. Medulloblastoma is more often lateral in site and desmoplastic in histology in adults than in children. On our evidence the age at tumour onset not affect survival.     

Incidence and spatial distribution of adult-onset primary malignant and other central nervous system tumors in Southern Sardinia,Italy

Neurological Sciences - To study for the first time the incidence of adult-onset CNS tumors in Southern Sardinia, Italy. Clinical records of patients > 18 years old who...     

Phase II trial of weekly intravenous gemcitabine administration with interferon and interleukin-2 immunotherapy for metastatic renal cell cancer

PURPOSE: Since metastatic renal cell carcinoma has a poor prognosis and treatment strategies, including hormone therapy, chemotherapy and immunotherapy, have little impact on the quality of life and global survival statistics, new interest has recently focused on the combination of immuno-chemotherapy using pyrimidine analogues, such as gemcitabine. MATERIALS AND METHODS: In a phase II study 16 patients with metastatic renal cell carcinoma were treated with 1,000 mg./m. gemcitabine intravenously on days 1, 8, 15 and 28 for 6 months, 3 MU (1 MU = 1 x 10(6) IU) interferon (IFN)-alpha intramuscularly 3 times a week and 4.5 million IU interleukin (IL)-2 subcutaneously daily for 5 days a week for 2 consecutive weeks every month for 6 months. Responding and nonprogressing cases were maintained on immunotherapy consisting of IFN-alpha and IL-2 for further 6 months. RESULTS: In 15 evaluable patients overall response rate (1 complete response plus 3 partial response) was 28% while stable disease was achieved in 7 (47%). Median survival duration was 20 months (range, 9 to 26+) and median time to tumor progression was 14 months (6 to 26+). The complete response lasted 24+ months and partial response lasted 16 months. The regimen was well tolerated with only 1 case of neutropenia (WHO grade 3), while anorexia, fatigue and flu-like symptoms were the most common toxicity problems but were never greater than grade 2. CONCLUSIONS: Despite the small sample size, this study demonstrates that gemcitabine combined with standard doses of IFN-alpha and low doses of IL-2 is effective treatment for metastatic renal cell carcinoma. This biotherapy was well tolerated and resulted in an optimum objective response and relatively long-term survival.     

Malignant monstrocellular brain tumours

A series of 42 cases of monstrocellular brain tumour (MBT) who received surgery over a 34 year period is reviewed with the aim of gaining more understanding of the biology of this oncotype. A case of benign pleomorphic xanthoastrocytoma was identified among the cases and is discussed in another paper. In this series, as in most others taken from the literature, MBTs were more frequent in young subjects (55% were under 50 years of age and 17% were children) and presented as a superficially located (63%), often sharply circumscribed (42%) mass. The mean survival time for 24 patients treated by surgery and radiotherapy (RT) was 57 weeks compared to 32 weeks for 12 treated by surgery alone (p less than 0.02). Patients younger and older than 50 years showed the same survival and the slightly better course of children did not reach statistical significance. Besides postoperative RT, the only other factor that showed a significantly positive influence on survival was the presence of a definite lymphocytic infiltration (LI) in the tumour (p less than 0.05). This result confirms our previous study on the prognostic influence of LI in glioblastoma (GB). Actually, we noted that the prolonged survival of both MBT and GB patients exhibiting a definite LI (67% and 11.5%, respectively, in the two series) was strikingly similar. We conclude that MBT is a peculiar oncotype with a probably better prognosis than GB in the majority of cases. Lymphocytes seem to play a major prognostic role and giant-monstrous cells are indirectly implicated, reasonably enhancing the host's immune response by magnifying the antigenic stimulus.     

Chronic subdural hematoma: results of a homogeneous series of 159 patients operated on by residents

AIMS: A series of cases with chronic subdural hematoma operated upon by residents in neurosurgery is analysed. MATERIALS AND METHODS: 159 patients treated between 1998 and 2001 were included in the study. Mean age was 76.4 years and male/female ratio was 1.7/1. The patients were classified both on admission and at discharge according to the Markwalder scale. The standard operative procedure consisted of an enlarged single burr-hole, rinsing the subdural space with iso-osmotic saline solution and insertion of a subdural drain. CONCLUSION: In CSDH, operation by the residents is safe and the results are comparable to those of the major series of the literature as the surgical procedure is standardized.     

Giant stone in a complete duplex ureter with ureterocele. A case report

We describe a case of a monolateral duplex system and a ureterocele containing a gigantic stone in a 65-year-old woman who presented with pyelonephritis without any previous history of urinary tract infections or stone disease. Stone removal and double left ureteroneocystostomy with plastic widening of a narrowed obstructive side were performed. The ureteral stone measured 10.5 cm in greatest diameter, weighed 85 g and contained calcium oxalates and phosphates. Three months after surgery, radiology (intravenous urography and cystography) showed left unobstructed upper and lower urinary tracts and the absence of vesicoureteral reflux. Urine culture was negative 3, 6 and 9 months after surgery.     

Correlation between GABA(A) receptor density and vagus nerve stimulation in individuals with drug-resistant partial epilepsy

Vagus nerve stimulation (VNS) is an important option for the treatment of drug-resistant epilepsy. Through delivery of a battery-supplied intermittent current, VNS protects against seizure development in a manner that correlates experimentally with electrophysiological modifications. However, the mechanism by which VNS inhibits seizures in humans remains unclear. The impairment of gamma-aminobutyric acid (GABA)-mediated neuronal inhibition associated with epilepsy has suggested that GABA(A) receptors might contribute to the therapeutic efficacy of VNS. We have now applied single photon emission computed tomography (SPECT) with the benzodiazepine receptor inverse agonist [123I]iomazenil to examine cortical GABA(A) receptor density (GRD) before and 1 year after implantation of a VNS device in 10 subjects with drug-resistant partial epilepsy. VNS therapeutic responses resulted significantly correlated with the normalization of GRD. Moreover, a comparable control group, scheduled for a possible VNS implant, failed to show significant GRD variations after 1 year of a stable anti-epileptic treatment. These results suggest that VNS may modulate the cortical excitability of brain areas associated with epileptogenesis and that GABA(A) receptor plasticity contributes to this effect.