The syndrome of normal-pressure hydrocephalus (NPH) remains a diagnostic and therapeutic challenge, especially as many patients do not display the classical clinical and neuroimaging patterns of NPH, thus questioning the usefulness of a shunt. Gait impairment remains the cardinal symptom, while mental deterioration may be subtle and even unrecognized. NPH is rarely the cause of severe dementia, and substantial improvement in NPH-related mental deterioration is limited to 30-40% of shunted patients. Many ancillary investigations have been described that can increase the probability of selecting the appropriate candidates for a shunt. The reliability and reproducibility of these tests are limited. Unfortunately, the best predictive tests are technically complex and are used only in a few specialized centers. The best management is still to adhere to strict clinical and magnetic resonance imaging criteria and to rely on a positive - but not negative - CSF tap test and the occurrence of B-waves during at least 50% of the continuous intracranial pressure recording time, when this procedure is available.
We performed a multicenter retrospective study in 166 consecutive patients shunted for presumed normal-pressure hydrocephalus (NPH) in the four neurosurgical departments of Amsterdam. Overall improvement occurred in 36%, substantial improvement in 21%. In the subgroup of idiopathic NPH (N = 127), marked improvement was only 15%. The incidence of shunt-responsive NPH in our area was 2.2/million/year. The rate of severe and moderate shunt-related complications was 28%, leading to death or severe residual morbidity in 7%. The substantial benefit/serious harm ration in the whole group was only three (21%/7%), decreasing to 1.7 in idiopathic NPH. By excluding patients at high surgical risk, this ratio might have risen to 10 in the whole group and to six in idiopathic NPH. Our experience is much less favorable than that encountered in the literature, reporting overall improvement in 74% and marked improvement in 55% of the shunted patients. We conclude that NPH is probably a very rare and still overdiagnosed syndrome and that the overall morbidity rate for each patient demonstrating meaningful improvement is high.
Forty-eight patients with noncancer neuropathic pain who had participated in a randomized controlled trial with intravenous fentanyl (FENiv) infusions received prolonged transdermal fentanyl (FENtd) in an open prospective study. Pain relief, side effects, tolerance, psychological dependence, mood changes, and quality of life were evaluated. The value of clinical baseline characteristics and the response to FENiv also was evaluated in terms of the outcome with long-term FENtd. Eighteen patients stopped prematurely because of insufficient pain relief, side effects, or both. Among the remaining 30 patients completing the 12-week dose titration protocol, pain relief was substantial in 13 and moderate in five. Quality of life improved (23%, P < 0.01). Psychological dependence or the induction of depression was not observed. In only one patient did tolerance emerge. There was a significant positive correlation between the pain relief obtained with FENiv and that with prolonged FENtd (r = 0.59, P < 0.0001). We conclude that (1) long-term transdermal fentanyl may be effective in noncancer neuropathic pain without clinically significant management problems and (2) A FENiv-test may assist in selecting neuropathic pain patients who might benefit from prolonged treatment with FENtd.
We describe a 29-year-old woman who presented with progressive neck pain, sensory deficit and weakness in both arms. Magnetic resonance imaging (MRI) of the cervical spine revealed an extramedullary tumor with severe spinal cord compression. During surgery an intradural extramedullary tumor was found. Further imaging showed a second lumbar spinal tumor. Microscopy of both tumors showed that both tumors were anaplastic ependymomas, which almost never present as extramedullary tumors. Two years after surgery, an intracranial extracerebral metastasis was found, without evidence of spinal recurrence.
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