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pathway and the ascending reticular activating system may also be injured in some cases of severe and longstanding iNPH (1).
pathologies, which made possible a correct diagnosis even in complex and borderline cases.
The exact incidence of this association is not clear. Some investigators do not even report such a relationship, whereas others describe parkinsonian symptoms in patients with iNPH in a range between 11% and 70% of cases (1). This condition is probably underestimated as the diagnosis is sometimes difficult and requires two skilled specialists.
Our preliminary results suggest that patients affected by iNPH associated with parkinsonism may benefit from VP shunt plus oral dopamine therapy. As soon as the trial will be completed, we will share more exhaustive results with the scientific community.
We designed a study aiming at clarifying the actual incidence of this association and comparing the clinical outcome between VP shunt alone and VP shunt plus oral dopaminergic therapy for patients affected by iNPH associated with parkinsonism. The outcome is evaluated through the revised Japanese Normal Pressure Hydrocephalus grading scale- (JNPHGSR) and the third section (motor examination) of the Unified Parkinson’s Disease Rating Scale (UPDRS). The protocol has strict inclusion criteria: patients between ages 50 and 80 years, whose symptoms began at least 6 months before diagnosis, and having a diagnosis of iNPH associated with parkinsonism, made by a neurologist and a neurosurgeon. Diagnosis and enrolment are done in several steps: clinical and radiologic evaluation, invasive tests (cerebrospinal fluid tap test and determination of outflow resistance), levodopa test. neurocognitive tests (e.g., Mini Mental State Examination, Frontal Assessment Battery, Milan Overall Dementia Assessment, Tinetti’s scale) and a 123I-ioflupane cerebral singlephoton emission computed tomography (DaTSCAN). After enrollment, patients were randomized to group A (adjustable VP shunt plus levodopa/carbidopa oral therapy) or group B (surgical operation with adjustable VP shunt alone). During VP shunt operation, cerebrospinal fluid was sent to the laboratory for t-tau, p-tau-181, 14.3.3, and Ab42 protein analyses. Patients were then followed for 12 months, with visits (neurological and neurosurgical) every 3 months during which valve or levodopa/ carbidopa dosage adjustments were possible. The primary end point, in terms of outcome, was the percentage of patients who show an improvement more than 30% in the third section of the UPDRS. Until present (18 months), 34 patients were screened: A total of 20 patients (58%) (12 women, 8 men;, mean age, 73.2 years, range, 61e80 years) met the inclusion criteria and were enrolled in the study. All patients underwent a DaTSCAN before the operation and this revealed some striatal dopaminergic deficit in 9 patients (45%). Ten patients were randomized to group A and 10 to group B. A total of 15/20 patients (75% overall, 8 from group A and 7 from group B) showed an improvement of the JNPHGRS score; 8/ 10 patients of group A and 5/10 patients of group B showed an improvement more than 30% in the third section of the UPDRS score. In this relatively small series an association between iNPH and parkinsonism was present in more than half of patients. Several reasons could have contributed to these data; above all, the fact that the Fondazione IRCCS Istituto Besta, Italy, is a referral center for movement disorders and, consequently, many patients with parkinsonian-like symptoms are referred to this center. We want to stress the need for a close cooperation between the neurosurgeon and the neurologist in dealing with these
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Morgan Broggi1, Luigi Romito2, Veronica Redaelli2, Angelo Franzini1 From the Departments of 1Neurosurgery and 2Neurology, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milano, Italy To whom correspondence should be addressed: Morgan Broggi, M.D., Ph.D. [E-mail:
[email protected]] Published online 20 August 2014; http://dx.doi.org/10.1016/j.wneu.2014.09.020.
REFERENCES 1. Akiguchi I, Ishii M, Watanabe Y, Watanabe T, Kawasaki T, Yagi H, Shiino A, Shirakashi Y, Kawamoto Y: Shunt-responsive parkinsonism and reversible white matter lesions in patients with idiopathic NPH. J Neurol 255:1392-1399, 2008. 2. Scafato E, Gandin C, Galluzzo L, Ghirini S, Cacciatore F, Capurso A, Solfrizzi V, Panza F, Cocchi A, Consoli D, Enzi G, Frisoni GB, Gandolfo C, Giampaoli S, Inzitari D, Maggi S, Crepaldi G, Mariotti S, Mecocci P, Motta M, Negrini R, Postacchini D, Rengo F, Farchi G, ; I.PR.E.A. Working Group (Italian PRoject on Epidemiology of Alzheimer’s disease): Prevalence of aging-associated cognitive decline in an Italian elderly population: results from cross-sectional phase of Italian PRoject on Epidemiology of Alzheimer’s disease (IPREA). Aging Clin Exp Res 22:440-449, 2010.
Gullain-Barré Syndrome After Posterior Fossa Tumor Surgery LETTER: syndrome (GBS) is an acute demyelinating polG uillain-Barré yneuropathy with an incidence of about 0.4e2.3 per 100,000 person-years. It is disorder that occurs after an infection, and frequently identified infectious agents include Campylobacter jejuni, cytomegalovirus, Mycoplasma pneumoniae, Epstein-Barr virus, and influenza virus. Many reports have documented the occurrence of GBS shortly after vaccinations, operations, or stressful events. The infectious agents that trigger GBS have epitopes that mimic the epitopes on peripheral nerves (2, 5). However, the immunopathogenesis of the disease remains uncertain. The clinical course of GBS includes rapidly progressive weakness over a period that may range from days to several weeks. This phase is followed by a usually much slower and variable recovery phase. About 25% of patients with GBS may need artificial ventilation. The cause is predominantly due to weakness of the respiratory muscles. Despite standard treatment with intravenous immunoglobulin or plasma exchange treatment, 20% of severely affected patients remain unable to walk after 6 months (5). GBS has not been reported after intracranial surgery. We describe a case of GBS occurring in a 45-year-old woman after surgery for a posterior fossa tumor. A 45-year-old woman presented with complaints of giddiness and headache for 1 month. She had mild left-sided cerebellar signs and papilledema. Magnetic resonance imaging of the brain revealed a left-sided cerebellopontine angle meningioma with mass effect on the fourth ventricle (Figure 1). She underwent a left
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showed improvement after 6 cycles of plasmapheresis. She was discharged 4 weeks postoperatively with residual quadriparesis. The overall complication rate after posterior fossa surgery is about 31.8%, of which about 5% of the complications are related specifically to surgeries involving cerebellum or brainstem (1). Complications include cerebellar edema, hematoma, hydrocephalus, and vascular complications arising from arterial or venous insults. Delayed neurologic deterioration after surgery for a posterior fossa tumor requires a meticulous work-up to evaluate for infection and brainstem dysfunction from cerebellar edema, hydrocephalus, or venous infarcts. Delay in diagnosis and treatment leads to undesirable morbidity and can be potentially fatal. Figure 1. Contrast-enhanced magnetic resonance imaging brain coronal images with gadolinium showing a homogeneously enhancing lesion in the left cerebellopontine angle with attachment to the tentorium and the dura mater over the petrous bone.
retromastoid suboccipital craniectomy and excision of the tumor. Perioperative and intraoperative periods were uneventful except for ataxia and left facial nerve paresis. On postoperative day 11, she developed rapidly progressing weakness of her upper and lower limbs and respiratory distress. She became unconscious and unresponsive with small reacting pupils. Physical examination revealed flaccid areflexic quadriparesis with poor respiratory efforts. We suspected delayed complications of posterior fossa surgery, such as cerebellar edema, hydrocephalus, or venous thrombosis involving the superior petrosal vein. She was intubated and resuscitated. Magnetic resonance imaging of the brain ruled out the above-mentioned complications (Figure 2). She regained consciousness after a few hours of resuscitation and correction of hypoxia. However, only vertical eye movements were possible, and all other motor activity could not be performed. Nerve conduction studies revealed a severe axonal-type polyradiculoneuropathy, and GBS was considered a diagnostic possibility. Cerebrospinal fluid analysis revealed albumin cytologic dissociation. The patient
Figure 2. T2-weighted axial section of posterior fossa showing postoperative changes including normal fourth ventricle without edema or infarcts.
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The incidence of GBS after surgical excision of a posterior fossa tumor has not been reported in the literature. For this reason, we did not expect or accept the diagnosis of GBS until all possible surgical complications were ruled out and investigations to establish the diagnosis were complete. Only a few studies have included surgery as a potential trigger for GBS; Gensicke et al. (2) reported an incidence of postsurgical GBS of 9.5%. The attributable risk was reported as 4.1 GBS cases per 100,000 surgeries, which means the relative risk of developing GBS during the 6-week postsurgery period was 13.1 times higher than the normal incidence in the study population. All the cases reported in the study by Gensicke et al. (2) involved patients who underwent orthopedic procedures or gastrointestinal surgeries within 6 weeks before the onset of GBS. There are other reports of GBS after cardiac surgeries such as cardiopulmonary or coronary artery bypass surgeries; none of them reported a neurologic surgery antecedent to GBS (3, 4). The postulated etiology or triggers causing GBS include viral and bacterial infections and de novo cases following surgery. An immune-mediated response is common to all etiologies. The pathophysiology of GBS occurring after surgery is presumed to be surgical stresseinduced activation of the neuroendocrine stress axis and cell-mediated immunosuppression, which may promote the infections leading to cross-reactive antibodies (5). In this regard, we presume that administration of exogenous steroids in patients undergoing surgery for an intracranial tumor is possibly an added risk factor for cellular immunosuppression. However, among numerous patients being treated with steroids for a surgically or medically managed neurologic condition, there is no report of any increased incidence of GBS among these patients. Although immunosuppression could be considered as a trigger for the onset of GBS, immunomodulation therapy with plasmapheresis or intravenous immunoglobulin is the standard treatment protocol for all cases of GBS regardless of etiology (5). In conclusion, methodical evaluation and exclusion of the common complications of posterior fossa surgery are mandatory when clinical deterioration is observed in a postoperative patient. We do not have any evidence to hypothesize the possible causes for GBS in this patient following posterior fossa surgery, and further studies are needed. The incidence of GBS after surgery is very rare, although surgical stress could be considered as a triggering factor for GBS.
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Santhanam Rengarajan1, Manjesh Rathi2, Suresh Kumar2, Preetham Chennareddy2, Karthikeyan Napa1 From the Departments of 1Neurosurgery and 2Neurology, Sree Balaji Medical College & Hospital, Bharath University, Chennai, India To whom correspondence should be addressed: Santhanam Rengarajan, M.Ch. [E-mail:
[email protected]] Published online 6 September 2014; http://dx.doi.org/10.1016/j.wneu.2014.09.003.
REFERENCES 1. Dubey A, Sung WS, Shaya M, Patwardhan R, Willis B, Smith D, Nanda A: Complications of posterior cranial fossa surgery—an institutional experience of 500 patients. Surg Neurol 72:369-375, 2009. 2. Gensicke H, Datta AN, Dill P, Schindler C, Fischer D: Increased incidence of Guillain-Barre syndrome after surgery. Eur J Neurol 19:1239-1244, 2012. 3. Hogan JC, Briggs TP, Oldershaw PJ: Guillain-Barre syndrome following cardiopulmonary bypass. Int J Cardiol 35:427-428, 1992. 4. Renlund DG, Hanley DF, Traill TA: Guillain-Barre syndrome following coronary artery bypass surgery. Am Heart J 113:844-845, 1987. 5. Vucic S, Kiernan MC, Cornblath DR: Gullain Barre syndrome: an update. J Clin Neurosci 16:733-741, 2009.
Hemicraniectomy for Older Patients in Low-Income Countries? alignant middle cerebral artery infarction represents a devastating type of ischemic stroke associated with fatal outcome or long-term disability. Only early decompressive hemicraniectomy (DHC) has been shown to be effective in lowering the rate of mortality and improving outcomes, especially in younger patients. Recently, Jüttler et al. (4) reported the results of DHC in older patients with extensive middle-cerebral artery stroke in a randomized trial. They demonstrated a survival and functional benefit of this procedure in patients older than 61 years of age. As the authors note, there has been significant controversy surrounding this procedure, some of it directed at the prospect of increasing survival in patients who will ultimately suffer from severe disability and possibly reduced quality of life. Although this study answered some important questions, at the same time it raises questions about whether survival is necessarily the best outcome for older patients who have suffered a large stroke and who will suffer from severe disability for the rest of their lives. A year after stroke, a significant proportion of patients in the hemicraniectomy group and patients treated with standard care were unable to walk or attend to own bodily needs without assistance. Although
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Key words Advanced age - Cost - Hemicraniectomy - Low-income countries - Outcome - Stroke -
Abbreviations and Acronyms DHC: Decompressive hemicraniectomy mRs: Modified Rankin scale
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hemicraniectomy is a life-saving procedure, we believe its international applicability could still be questioned, particularly in low-income countries. In this issue of WORLD NEUROSURGERY, Suyama et al. (6) analyzed the clinical characteristics of patients undergoing DHC for malignant middle cerebral artery stroke in Japanese neurosurgical departments. The majority of their patients (a little more than 80%) were older than 60 years of age, and age was not found to be an important prognostic factor. In their 3month follow-up, only 5.2% had modified Rankin scale (mRs) scores less than 3, 49.8% had mRs 4 5, and 45.1% patients had died. These data are somewhat different from Jüttler et al’s. They reported a mortality of 33% in patients undergoing DHC, and 3% of those patients had a mRs of 3, whereas 27% had mRs 4 5. Although Suyama et al. reported retrospective data, their outcome assessment was performed at 3 months, whereas Jüttler et al performed it at 6 months in. Interestingly, the point where these 2 studies seem to agree is in the low proportion of patients resulting with good functional outcome (mRs <3 in 5.2% and 3%, respectively). This finding contrasts with a recent systematic review including 459 patients that showed that 39% of patients (of all ages) undergoing DHC for malignant middle cerebral artery infarction achieved good functional outcomes, with good quality of life (quality of life was also lower for those aged >60 years of age) (8). Little attention has been paid to the implications of this procedure in third-world and low-income countries. Both Germany (Jüttler et al.) and Japan (Suyama et al.) are countries that share high economic and health-related indices, and even then their results of DCH outcomes appear to vary. It is true that costeffectiveness analyses should not independently decide the value of a therapeutic intervention. Nonetheless, Hofmeijer et al. for the HAMLET (Hemicraniectomy After Middle Cerebral Artery Infarction with Life-threatening Edema Trial) investigators recently reported an incremental costs of V127,000 per qualityadjusted life year gained after hemicraniectomy in young patients with malignant stroke (3), representing a cost 11 times the gross domestic product per capita for a middle-income country like Mexico, well above what would normally be considered as costeffective. The follow-up for up to 12 months also implies that severely disabled patients (60% with a mRs score 4 or 5) received long-term institutionalized care and rehabilitation (but this information is lacking), which in the context of a clinical trial and in a high-income country would be expected to be offered without cost, but in most middle- and low-income countries would represent additional out-of pocket expenditures for the patients, if it could be afforded at all. Out-of-pocket expenditures and days without income increase substantially in the families of hospitalized patients older than 60 years of age in Mexico (5), even in patients with social security (statedependent health insurance). In addition, severe disability is associated with almost a 2-fold increase in health-related costs in the poor (7). How these hardships would influence quality of care and outcome can only be hypothesized but could significantly affect the decision to perform a craniectomy in low- or middle-income countries.
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