Clinical Neurology and Neurosurgery

Editorial Board

2012-02-01

Symptomatic cranial neuralgias in multiple sclerosis: Clinical features and treatment

Lorenzo De Santi, Pasquale Annunziata — 2011-11-30

Abstract: In multiple sclerosis, neuropathic pain is a frequent condition, negatively influencing the overall quality of life. Cranial neuralgias, including trigeminal, glossopharyngeal neuralgias, as well as occipital neuralgia, are typical expression of neuropathic pain. Neuralgias are characterised by paroxysmal painful attacks of electric shock-like sensation, occurring spontaneously or evoked by innocuous stimuli in specific trigger areas. In multiple sclerosis, demyelination in the centrally myelinated part of the cranial nerve roots plays an important role in the origin of neuralgic pain. These painful syndromes arising in multiple sclerosis are therefore considered “symptomatic”, in contrast to classic cranial neuralgias, in which no cause other than a neurovascular contact is identified. At this time, the evidence on the management of symptomatic cranial neuralgias in multiple sclerosis is fragmentary and a comprehensive review addressing this topic is still lacking. For that reason, treatment is often based on personal clinical experience as well as on anecdotal reports.The aim of this review is to critically summarise the latest findings regarding the pathogenesis, the diagnosis, the instrumental evaluation and the medical as well as neurosurgical treatment of symptomatic trigeminal, glossopharyngeal and occipital neuralgia in multiple sclerosis, providing useful insights for neurologists and neurosurgeons and a broad range of specialists potentially involved in the treatment of these painful syndromes.

The safety and feasibility of outpatient carotid endarterectomy

Curtis E. Doberstein, Marc A. Goldman, Jonathan A. Grossberg, Heather S. Spader — 2011-10-14

Abstract: Background: Carotid endarterectomy (CEA) is one of the most commonly performed and studied surgical procedures for extracranial ischemic disease.Objective: The authors reviewed the outcome of 39 consecutive carotid endarterectomy procedures performed by a single surgeon with emphasis on the safety of discharging patients the same day of the procedure.Methods: Retrospective analysis was performed over a two-year period on patients who were admitted as outpatients and underwent CEA. Following CEA, patients were observed for 4–6h in the recovery room and Duplex ultrasonography was completed to assess the endarterectomy repair. Determination was then made whether patients could be safely discharged home.Results: Over a two year period, CEA was performed 39 times in 37 outpatients. Twenty-five patients (64%) were discharged within 6h of surgery completion. The remaining 14 patients (36%) were admitted to the hospital for varying reasons. Six patients (43%) stayed either due to personal preference or the lack of supervision at home and six other patients (43%) stayed because of mild hemodynamic instability. Of the two remaining patients, one was admitted for chest pain and the other for a small wound hematoma. No patients developed postoperative neurologic deficits. Two-tailed Fisher test analysis of collected variables revealed that patients who had general anesthesia were more likely to be admitted (p<0.02).Conclusion: Patients undergoing CEA can be safely discharged the same day after a brief period of postoperative observation. One factor that may predict the need for postoperative admission is the use of general anesthesia.

Infrasellar craniopharyngioma

Xin Yu, Rui Liu, Yaming Wang, Hongwei Wang, Hulei Zhao, Zhaohun Wu — 2011-10-24

Abstract: Object: Infrasellar craniopharyngioma (IC) is a rare tumor. This study aimed to investigate the clinical manifestations, treatment methods, and prognosis of IC, which invades the cranial base, nasal sinuses, nasopharynx and clivus.Methods: Eleven consecutive cases of IC who received treatment in People's Liberation Army Navy General Hospital from 1988 to 2007 were retrospectively analyzed, and the clinical manifestations of IC were summarized. At the same time, literature pertinent to IC was reviewed. These patients consisted of six males and five females with an average age of 28.5 years (range 7–52 years old). Among them, nine cases were identified as simple IC and two cases as suprasellar and IC. Clinical manifestations included headache (seven cases), nasal obstruction (four cases), polydipsia and polyuria (four cases), visual disorder (five cases), delayed sex organ development (three cases), menstrual disorder (one case) and no symptoms (one case). Tumor invasion regions included sellar bottom, ethmoidal sinus, maxillary sinus, sphenoidal sinus, infrasellar region, clivus, nasopharynx and nasal cavity. Solid craniopharyngioma was observed in three cases, cystic craniopharyngioma in seven cases, and mixed cystic and solid type in one case. Four cases underwent craniotomy for tumor resection (three cases also received adjuvant external beam radiation therapy), two cases underwent transnasal approach tumor resection under endoscope guidance (one case simultaneously received adjuvant interstitial brachytherapy) and four cases underwent stereotactic interstitial radiation (radioisotope 32P brachytherapy).Results: All cases were followed up for an average of 22.5 years (range 9–98 months). Imaging results showed that tumors disappeared in one case, were clearly reduced in eight cases and were stable in two cases. Clinical symptoms disappeared in three cases, and improved in seven cases. No symptoms appeared in the case presenting with no symptoms. All patients were able to resume work, study and daily tasks.Conclusions: IC is rare (the present cases account for 0.23% of all retrieved cases). Its chief clinical manifestations include headache, nasal obstruction, polydipsia and polyuria, and visual disorder. Lesions include solid, cystic, and mixed cystic and solid types. It is very difficult to resect the whole diseased region because this disease invades the cranial base, nasal sinuses and nasopharynx. Individualized treatments should be used according to lesion characteristics and invasion range, for example, stereotaxic interstitial brachytherapy. Radical resection or partial resection plus external beam radiation therapy produces better prognosis in IC than intracranial craniopharyngioma.

Outcome of Chlamydia pneumoniae associated acute ischemic stroke in elderly patients: A case–control study

2011-10-26

Abstract: Background: Limited data exists about the role of Chlamydia pneumoniae elderly patients with acute ischemic stroke.Objective: To study the role of C. pneumoniae in elderly patients (age more than 65years) with acute ischemic stroke and its impact on stroke out come.Methods: We recruited 100 elderly patients with acute ischemic stroke and 100 age and sex matched controls over a period of 2years. IgG and IgA anti C. pneumoniae antibodies were measured by microimmunofluorescence technique in patients and controls. Good outcome was defined as a Modified Rankin score (mRS) of ≤2.Results: We found C. pneumoniae antibodies in 35% stroke patients and in 18% control subjects (p=0.01). Good out come at 90days follow up was found in 20/35(57.1%) seropositive stroke patients compared to 37/65(56.9%) seronegative stroke patients (p=0.9).Conclusions: C. pneumoniae antibody positivity was independently associated with ischemic stroke in elderly patients and its presence does not alter the stroke outcome.

Clinical and cellular characterization of two novel MPZ mutations, p.I135M and p.Q187PfsX63

2011-10-24

Abstract: Objectives: We report the clinical and cellular phenotypes of two novel MPZ mutations associated with CMT1B.Methods: The two families were evaluated clinically, electrophysiologically, and genetically. The wild-type and mutant P0 fused with fluorescent proteins were expressed in vitro to monitor their intracellular trafficking. Adhesion assay was also performed to evaluate the adhesiveness of cells.Results: The two novel heterozygous MPZ mutations, p.I135M and p.Q187PfsX63, are associated with a childhood-onset demyelinating polyneuropathy. The median motor nerve conduction velocities of the two index patients carrying each mutation were 12.9 and 13.6m/s, respectively. Fluorescence analysis demonstrated that the P0 I135M protein was located on the cell membrane, but the P0 Q187PfsX63 protein was retained ectopically in the endoplasmic reticulum and Golgi apparatus. Adhesion assay demonstrated a defective adhesiveness of cells expressing either mutant P0 protein, and P0 Q187PfsX63 had a more prominent defect of self-adhesive ability than P0 I135M.Conclusions: This study expanded the spectrum of the MPZ mutations and revealed two disparate mechanisms of MPZ mutations associated with a typical CMT1B phenotype. Other modifying genetic, epigenetic, or environmental factors on CMT1B may exist to explain the discrepancy between the cellular phenotypes.

The neuropsychology of iNPH: Findings and evaluation of tests in the European multicentre study

Per Hellström, Petra Klinge, Jos Tans, Carsten Wikkelsø — 2011-10-24

Abstract: Objective: Neuropsychological dysfunction is common in patients with idiopathic normal pressure hydrocephalus (iNPH). Shunt treatment is beneficial, some patients reaching complete or almost complete recovery, while others show only minor improvement. We aimed to assess the efficacy of a small selection of well characterized and sensitive neuropsychological tests in the context of the European multicentre study on iNPH (Eu-INPH).Methods: One hundred and forty-two iNPH patients included in Eu-iNPH were tested with the Rey Auditory Verbal Learning Test (RAVLT), the Grooved Pegboard and the Stroop test before and after three and twelve months of treatment with a ventriculoperitoneal shunt. Their performance was compared to that of 108 healthy individuals (HI).Results: INPH patients performed significantly worse than HI on all of the neuropsychological measures at entry. The discriminative capacities of the eight variables were similar, with areas under the curve (AUC; ROC analysis) ranging between .86 (Delayed Recall) and .95 (Grooved Pegboard). The most usable test was RAVLT (Learning and Delayed Recall), administered to ≥90% of the patients at all occasions. However, the Grooved Pegboard and the Stroop test were more sensitive to treatment effects.Conclusion: The three neuropsychological tests used in the Eu-iNPH are expedient, highly diagnostically discriminative, and well suited to evaluate changes following shunt treatment.

Feasibility of cerebral magnetic resonance imaging in patients with externalised spinal cord stimulator

2011-10-24

Abstract: Object: Spinal cord stimulation (SCS) is a well-known treatment option for intractable neuropathic pain after spinal surgery, but its pathophysiological mechanisms are poorly stated. The goal of this study is to analyse the feasibility of using brain MRI, functional MRI (fMRI) and Magnetic Resonance Spectroscopy (MRS) as tools to analyse these mechanisms in patients with externalised neurostimulators during trial period.Methods: The authors conducted in an in vitro and in vivo study analysing safety issues when performing brain MRI, fMRI and MRS investigations in human subjects with externalised SCS. Temperature measurements in vitro were performed simulating SCS during MRI sequences using head transmit-receive coils in 1.5 and 3T MRI systems. 40 Patients with externalised SCS were included in the in vivo study. 20 patients underwent brain MRI, fMRI and another 20 patients underwent brain MRI and MRS.Results: A maximal temperature increase of 0.2°C was measured and neither electrode displacements nor hardware failures were observed. None of the patients undergoing the MRS sequences at the 1.5 or 3T MRI scanners described any discomfort or unusual sensations.Conclusion: We can conclude that brain MRI, fMRI and MRS studies performed in patients with externalised SCS can be safely executed.

Modified Lund concept versus cerebral perfusion pressure-targeted therapy: A randomised controlled study in patients with secondary brain ischaemia

Kemal Dizdarevic, Alhafidz Hamdan, Ibrahim Omerhodzic, Elvedina Kominlija-Smajic — 2011-10-31

Abstract: Purpose: Secondary brain ischaemia (SBI) usually develops after aneurysmal subarachnoid haemorrhage (SAH) and severe traumatic brain injury (TBI). Current approaches to managing these conditions are based either on intracranial pressure-targeted therapy (ICP-targeted) with cerebral microdialysis (CM) monitoring according to the modified Lund concept or cerebral perfusion pressure-targeted therapy (CPP-targeted). We present a prospective, randomised controlled study comparing relative effectiveness of the two management strategies.Methods: Sixty comatose operated patients with SBI following aneurysmal SAH and severe TBI were randomised into ICP-targeted therapy with CM monitoring and CPP-targeted therapy groups. Mortality rates in both groups were calculated and tissue biochemical signs of cerebral ischaemia were analysed using CM. Measured CM data were related to outcome (Glasgow Outcome Scale [GOS] score 1, 2 and 3 for poor outcome or GOS score 4 and 5 for good outcome).Results: Patients treated with ICP-targeted therapy with CM monitoring had significantly lower mortality rate as compared with those treated with CPP-targeted therapy (P=0.03). Patients monitored with CM who had poor outcome had lower mean values of glucose and higher mean values of glycerol and lactate/pyruvate ratio as compared with those who had good outcome (glucose: P=0.003; glycerol: P=0.02; lactate/pyruvate ratio: P=0.01). There was no difference in the mortality outcome between aneurysmal SAH and severe TBI in the two groups (P=0.28 for ICP-targeted therapy with CM monitoring, P=0.36 for CPP-targeted therapy). Also, there were no differences in the CM values between patients with aneurysmal SAH and severe TBI who underwent ICP-targeted therapy (glucose: P=0.23; glycerol: P=0.41; lactate/pyruvate ratio: P=0.40).Conclusion: The modified Lund concept, directed at bedside real-time monitoring of brain biochemistry by CM showed better results compared to CPP-targeted therapy in the treatment of comatose patients sustaining SBI after aneurysmal SAH and severe TBI.

An analysis of related factors of surgical results for patients with craniopharyngiomas

Xintong Zhao, Xuxia Yi, Haijun Wang, Hongyang Zhao — 2011-11-07

Abstract: Objective: The objective of this study was to retrospectively review the surgical results following gross total resection and partial resection with or without radiotherapy for craniopharyngiomas and analyze the related factors of surgical results.Methods: From 1994 to 2009, 214 patients underwent 219 procedures for craniopharyngiomas. We retrospectively reviewed the pre- and postoperative data of patients, reported the perioperative and long-term surgical results and analyzed the influencing factors and the relationship between hypothalamic involvement and postoperative quality of life.Results: Gross total resection was achieved in 154 procedures (70.3%). Perioperative mortality was 5%. Perioperative hyperpyrexia was the most significant risk factor for perioperative mortality. A total of 151 patients were followed from 6 months to 190 months. There were significant differences in recurrence rate and overall survival between gross total resection and limited resection (P<0.05). There was significant difference in recurrence rate between limited resection and limited resection with radiotherapy (P<0.01), but it did not reach statistical difference between gross total resection and gross total resection with radiotherapy. The factors strongly influencing overall survival include old patients, partial resection and recurrent tumors. The preoperative hypothalamic involvement negatively correlates with the postoperative quality of life in patients with craniopharyngiomas.Conclusion: The preoperative CT/MR imaging provides clues of the relationship between tumor and surrounding structures. Gross total resection should be achieved in the treatment of craniopharyngiomas on the condition that hypothalamus is preserved. The patients who undergo limited resection should receive conventional radiotherapy or gamma knife surgery.

A case of parakinesia brachialis oscitans

2011-09-28

There have been few described cases of hemiplegia with involuntary elevation of paralyzed arms while yawning, symptoms referred to as parakinesia brachialis oscitans . Brain imaging shows that lesions in the internal capsule or basal ganglia are mainly associated with this movement . We report a patient of parakinesia brachialis oscitans after acute infarction involving the right motor cortex and frontal subcortex.

Neuroborreliosis mimicking brachial amyotrophic diplegia

Merijn P. te Lintelo, Peter J. Koehler, Ton H.J. van Diepen, Roy Beekman — 2011-10-13

Neuroborreliosis in Europe usually presents with radicular pain while paresis is present in more than half of the patients . Rarely an isolated lower motor neuron syndrome may occur in the absence of pain, sensory loss or typical Lyme disease. We describe a patient who presented with a “polio-like” syndrome with brachial diplegia caused by neuroborreliosis.

Crossed aphasia following an infarction in the right corpus callosum

2011-10-17

Abstract: A 68-year-old right-handed woman with no history of brain damage or familial left-handedness was admitted to our hospital due to the acute onset of speech difficulty; her speech was nonfluent. Literal and phonological paraphasias, agrammatism and paragrammatism were observed. Brain MRI revealed an acute infarction in the right anterior cerebral artery territory, involving the right corpus callosum. Moreover, cerebral blood flow was decreased not only in the area of the right corpus callosum but also in the left fronto-temporal lobe, suggesting crossed diaschisis. This is a rare case of crossed aphasia following an infarction in the right corpus callosum.

Slow progression and benign course of a primary malign melanoma of a lumbar nerve root

Ozan Ganiüsmen, Füsun Demirçivi Özer, Mesut Mete, Nail Özdemir, Ümit Bayol — 2011-10-24

Over 90% of malignant melanomas of the spine are metastatic. Rarely, however, a primary malignant melanoma may originate from the spine. In this setting, an intramedullary location is common. The remaining malignant melanomas in this region originate from the spinal cord or leptomeninges, which surround the nerve roots. The diagnosis is confirmed immunohistologically, as it is difficult to distinguish a malignant melanoma from melanotic schwannoma and meningeal melanocytoma . Total excision of a spinal malignant melanoma can have a better course with adjuvant radiotherapy and chemotherapy . This case report presents a patient who underwent surgery in 2007 and the pathological diagnosis was a primary spinal malignant melanoma originating from a nerve root. Despite metastasis, the patient was pain free and had no neurological deficits at the 4-year follow-up.

Tanycytic ependymoma of filum terminale: A case report

2011-11-07

Abstract: Tanycytic ependymoma is an uncommon but well-recognized variant of ependymoma. Here we report a case of tanycytic ependymoma occurring at the region of filum terminale in a 44-year male who presented with low backache, bilateral lower limb weakness and urinary incontinence. MR imaging in this patient showed a lesion that was composed of solid and cystic components and was suggestive of ependymoma. The filum terminale region is an extremely unusual location for the occurrence of tanycytic ependymoma. To the best of our knowledge this is the third case of tanycytic ependymoma occurring in the filum terminale region.

A case of Powassan viral hemorrhagic encephalitis involving bilateral thalami

Ellie Eun Ju Choi, Robert A. Taylor — 2011-10-31

Since McLean and Donahue discovered the Powassan virus in 1958, there have been approximately 34 cases of confirmed infection. This is the first case report to describe a serologically confirmed example of Powassan viral hemorrhagic encephalitis, which involved lesions of the bilateral thalami. MRI images of the patient's central nervous system (CNS) were unique, and when such images are encountered in the clinical setting, Powassan viral infection should be considered.

Idiopathic hypertrophic pachymeningitis mimicking neurosarcoidosis

Panos G. Christakis, Duarte G. Machado, Pooia Fattahi — 2011-11-09

The approach to a patient presenting with multiple cranial neuropathies is complex given the diverse number of causes including neoplastic, vascular, traumatic, infectious, autoimmune and inflammatory etiologies. In cases where imaging reveals thickened dura with enhancement around multiple cranial nerves and no primary cause can be found, the diagnosis of idiopathic hypertrophic pachymeningitis (IHP) is given . Here we present the case of a patient with multiple worsening cranial neuropathies who underwent extensive evaluation consistent with neurosarcoidosis, but whose dural biopsy revealed IHP.

Atypical cystic meningiomas arising from the trigeminal nerve: Surgical and neuroradiological consideration

2011-11-21

Reports on tumours originating from trigeminal nerve/Cavum Meckelii (CM) are scarce. Such neoplasms represent less than 0.5% of histologically proved intracranial tumours; among them approximately one-third are trigeminal schwannomas with the remainder consisting of meningiomas, lipomas, and epidermoid tumours .

Atypical central neurocytoma of fourth ventricle with hemorrhagic complication during surgery in a child

Deepali Jain, Subimal Roy, Sandeep Chopra — 2011-11-09

Central neurocytomas (CN) comprise only 0.25–0.5% of brain tumors. They mainly located in lateral ventricles. CN generally occur in young adults with a favorable prognosis and benign biologic behavior. Primary occurrence of CN in fourth ventricle has been reported very rarely. Only 5 case reports are available in the literature (). The WHO classification considers central neurocytomas as grade II tumors. The proliferative index is usually low, except for the rare “atypical” variants. “Atypical” histological features include high proliferative activity, vascular proliferation, and necrosis . We herein report an interesting case of atypical neurocytoma which was present in fourth ventricle with extensive hemorrhage occurred during surgery.

Gamma knife radiosurgery for giant cell tumor of the petrous bone

2011-11-14

Giant cell tumor (GCT) is uncommon bone tumor, and the skull involvement in this tumor is very rare . This tumor frequently shows locally aggressive behavior , and a relatively high recurrence rate . Resection is the preferred treatment for GCTs of the skull, and the outcome correlated the extent of resection . However, considering the limitation in surgical extent of skull base tumors, high recurrence rates, adjuvant treatments, and management for recurrence seem to be important issues in GCTs.

Management of symptomatic intrathecal catheter-associated inflammatory masses

2011-11-07

Abstract: Objective: Intrathecal catheter-associated inflammatory masses (CIMs) are a serious complication of implanted drug pumps. The goal of this study was to review our experience with CIMs, including the pathology of all resected CIMs, and identify objective data which may guide management.Methods: We performed a retrospective review of 13 patients who developed symptomatic CIMs during continuous intrathecal opioid therapy for chronic pain. Eight patients presented with pain plus neurologic deficit and 5 patients presented with pain alone.Results: CIM resection via laminectomy and intradural exploration was ultimately performed in 8 patients, 3 of whom were initially treated with a non-resective surgical approach (catheter repositioning or pump removal) that failed.All 3 patients who experienced a failure with non-resective surgery had CIMs located in the thoracic spine with a maximum diameter≥13mm and 2 of these patients had neurologic deficits on presentation.Conclusions: Our experience, with the largest reported single-surgeon series of patients harboring CIMs, favors early resection, especially in patients with neurologic deficit. Resection may also be a prudent first-line strategy for patients with larger thoracic masses (≥13mm) regardless of neurologic status. Neurologic deficits engendered by CIM usually improve after resection and the majority of patients in our series would have still elected to have an intrathecal pump for pain control knowing a CIM would have developed.

Shunt overdrainage after mild head trauma

Bora Gürer, Erdal Resit Yilmaz, Hüseyin Hayri Kertmen, Zeki Sekerci — 2011-10-31

Shunt overdrainage leading to subdural hematoma (SDH) formation is known to be one of the major complications of ventriculoperitoneal (V-P) shunt implantation . Shunt overdrainage after mild head trauma is rare.