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Multifocal motor neuropathy (MMN) is a unique disorder characterized by slowly progressive, asymmetric, distal and upper limb predominant weakness without significant sensory abnormalities. Electrophysiology is crucial to the diagnosis, revealing the hallmark partial conduction block. MMN is considered immune mediated due to the association with anti-GM1 antibodies and the response to immunomodulatory treatment. It is paramount to recognize MMN from other motor neuronopathies or peripheral neuropathies as it is treatable. Advances in pathogenesis, clinical features, electrophysiology, diagnostic studies and treatment are reviewed. References for this review were identified from literature search on Pubmed limited to dates from 1988 to 2011. Papers were selected if relevant to the review topic and published in English.

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Background: Transformational epidural steroid (TFES) is commonly used to treat lumbosacral radicular pain. However, very few studies have systematically evaluated the quality of analgesia following such procedures with respect to time. Objective: To evaluate long-term efficacy of TFES in patients with lumbosacral radiculopathy. Materials and Methods: A prospective study including 30 patients having lumbosacral radiculopathy secondary to prolapsed disc. Outcome variables were the amount of improvement just after the procedure and thereafter at 24 hrs, 1 month, 6 month and 1 year post-procedure, respectively, using visual analog scale (VAS) and numeric rating scale (NRS). Patients also filled Roland-Morris questionnaire pre-procedure, 6 month and 1 year follow-up. All patients received Ibuprofen for 3 days following the procedure, to alleviate post- procedural pain. An option of rescue surgery was reserved in case of unbearable pain (>7 VAS), appearance of sudden motor deficit or if patient opts for surgery. Same injection was repeated if at any point of time pain had >5 in VAS. Results: As per NRS, almost all patients had complete pain relief (mean 98%) immediate postprocedure. At 24 hrs, the score was 79%, at 1 month 60%, at 6 months 58.5% and at 1 year 59%. Preprocedure VAS was 9.2 and thereafter 0.6, 1.8, 3.9, 3.8 and 4.2 at similar time points. Roland-Morris score was 18/24, 10/24, 9/24, at pre-procedure, at 6 months and at 1 year, respectively. No complication was noted in any patient except post procedural local pain. Conclusion: Quality of pain relief produced by TFES was significant. Long-term quality of pain relief was better in patients with pain duration less than 6 months. Even though, the study was designed to inject the drug once, many of the patients required second injection. A further study with multiple injections at prefixed time interval might probably result in a better overall outcome.

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Introduction: Severe Guillain-Barré syndrome (GBS) is associated with significant morbidity and also mortality. Identification of modifiable risk factors may help in reducing the morbidity and mortality. Objective: To study the prognostic factors in a selected cohort of mechanically ventilated GBS patients. Materials and Methods: Case records of GBS patients requiring mechanical ventilation admitted between 1997 and 2007 were analyzed. All patients satisfied the National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria for GBS. Primary outcome parameters included mortality and GBS disability (Hughes) scale score at discharge. Results: During the study period, 173 (118 men and 55 women; mean age of 33.5 ± 21 years) GBS patients were mechanically ventilated. A history of antecedent events was present in 83 (48%) patients. In addition to motor weakness, In all facial palsy was present in 106 (61%), bulbar palsy in 91 (53%), sensory involvement in 74 (43%), and symptomatic autonomic dysfunction in 27 (16%). The overall mortality was 10.4%. On univariate analysis the risk factors for mortality included elderly age (P = 0.014), autonomic dysfunction (P = 0.002), pulmonary complications (P = 0.011), hypokalemia (P = 0.011), and bleeding (P = 0.026). All these factors were significant in multivariate analysis except for bleeding from any site and hypokalemia. In univariate analysis factors associated with Hughes scale score ≤ 3 at discharge included younger age (P = 0.02), presence of bulbar symptoms (P = 0.03) and less severe weakness at admission (P = 0.02), slower evolution of disease over more than 3 days (P = 0.01), electrodiagnostic evidence of demyelinating neuropathy (P = 0.00), and absence of sepsis (P = 0.01), hyperkalemia (P = 0.0001), and anemia (P = 0.02). In multivariate analysis age was the only significant factor. Conclusions: Early identification of modifiable risk factors, such as pulmonary involvement, autonomic dysfunction, hypokalemia, sepsis, bleeding, and nutritional complications, may reduce the mortality and morbidity associated with GBS.

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Background: The Guillain Barre' syndrome (GBS) is a heterogeneous disease with various subtypes, the prevalence of which would depend on the geographic region. Recognition of these subtypes is of clinical importance since each subtype has an independent pathogenesis and different type of pathology and prognosis. Objectives: To study the various subtypes of GBS using the various published electrophysiological criteria. Design: Retrospective descriptive study. Materials and Methods: In a tertiary care hospital setting, the study compared the various published criteria for demyelination in GBS. The charts of 115 consecutive patients referred for electrodiagnostic evaluation to the Electromyography laboratory between July 2000 and June 2006 were reviewed. Results: Of the 115 patients, 51 (44.4%) patients had axonal forms of GBS and 44 (38.2%) patients had acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Applying the various published criteria, the number of patients categorized under the AIDP subtype ranged between 23.4% and 67.2%. Conclusion: In this study 44% patients had axonal forms of the disease, 38.2% patients had AIDP subtype and 17% remained unclassified. The most sensitive criteria to identify AIDP were the criteria proposed by Albers and colleagues and the Dutch group.

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Background: Computed-assisted three-dimensional data (3D) allows for an accurate evaluation of volumes compared with traditional measurements. Aims: An in vitro method comparison between geometric volume and 3D volumetry to obtain reference data for pituitary volumes in normal pituitary glands (PGs) and PGs containing adenomas. Design: Prospective, transverse, analytical study. Materials and Methods: Forty-eight subjects underwent brain magnetic resonance imaging (MRI) with 3D sequencing for computer-aided volumetry. PG phantom volumes by both methods were compared. Using the best volumetric method, volumes of normal PGs and PGs with adenoma were compared. Statistical analysis used the Bland-Altman method, t-statistics, effect size and linear regression analysis. Results: Method comparison between 3D volumetry and geometric volume revealed a lower bias and precision for 3D volumetry. A total of 27 patients exhibited normal PGs (mean age, 42.07 ± 16.17 years), although length, height, width, geometric volume and 3D volumetry were greater in women than in men. A total of 21 patients exhibited adenomas (mean age 39.62 ± 10.79 years), and length, height, width, geometric volume and 3D volumetry were greater in men than in women, with significant volumetric differences. Age did not influence pituitary volumes on linear regression analysis. Conclusions: Results from the present study showed that 3D volumetry was more accurate than the geometric method. In addition, the upper normal limits of PGs overlapped with lower volume limits during early stage microadenomas.

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Moyamoya disease is a progressive steno-occlusive disease of bilateral carotid forks with the formation of fine collateral vascular network and is an angiographic diagnosis. We analyzed case records of 11 patients with "adult-onset moyamoya disease." Six patients presented with intracranial hemorrhage (intracerebral and/or intraventricular) and 5 with focal ischemia. Angiography revealed bilateral Internal carotid artery involvement in 8 patients and unilateral involvement in 3. Posterior cerebral artery involvement was seen in 3 patients. Saccular aneurysm involving posterior circulation was seen in only 1 patient. Although rare, adult-onset moyamoya disease should be considered as one of the causes for intracerebral and intraventricular hemorrhage in adults.

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We report two patients manifesting with involvement of central and peripheral nervous system with brain magnetic resonance imaging (MRI) changes and pathological features of neuropathy possibly due to harmful and chronic use of various nitroimidazole group of medications for recurrent diarrheal illness. Patient 1, a 21-year-old man with obsessive-compulsive disorder, impulsive behavior and harmful use of substance (tinidazole), had developed encephalopathy and biopsy-proven neuropathy with partial remission. The MRI of brain showed involvement of bilateral caudate, lentiform and dentate nuclei, and splenium, with contrast enhancement of the caudate and putaminal lesions and restricted diffusion of the splenial lesion. Patient 2 was a 50-year-old woman with irritable bowel syndrome and was on harmful use of tinidazole and metronidazole. She manifested with encephalopathy, ataxia, and neuropathy. Her MRI of brain revealed involvement of bilateral putamen, dentate nuclei and periventricular white matter with restricted diffusion. Sural nerve biopsy revealed evidence of vasculitic neuropathy. At follow-up, there was definite, though incomplete, recovery in both the patients. The MRI alterations improved completely in patient 2 and substantially in patient 1. Increasing awareness among the physicians may enable early recognition of potentially reversible neurotoxicity and avoid unwarranted prescription of such medications.

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Background: Guillain-Barré syndrome (GBS) has been the most common cause of flaccid paralysis in children after the decline in the incidence of poliomyelitis. There are not any published data from the Indian subcontinent documenting electrophysiological patterns and antiganglioside antibodies in pediatric GBS. Materials and Methods: The study population included children with GBS referred for electrodiagnostic evaluation and also children with GBS admitted to our institute between August 2006 and July 2007. Nerve conduction studies were done to determine GBS subtypes and serum antiganglioside antibodies were measured using enzyme-linked immunosorbent assay (ELISA). Clinical and electrophysiological features were correlated with antiganglioside antibody results. Results: Of the 43 (male to female ratio = 2.1:1) children studied, 97.6% had motor weakness, 76.7% had cranial nerve palsies, 13.9% had autonomic disturbances and respiratory paralysis was found in 9.3% children. Antecedent illness was recorded in 69.8% children. The GBS subtype distribution was as follows: acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 21 (48.8%), acute motor axonal neuropathy (AMAN) in 19 (44.2%), and 3 (6.9%) children were unclassified. The severity of illness was similar in both AMAN and AIDP subtypes and the recovery in both the subtypes was complete without any significant difference in the duration of recovery. Preceding diarrheal illness was more common in AMAN subtype as compared to AIDP subtype (57.9% vs. 4.7%, P = 0.007). Sensory symptoms were more common in AIDP subtype than in AMAN subtype (66.6% vs. 21%, P = 0.03}. The commonest ganglioside antibody was IgM GM2. Anti GM3 antibodies were exclusively seen in children with AMAN and IgG GD1b was significantly associated with (36.7 vs. 4%; P = 0.007) AMAN subtype. IgG GT1b was identified in 50% of patients with AIDP as compared to 22.7% in patients with AMAN. Conclusion: In this study, AMAN subtype accounted for a significant proportion of pediatric GBS. AMAN was associated with diarrhea and specific antiganglioside antibodies. Recovery in children with GBS was complete, irrespective of the subtype.

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Background: The clinical presentation, neurophysiological findings, and outcome may vary between primary and secondary chronic inflammatory demyelinating polyradiculopathy (CIDP). Objective: To compare clinical and electrodiagnostic features of primary and secondary CIDP. Setting: Tertiary care teaching referral hospital. Materials and Methods: The CIDP patients who were diagnosed as per European Federation of Neurological Societies/Peripheral Nerve Society criteria were included and subjected to detailed history and examinations. The clinical disability was graded on a 0-10 scale. Neurophysiology included motor and sensory nerve conductions and F wave studies of all four limbs. Based on investigations for underlying diseases, the patients were categorized into primary or secondary CIDP. Prednisolone was prescribed in all and azathioprine added in resistant cases. The secondary CIDP group received specific treatment in addition. The outcome was assessed at 3 months, 6 months, and last follow-up. Results: A total of 65 patients aged 17 to 72 years were included and 20 were females. Twenty-five patients had secondary CIDP and include diabetes mellitus (16), POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) (4), monoclonal gammopathy of undetermined significance (2), myeloma (1), lymphoma (1), and malignancy (1). The secondary CIDP patients were older (48.35 vs 41.0 years), had less relapsing remitting (0 vs 6) and more frequent dysautonomia (7 vs 1). The demyelinating features were more marked in primary CIDP group and had better outcome compared with secondary CIDP. Conclusions: Of the total patients with CIDP, 38.5% of patients had secondary CIDP which was associated with progressive course, less demyelinating features, and worse prognosis.

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Background: Parkinson's disease (PD) is associated with autonomic dysfunction and chronic levodopa therapy has been reported to impair the autonomic control of heart rate. Aim: Our aim was to assess the immediate effect of a single dose of levodopa on heart rate variability (HRV) in idiopathic PD. Materials and Methods: Eleven patients of idiopathic PD (F:M =2:9, mean age 57.3±8.6 years, duration of illness 4.1±2.8 years, Hoehn and Yahr stage 2.1±0.2) on stable levodopa dosage were studied. Motor part of unified Parkinson's disease rating scale and resting Lead II electrocardiogram (ECG) recordings were performed at baseline (12 hours off medication) and after two tablets of 100/10 mg of standard levodopa/ carbidopa. ECG was recorded continuously in the first hour (H1) followed by a 15-min recording in second (H2), third (H3) and fourth (H4) hours. Artifact free 5-min segments of the ECG were analyzed offline to obtain the HRV parameters in time domain (ms) and frequency domains (ms ² ). Results: Significant increase was observed in standard deviation of normal to normal intervals (23.5±2.7-46.2±6.6, P<0.05), root mean square of successive differences of NN intervals (16.3±2.9-30.7±5.1, P<0.01), total power (568.9±125.7-2739±667.5, P<0.01), low frequency power (146.5±40.8-614.1±206.7, P<0.05) and high frequency power (107.4±33.9-332.7±85.9, P<0.05) in H1. Conclusion: The results are suggestive of an improvement in the overall variability of the heart rate indicating an enhanced vagal tone.

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Objective: The aim of this study was to determine the incidence, risk factors and outcome of intra procedure rupture (IPR) during endovascular coiling of intracranial aneurysms, a neurosurgeons' experience. Materials and Methods: The frequency of IPR was studied in 168 aneurysms treated by endovascular coiling in 150 consecutive patients. Aneurysm size, morphology, history of previous subarachnoid hemorrhage (ruptured) and timing of treatment after subarachnoid hemorrhage were the data collected for comparison. Results: Procedure-related rupture during endovascular coiling occurred in five (2.97%) of the 168 aneurysms treated. IPR was the cause for 1.78% treatment-related deaths. Small aneurysm size was the major risk factor for IPR in this series (P < 0.001). Conclusions: In this study, the frequency of IPR was similar to the reported frequency in the procedures performed by neurointerventionists.

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Background: Guillain-Barré syndrome (GBS), is a common post-infectious polyradiculoneuropathy worldwide. The commonest implicated causative organism the world over is Campylobacter jejuni (C. jejuni). This study was carried out to determine the relationship between C. jejuni infection and GBS in an Indian setting. Materials and Methods: This prospective study was carried out on a cohort of 50 patients with GBS who were treated in a tertiary care hospital in India. Based on electrophysiological findings the patients were divided into various subtypes. Serology for C. jejuni (Immunoglogulin G, IgG and Immunoglogulin, IgM) using an enzyme-linked immunosorbent assay method (ELISA) was done both in patients and 40 age, sex and geographically matched controls. Results: Evidence of recent C. jejuni infection was present in 30% of GBS patients compared to 8% of controls (15/50 vs. 3/40 P<0.005). Eight (47%) C. jejuni-positive patients reported symptoms of gastroenteritis 4-30 days (mean 13 days) prior to onset of GBS. Of the 15 patients with evidence of C. jejuni infection, 10 (67%) patients had axonal type of GBS. Axonal variety of GBS presented in a younger age group compared to acute inflammatory demyelinating polyradiculoneuropathy (AIDP) patients (mean age: axonal vs. AIDP: 30.11 + 13.73 vs. 40.2 + 18.77). C. jejuni-positive patients presented mainly in spring and winter and had a similar age and sex incidence as compared to the rest of the GBS patients. Conclusions: Preceding C. jejuni infection is common among GBS patients and is often associated with the axonal variety of GBS. Axonal variety of GBS generally presents in a younger age group as compared to AIDP.

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Intraparenchymal schwannomas are rare and most of the reported cases are in supratentorial region with frontal lobe being most common. Infratentorial location is very rare. We report a posterior fossa midline large cystic schwannoma with mural nodule mimicking as pilocytic astrocytoma. The pathogenesis and neuroradiological findings of intraparenchymal schwannomas are discussed with review of the related literature.

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Neurosurgical patients, in particular patients with severe head injury require frequent computed tomogram (CT) of the head, usually at short notice. A mobile CT may prove to be invaluable for these patients. This report reviews the initial experience with a mobile CT at tertiary trauma center. A total of 1292 head CT scans were done during 9 months study period with an average of 4.78 CT scans for day. Of the 563 patients in whom the detailed prospective data was available, 456 (81%) patients were on ventilator, 450 (80%) patients were on sedation, and 37 (6.5%) patients were on ionotropic support at the time of doing CT scan. The mean Glasgow Coma Scale score at the time of CT was 8.1 (range 3-15). The average time to do a CT scan (from time of request to transmission into picture archiving and communication system) was 12.6 min (range 7.8-47 min). Image quality was judged to be excellent by all the neurosurgical faculty in the intensive care unit. During the study period, the main CT scanner broke down four times (for variable time periods) and the mobile CT scanner could manage the load of severe head injured patients in the casualty without any problems. The mobile CT machine itself broke down 36 times and the mean response time was 12.5 h (range 1-144 h) during each breakdown point. This experience suggests that mobile CT is extremely useful in the management of patients with severe traumatic brain injury and can be recommended for any high-volume neurosurgery department in the country.

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Background: Focused studies on sensory manifestations, especially pain and paresthesia in Guillain-Barre' (GB) syndrome are few and far between. Aim: To study the sensory manifestations in GB syndrome during 10 days of hospitalization with clinico-electrophysiological correlation. Materials and Methods: The study included 60 non-consecutive patients with GB syndrome, fulfilling National Institute of Neurological and Communicative Disorders and Stroke (NINCDS) criteria for GB syndrome. Data especially related to clinical and electrophysiological evidence of sensory involvement were analyzed. Pain was assessed using a) visual analogue paraesthesias (Vapar), b) visual analogue for pain (Vap) and c) verbal rating scale for pain (Verp). Results: Sensory symptoms were widely prevalent: paraesthesia in 45 (75%) patients and pain in 30 (50%) patients. Impairment of different sensory modalities included: pain in 8 (13.3%), joint position sense in 14 (23.3%), and vibration in 11 (18.3%). Electrophysiological evidence of abnormal sensory nerve conduction was noted in 35 (58.3%) patients. Pain assessment using Vapar, Vap and Verp for from Day 1 to Day 10 of hospitalization revealed that from Day 7 onwards the degree and frequency of sensory symptoms and signs decreased. On comparing various clinico-electrophysiological parameters among patients of GB syndrome with and without pain and paresthesia. Presence of respiratory distress correlated with pain and paresthesia (P=0.02). Conclusions: Sensory manifestations in GB syndrome are often under-recognized and under-emphasized. This study analyzed the evolution and the profile of pain and paresthesia in GB syndrome during hospitalization. Knowledge, especially about evolution of pain and paresthesia during hospitalization might improve understanding and patient care.

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Background: Evaluation of the degree of severity of injury, coma duration, and prediction of outcome are integral parts of present-day management of severe traumatic brain injury (TBI). Objective: To investigate whether evaluation and prediction of outcome in early phase after severe TBI is possible by means of single-voxel proton magnetic resonance spectroscopy ( ¹ H-MRS). Materials and Methods: Proton spectra were acquired from the posterior part of normal-appearing frontal lobes having predominantly white matter in 72 patients with severe TBI within a few days of trauma, mean 9.5 days and also in 30 controls. Results: ¹ H-MRS studies revealed lower ratios of N-acetylaspartate (NAA)/Choline (Cho) and NAA/ Creatine (Cr) and higher ratios of Cho/Cr in patients with TBI when compared to the control group. In patients with severe TBI, NAA/Cr, NAA/Cho and Cho/Cr ratios were significantly correlated with the initial Glasgow Coma Scale score (GCS) (P=0.004, r =0.439, P=0.018, r =0.364, P=0.004, r = -0.762, respectively), and with the clinical outcome, Glasgow Outcome Scores (GOS) (P=0.006, r =0.414; P=0.007, r =0.412; P=0.016, r = -0.775, respectively). An equation including clinical and spectroscopic variables, which can predict coma duration fairly accurately, was also obtained. Conclusions: ¹ H-MRS may be a novel method of assessing brain function, estimating coma duration, and predicting outcome in patients with severe TBI.

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Background: Several malignant tumors and brain abscesses have similar magnetic resonance imaging (MRI) findings. Because the prognosis and treatment strategies differ among these disorders, accurate preoperative diagnosis is very significant. Recently, diffusion-weighted imaging (DWI) and calculation of apparent diffusion coefficients (ADCs) have been reported to be useful to differentiate between the two disorders. Materials and Methods: MRI and DWI were performed in 37 patients: 13 with glioblastomas, 14 with metastatic tumors, 7 with malignant lymphomas, and 3 with brain abscesses. The minimum ADC value (ADC _min ) and mean ADC value (ADC _mean ) were taken into consideration. Results: Statistically significant differences were found in the enhanced lesions for ADC _min and ADC _mean between metastatic tumors and malignant lymphomas. The receiver operating characteristic analyses revealed no statistically significant differences among them. There were statistically significant differences in ADC _mean in the cores of cystic lesions between brain abscesses and metastatic tumors or glioblastomas. Conclusions: Our results suggest that evaluation using ADC values may facilitate differentiating metastatic tumors from malignant lymphomas, and also metastatic tumors or glioblastomas from brain abscesses.

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Objectives : T0 o evaluate the advantages and disadvantages of a fast post-operative screening method using deep brain stimulation (DBS) of the subthalamic nucleus (STN) in patients with Parkinson's disease. Materials and Methods : T0 wenty-four patients implanted with electrodes for STN-DBS were divided into two groups : G0 roup I received traditional programming and Group II was treated with the fast method. The time required for programming in each group and the treatment effects were analyzed. Results : S0 ignificant postoperative improvement of the Unified Parkinson's Disease Rating Scale III was achieved in the "off medication" and "on stimulation" condition in both the groups (Group I: 51.2% vs. Group II: 54.0%, P<0.05). The average time needed for programming, however, was significantly shorter in Group II as compared to Group I (P<0.05). Conclusions : T0 he new fast method can significantly reduce the time required to find a preliminary effective STN-DBS protocol in the early postoperative phase without sacrificing clinical efficacy.

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We describe the surgical treatment of tuberous sclerosis complex (TSC)-related refractory epilepsy in three patients. All three had multiple daily seizures, and each had more than three cortical tubers. High-resolution magnetic resonance imaging, ictal and interictal scalp electroencephalogram (EEG), positron emission tomography, magnetoencephalography as well as acute and intracranial video-EEG were used to identify the epileptogenic tubers. After localization, five cortical tubers (two in the right temporal lobe in patient 1; one each in the left frontal and temporal lobes in patient 2; and one in the right frontal lobe in patient 3) were resected. At minimum follow-up of 13 months (range, 13-35 months), patient 3 had seizure remission; patient 2 had only rare seizures; while patient 1 had 60% reduction in seizure frequency. Successful epilepsy surgery is possible in patients of TSC with multiple tubers using the multimodal approach.

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A superficial temporal artery (STA) false aneurysm caused by surgery of a traumatic intracranial false aneurysm is reported. A 28-year-old man underwent craniotomy for aneurysm clipping 20 days after traumatic head injury. At surgery the aneurysm was a false aneurysm due to its avulsion from the parent artery without a real neck. A "clip wrapping" technique was used to repair the deficit on the parent artery. On postoperative Day 25, repeat digital subtraction angiogram (DSA) revealed a new right STA aneurysm, which was not apparent in the preoperative DSA. We feel that this aneurysm might have probably resulted from the iatrogenic injury to the STA during the initial surgery as the location of aneurysm was at the initial craniotomy site. The pathophysiology, etiology, surgical treatment and preventive measures of false aneurysms have been discussed.

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