Intracranial Hemorrhage Associated With T-Cell Acute Lymphoblastic Leukemia With Hyperleukocytosis: A Case Report.

Acute leukemia in children may present with hyperleukocytosis. Symptomatic hyperleukocytosis is a medical emergency that necessitates rapid stabilization of the patient and prompt lowering of the leukocyte count. We report on a patient with intracranial hemorrhage associated with T-cell acute lymphoblastic leukemia with hyperleukocytosis, which is a rare occurrence. A 16-year-old boy with hyperleukocytosis (total white cell count; 398×103/µL) underwent repeated leukapheresis and received supportive treatment until a definite diagnosis of T-cell acute lymphoblastic leukemia was made and chemotherapy was started at 10% of the usual dose. On day 2 of treatment, he had headache, vomiting, and was agitated. Brain magnetic resonance imaging showed bilateral extensive hemispheric and cerebellar punctate areas of hemorrhage and perilesional edema. Chemotherapy intensified to a maximum dose on day 3. If supportive care for tumor lysis syndrome can be promptly provided, initial chemotherapy regimen can immediately be begun at an optimal dose.

A Rare and Subtle Etiology of Chronic Oropharyngeal Pain: Isolated Internal Carotid Artery Kinking without Stenosis.

Carotid artery kinking is a frequent finding in duplex ultrasonography. However, isolated morphological changes without significant carotid stenosis are rarely symptomatic. Neck pain is a rare symptom in patients with carotid artery kinks. The vascular etiology in patients with persistent neck pain is usually overlooked. A 58-year-old female patient with chronic neck pain presented to our clinic. Following multidisciplinary team review, the symptoms were found due to the kinking of the internal carotid artery. In this report, we present the clinical presentation of the patient with the kinking of the internal carotid artery without stenosis, surgical management of the pathology, and a brief literature review.

Clinicopathological significance of baseline T2-weighted signal intensity in functional pituitary adenomas.

PURPOSE: To assess baseline T2-weighted signal intensity (T2-WSI) of functional pituitary adenomas (FPA), and to investigate the relationship of baseline T2-WSI with clinical features, histopathological granulation patterns, and response to treatment in patients with acromegaly, prolactinoma and Cushing's disease (CD). METHODS: Somatotroph adenomas (n = 87), prolactinomas (n = 78) and corticotroph adenomas (n = 29) were included in the study. Baseline T2-WSI findings (grouped as hypo-, iso- and hyperintense) were compared with hormone levels, tumor diameter, granulation patterns and response to treatment. RESULTS: Somatotroph adenomas were mostly hypointense (53%), prolactinomas were dominantly hyperintense (55%), and corticotroph adenomas were generally hyperintense (45%). Hyperintense somatotroph adenomas were larger in size with sparsely granulated pattern and tumor shrinkage rate was lower after somatostatin analogues (SSA) (p = 0.007, p = 0.035, p = 0.029, respectively). T2 hypointensity was related with higher baseline IGF-1% ULN (upper limit of normal) levels and a better response to SSA treatment (p = 0.02, p = 0.045, respectively). In female prolactinomas, hyperintensity was correlated with a smaller adenoma diameter (p = 0.001). Hypointense female prolactinomas were related to younger age at diagnosis, higher baseline PRL levels and dopamine agonist (DA) resistance (p = 0.009, p = 0.022, p < 0.001, respectively). Hyperintense corticotroph adenomas were related to larger adenoma size and sparsely granulated pattern (p = 0.04, p = 0.017, respectively). There was no significant difference in the recurrence with T2WSI in CD. CONCLUSION: Baseline hypointense somatotroph adenomas show a better response to SSA, whereas hypointensity was related to DA resistance in female prolactinomas.

How Different Are the Patients With Bilateral Hippocampal Sclerosis From the Unilateral Ones Clinically?

PURPOSE: There is a lack of knowledge on consecutive patients with epilepsy associated with bilateral hippocampal sclerosis (BHS). We aimed to investigate the differentiating features of BHS in comparison with unilateral HS (UHS). METHOD: We investigated our database for patients with epilepsy fulfilling the major magnetic resonance imaging criteria for BHS; namely, presence of bilateral atrophy and high signal changes on T2 and FLAIR series in the hippocampi. UHS patients seen in past 2 years were included as the control group. Clinical, EEG, and other laboratory findings, data on treatment response and epilepsy surgery were investigated from their files. RESULTS: A total of 124 patients (31 with BHS and 93 with UHS; 49 right-sided and 44 left-sided) were included. We found that 16.1% of the BHS and 18.3% of the UHS groups were not drug-refractory. A binary logistic regression analysis performed with significant clinical features disclosed that history of febrile status epilepticus, mental retardation, and status epilepticus were statistically more common in BHS group. Moreover, diagnosis of psychosis established by an experienced psychiatrist and slowing of the EEG background activity were both found significantly more frequent in BHS. 66.67% of the operated BHS patients showed benefit from epilepsy surgery. CONCLUSIONS: BHS is a heterogeneous group, showing significant differences such as increased frequencies of mental retardation, status epilepticus, febrile status epilepticus and psychosis, in comparison to UHS. In all, 16.1% of the BHS cases showed a benign course similar to the UHS group and some patients with drug-resistant epilepsy may show benefit from epilepsy surgery.

Transcortical Removal of Third Ventricular Colloid Cysts: Comparison of Conventional, Guided Microsurgical and Endoscopic Approaches and Review of the Literature.

AIM: To compare the clinical results of different transcortical approaches for the removal of third ventricular colloid cysts (CC). MATERIAL AND METHODS: Records of 41 colloid cyst patients who underwent transcortical conventional microsurgical, microsurgical with the aid of stereotactically guided cylindrical retractor (minitubular), and endoscopic removal between 1999 and 2015 were retrospectively analyzed. The clinical results of these three different transcortical surgical approaches were compared and a literature review of published series was conducted. RESULTS: Ages ranged between 16 and 66 years (mean: 36.8). There were 18 female and 23 male patients. Headache was the main presenting symptom. The mean CC diameter was 15.5 mm. Intraventricular hemorrhage was encountered in 2 patients. Three patients needed postoperative ventriculoperitoneal shunt surgery. Postoperative seizures were seen in 3, postoperative neurological deficit in 3 and residual tumor in 2 patients. The conventional approach resulted in significantly higher rates of postoperative seizure compared to the minitubular and endoscopic approaches (p=0.012). The rate of residual cyst was significantly higher in endoscopic approach compared to conventional and minitubular approaches (p=0.024). Conventional approach led to significantly higher rates of neurological deficit compared to the two other approaches (p < 0.05). CONCLUSION: With respect to complication rates, the stereotactic and endoscopic approaches are both safe and reliable compared to conventional microsurgical approach that has unacceptably high rates of seizures and neurological deficit. Concerning completeness of removal, both microsurgical approaches are by far superior to neuroendoscopy. The stereotactic microsurgical approach compares favorably in both respects with endoscopic and conventional microsurgical approaches.

Superselective intra-arterial chemotherapy in the primary management of advanced intra-ocular retinoblastoma: first 4-year experience from a single institution in Turkey.

PURPOSE: To report our 4-year experience in Turkey, with advanced intra-ocular retinoblastoma managed primarily with intra-arterial chemotherapy (IAC). METHODS: From October 2011 to September 2015, 26 group D eyes of 24 treatment-naïve retinoblastoma patients managed primarily with IAC were evaluated in this prospective study. RESULTS: Of 76 procedures, ophthalmic artery cannulation failed in two patients with unilateral involvement. In the remaining 22 patients (24 eyes), the mean age at diagnosis was 18 months (range, 6-55 months). Each eye received a mean of 3 IAC sessions/eye (range, 2-5 sessions). After a median follow-up of 29 months (range, 6-55 months), complete regression of the main tumour was achieved in 23 of 24 eyes. One eye with partial regression required enucleation due to ciliary body involvement by the tumour. Overall, 16 eyes (66.6%) were salvaged with primary IAC with or without additional local treatments, and eight (33.3%) required enucleation. The main IAC-related periocular complications included transient eyelid oedema (n = 13), ptosis (n = 6) and forehead hyperpigmentation (n = 3), each resolving in 2 weeks to 4 months. Intra-ocular complications included chorioretinal atrophy (n = 9), newly noted retinal detachment (n = 5) and vitreous haemorrhage (n = 1). Kaplan-Meier eye estimates of enucleation-free survival rates were 83.3% (95% CI, 68.4-98.1%), 69.1% (95% CI, 49.8-88.3%) and 62.9 (95% CI, 41.9-83.8%) at 6 months, 1 and 2 years, respectively, and stable thereafter. CONCLUSION: Our first 4-year experience in Turkey showed that enucleation or external-beam radiotherapy could be avoided in two-thirds of eyes with advanced intra-ocular retinoblastoma managed primarily with IAC.

Surgical treatment of localized dissection of the internal carotid artery.

Dissection of the internal carotid artery is very rare; however, it is diagnosed more frequently with increasing radiographic diagnostic tools. Patients may be completely asymptomatic or may present with symptoms ranging between localized pain to severe cerebral ischemic events. Treatment is usually medical or with interventional radiographic tools. In this report, we present surgical management of internal carotid artery dissection in a 61-year-old female patient.

Treatment of ruptured blood blister-like aneurysms with flow diverter SILK stents.

INTRODUCTION: Blood blister-like aneurysms (BBAs) are fragile and difficult to treat. Routine surgical and endovascular treatment methods, such as clipping, clip wrapping, primary coiling, or stent assisted coiling, have relatively high morbidity and mortality rates. In this study, we report clinical and angiographic results for treatment of ruptured BBAs using flow diverter stents. METHODS: We retrospectively reviewed patients who presented with subarachnoid hemorrhages caused by rupture of BBAs and who were treated using flow diverter stents at three neurointervention centers between January 2009 and January 2013. Clinical and angiographic findings, together with the procedural data and follow-up results, are reported. RESULTS: 11 patients were identified in this study. BBAs were located on the supraclinoid internal carotid artery (nine patients) and basilar arteries (two patients). Eight patients were treated by implantation of a single flow diverter stent. Two flow diverter stents were telescopically deployed in each of three patients. The procedures were successful in all cases. No acute complications developed in any case. One patient who had an initial Hess-Hunt grade 4 died of septicemia 2 weeks after the procedure (9%). Another patient developed a minor stroke caused by parent artery thrombosis (9%). Control angiographies performed 3 and 6 months post stenting revealed complete occlusion of the aneurysms in all of the remaining nine patients (82%). 10 of the 11 patients (92%) had good clinical outcomes (modified Rankin Scale score ≤ 2). CONCLUSIONS: Implantation of flow diverter stents seems to be a safe and feasible alternative for treatment of ruptured BBAs.

Silent neurological involvement in biopsy-defined coeliac patients.

Coeliac disease (CD) is an autoimmune disease of small intestine associated with sensitivity to gluten. The clinical manifestations are often of gastrointestinal nature, although the disease may be present asymptomatically as well. It is a chronic disease and in the absence of overt neurological involvement, extended gluten exposure may give rise to silent or subtle morphological and white-matter changes in central nervous system. The present study investigates such changes using brain volumetry and the assessment of white-matter tissue in CD patients without neurological symptoms. Seventeen CD patients without any neurological involvement were included in the study and went under neurological evaluation and anatomical MRI. Individual gray- and white-matter, and subcortical structure volumes were acquired for using automated volumetric analyses. The observed white-matter hyperintensities (WMH) evaluated using Age-Related White-Matter Changes scale. Findings show a bilateral decrease in cortical gray-matter and caudate nuclei volumes in CD compared to controls. Negative correlations were found between the duration of the disease and the volumes of the affected regions. Cerebellum was seemingly unaffected. In addition, significantly higher proportion of WMH was found in CD patients, specifically in bilateral frontal and occipitoparietal cortices. We observed a significant gray-matter and caudate nucleus atrophy in the CD patients in the absence of marked neurological symptoms. Present findings point out to a need for histopathological investigations potentially focusing on anti-TG2 antibodies, and serial volumetric analyses on the CD-related cortical and subcortical changes.

Clinical features, prothrombotic risk factors, and long-term follow-up of eight pediatric Moyamoya patients.

BACKGROUND AND PURPOSE: The aim of this study was to elucidate the clinical features, prothrombotic risk factors, and outcome of pediatric Moyamoya patients. METHODS: Patients diagnosed with Moyamoya disease at a tertiary center between January 2000 and December 2006 were enrolled in this study. The clinical presentations, underlying diseases, prothrombotic risk factors, family history of thrombosis, radiological findings, treatment, and outcome of the patients were reviewed retrospectively. RESULTS: Eight patients with angiographically proven Moyamoya disease were identified, one of whom had neurofibromatosis type I and one had Down syndrome. The age at diagnosis varied between 19 months and 11 years (73.4±41.8 months, mean±SD). The follow-up period after diagnosis was 52.5±14.8 months. In six patients, the initial clinical presentation was hemiparesis. None of the patients had any identifiable prothrombotic factors. Despite medical and surgical treatment, three patients had recurrences and one died. Only two patients recovered without sequelae. CONCLUSIONS: The value of prothrombotic risk factor evaluation appears to be limited in Moyamoya patients; the outcome for pediatric patients remains dismal.

Myositic type of idiopathic orbital pseudotumor in a 4-year-old child: a case report.

Idiopathic orbital pseudotumor is a benign, noninfectious, and nonneoplastic disease with unknown cause. It is the third most common orbital disease after thyroid orbitopathy and lymphoproliferative disorder. Idiopathic orbital pseudotumor is extremely rare in pediatric age group and may cause real diagnostic problems. This paper describes a 4-year-old girl who presented with sudden ptosis in the right eye and swollen eyelid. She recovered completely with high-dose steroid therapy. We report clinical and magnetic resonance imaging findings of orbital myositis, which is a rare subtype of idiopathic orbital pseudotumor in children and needs to be differentiated from other orbital disease especially malignancy.

Localization of cerebrospinal fluid leaks by gadolinium-enhanced magnetic resonance cisternography: a 5-year single-center experience.

OBJECTIVE: Intrathecal gadolinium (Gd)-enhanced magnetic resonance (MR) cisternography is a newly introduced imaging method. Two main objectives of this study were to investigate the sensitivity of Gd-enhanced MR cisternography for presurgical localization of cerebrospinal fluid (CSF) leaks in patients with CSF rhinorrhea and to study the potential long-term adverse effects of intrathecal Gd application. METHODS: Fifty-one patients (19 women; mean age, 36.2 yr) with CSF rhinorrhea were included in the study. A total of 0.5 ml of Gd was injected into the lumbar subarachnoid space. T1-weighted MR cisternographic images were obtained to detect CSF leakage. The patient's neurological states and vital signs were recorded for the first 24 hours after the procedure. Neurological evaluations were repeated 1, 3, and 12 months after the procedure. The patients were followed for at least 3 years with annual neurological examinations. RESULTS: Gd-enhanced MR cisternography demonstrated CSF leaks in 43 of the 51 patients. The sensitivity of Gd-enhanced MR cisternography for localization of CSF leaks was 84%. Forty-four patients underwent surgery to repair dural tears. Surgical findings confirmed the results of Gd-enhanced cisternography in 43 of the 44 patients who underwent surgery (98%). Eight patients with negative Gd-enhanced MR cisternography had no active rhinorrhea at the time of procedure, and seven of them did not need surgery. None of the patients developed an acute adverse reaction that could be attributed to the procedure. None of the patients developed any neurological symptoms or signs caused by intrathecal Gd injection during a mean follow-up period of 4.12 years. CONCLUSION: Gd-enhanced MR cisternography is a sensitive and safe imaging method for detection of CSF leaks in patients with rhinorrhea.

Sporadic multiple cerebral cavernomatosis: report of a case and review of literature.

OBJECTIVE: Cerebral cavernous malformations (CCM) are vascular malformations causing seizures and cerebral hemorrhages. They occur in sporadic and familial forms. Familial cases are associated with a high frequency of multiple lesions, which are less frequently associated with sporadic cases. CASE REPORT: We report a 46-year-old woman presenting with epilepsy with multiple cerebral cavernomatosis on MRI. Because she had had a previous liver transplantation operation, and received immunosuppressants, she was not advised to have a brain operation. However, she had to be operated as a result of a bleeding in one of her cerebral cavernomas. The histologic diagnosis was cavernoma. She has been seizure free after the operation with levetiracetam therapy for the last 17 months. She had no positive family history for both epilepsy and cavernomatosis. CONCLUSION: When multiple cerebral cavernomatosis are identified in a patient, a detailed neurologic family history should be sought despite the possibility of its being a sporadic case. Our main intention is to present a patient who is surgically controversial and to point out the importance of genetic heredity.

An intrasellar germinoma with normal cerebrospinal fluid beta-HCG concentrations misdiagnosed as hypophysitis.

A patient with an intrasellar germinoma leading to pituitary stalk thickening is reported. The patient, a 24-year old woman, presented with hyperprolactinemia, secondary hypothyroidism, and hypogonadotropic hypogonadism with no evidence of diabetes insipidus. Cerebrospinal fluid (CSF) examination revealed an increased number of lymphocytes and histiocytes. Although beta-HCG concentration was normal (<2 mIU/mL) in the CSF, increased beta-HCG concentration was detected in the serum. Systemic glucocorticoid treatment led to a decrease in CSF cell count, but no regression of the sellar mass was noted. A diagnostic biopsy was performed and showed an intrasellar germinoma. The patient underwent conventional radiotherapy. Complete resolution of the mass lesion and normalization of beta-HCG concentration in the serum were observed three months after radiotherapy. The presence of intrasellar mass lesion in association with pituitary stalk thickening may cause difficulties in the differential diagnosis. Histopathological examination is essential in equivocal cases in order to reach accurate diagnosis and apply the most appropriate therapy.

Anesthesia following overfilling of a root canal sealer into the mandibular canal: a case report.

The surgical treatment of a case of anesthesia that occurred with the extrusion of Endomethasone root canal sealer into the mandibular canal is presented. Endomethasone is a neurotoxic root canal sealer containing paraformaldehyde and eugenol. The literature indicates immediate surgical decompression on the extrusion of Endomethasone into the mandibular canal. In our case, the decompression surgery was done 3 weeks after the endodontic mishap. The nearly complete resolution of anesthesia 4 months following the decompression surgery suggests that the neurotoxic effects of Endomethasone are still reversible after 3 weeks.

Evaluation of hemodynamic perfusion MR images.

Perfusion normally refers to the delivery of blood at the level of capillaries. Hemodynamic perfusion magnetic resonance (MR) imaging has been used in clinics for a series of applications including tumor characterization (histological type diagnosis and grading), diagnosis and the follow up of stroke, and several other disorders. For this study a platform to investigate the theoretical basis of perfusion imaging was developed. Using dynamic measurements of contrast agents in magnetic resonance imaging (MRI) relative cerebral blood volume (rCBV), relative cerebral blood flow (rCBF), and mean transit time (MTT) are calculated. These three parameters are quantized, and displayed as color images for diagnostic and follow up studies. The comparative studies in MR perfusion must address issues such as; image registration, region of interest (ROI) selection, threshold identification and quantization of rCBV, rCBF and rMTT. The evaluation process involved the comparison of the diagnostic capabilities of the three perfusion images (rCBV, rCBF, MTT). Digital Substraction angiography was used as the gold standard in these comparisons. The study group comprises 16 patients with the diagnosis of subarachnoid bleeding and intracranial aneurysms. The proposed cerebral MR perfusion analysis system has been accepted by the radiologists as a useful tool for their perfusion studies and clinical evaluation.

Surgical results in pediatric Spetzler-Martin grades I-III intracranial arteriovenous malformations.

OBJECTS: The goal of cerebral arteriovenous malformation (AVM) therapy in pediatric patients should be complete resection or obliteration of the AVM to eliminate subsequent hemorrhage, because of high mortality and morbidity rates related to hemorrhage in addition to the longer life expectation. Despite advances in Gamma knife radiosurgery and in endovascular embolization, surgical resection is still the gold standard for treating cerebral AVMs. METHODS: Between 1986 and 2003, 20 children were surgically treated for cerebral AVMs. The AVMs were graded I, II, and III using the Spetzler-Martin (S-M) Grading Scale. Good recovery was achieved in 18 out of 20 patients (90%) and only 1 patient was moderately disabled (5%). There was one mortality (5%) related to the preoperative deep comatose state of the patient. The total obliteration rate was 89% (17 out of 19). CONCLUSION: For S-M grade I-III AVMs, surgical resection is the treatment of choice, considering its high cure rate and low morbidity and mortality rates.

[A case of superior sagittal sinus thrombosis after closed head injury]. / Kapali kafa travmasi sonrasi gelisen süperior sagittal sinüs trombozu: Olgu sunumu.

Superior sagittal sinus thrombosis (SSST) is a rare entity, most often arising from infections, dehydration, and hematologic disorders. Development of this condition secondary to trauma is extremely rare. In this report, a 13-year-old boy who developed SSST following a closed head injury is presented. Imaging studies showed SSST caused by a depressed skull fracture. Neurologic examination of the patient was normal other than bilateral papillary stasis. He was treated with antiedematous and anticonvulsant drugs. Magnetic resonance venography obtained eight months after the diagnosis showed unoccluded superior sagittal sinus, neurologic examination findings were normal, as well.

Obsessive-compulsive disorder after epilepsy surgery.

It is a well-known fact that after epilepsy surgery (ES) preexisting psychopathology may deteriorate or de novo psychopathological syndromes, mainly of a depressive and psychotic nature, may appear. Previously, recovery of obsessive-compulsive disorder (OCD) after ES has been reported in patients who had comorbid OCD preoperatively; however, there have been no reports on the appearance of de novo OCD interfering with daily living activities post-ES. This is the first report of OCD after ES in patients with mesial temporal lobe epilepsy (MTLE). Five patients with MTLE were identified with obsessive personality traits before surgery. Within the first 2 months after ES, two of these MTLE patients fulfilled OCD diagnostic criteria. These OCD patients were not any different from the other three patients with respect to age, age of onset of epilepsy, seizure types, and seizure frequency. All patients stopped having seizures postoperatively, but the OCD patients had worse quality of life postoperatively than preoperatively. Our findings show that those patients with obsessive traits preoperatively should be carefully monitored after ES.

[Clinical and magnetic resonance imaging findings in patients with surgically treated mesial temporal sclerosis]. / Cerrahi uygulanan mezial temporal skleroz olgularinda klinik ve MRG bulgular.

PURPOSE: Mesial temporal sclerosis (MTS) is one of the leading causes of medically intractable complex partial seizures. Magnetic resonance imaging (MRI) and clinical findings in seventeen patients who had surgery for medically intractable mesial temporal lobe epilepsy (MTLE) are presented in this report. MATERIALS AND METHODS: 17 patients (9 females and 8 males) aged 19-35 years with clinically and electrophysiologically proven MTLE have been included in the study. Age at seizure onset was six months to 17 years, frequency of seizures 4-20 times/month, and duration of medical treatment 10-28 years with no response. MRI and volumetry of the mesial temporal structures were performed in all patients. RESULTS: On preoperative MRI and volumetry, pathologic signal, loss of internal structures and atrophy were present in the right hippocampus in twelve cases and left hippocampus in five. The hippocampal head and body were involved in ten cases and all three parts in seven. The Wada test was performed in three cases showing that memory and speech functions were controlled by the noninvolved side. All patients underwent surgery. The histopathological examination result was MTS in all cases. In the postoperative follow up period of one to 24 months, 12 patients were assessed as class I and II, and seven as III according to the Engel Classification. CONCLUSION: MRI and volumetry are among the most important tools in the preoperative diagnosis of MTS. All patients have benefited from surgery, verifying the preoperative clinical and MR imaging diagnosis. Interpretation of MR findings is important in determining surgical candidates and success of surgery in MTS, which is a major cause of medically intractable temporal epilepsy.