Novel use of propranolol for management of pain in children with vertebral hemangioma: report of two cases.

INTRODUCTION: Vertebral hemangioma (VH) is an exceedingly rare neoplasm in pediatric population with less than 10 cases reported in the literature. It is usually asymptomatic in adults and diagnosed incidentally at radiographic investigations of other medical conditions. In this report, we describe two children who presented to our institution with severe back pain and were diagnosed with VH. CASE REPORTS: Case 1 was an 8-year-old male with a pain score of 10 out of 10 at presentation. Clinical investigations eliminated the possibility of a neoplasm or infectious process and MRI findings were highly suggestive of an aggressive vertebral hemangioma. Case 2 was a 17-year-old female who presented with back pain radiating to shoulders. Her pain score was 4 out of 10 and she was diagnosed with vertebral hemangioma due to the specific findings on MRI studies. DISCUSSION: Both patients received propranolol with a dose of 20 and 40 mg per day, respectively. They were free of pain at 2 months follow-up. There are different invasive treatment modalities for the management of VH, including vertebroplasty, kyphoplasty, radiotherapy, alcohol injection, embolization, and surgery. These methods have been used in adult patients for several years, but each of them has potential risks which make these options unsuitable for children. CONCLUSION: Propranolol is a beta blocker which is safely used in the management of infantile hemangiomas. This is the first report demonstrating its efficacy in symptomatic treatment of childhood VH. The lesions did not show any regression, but the pain relief obtained was very significant under propranolol therapy.

White matter abnormalities in 15 subjects with SPG76.

BACKGROUND AND OBJECTIVES: Hereditary spastic paraplegias (HSPs) are heterogenous genetic disorders characterized by progressive pyramidal tract involvement. SPG76 is a recently identified form of HSP, caused by biallelic calpain-1 (CAPN1) variants. The most frequently described MRI abnormality in SPG76 is mild cerebellar atrophy and non-specific white matter abnormalities were reported in only one case. Following the identification of prominent white matter abnormalities in a subject with CAPN1 variants, which delayed the diagnosis, we aimed to verify the presence of MRI patterns of white matter involvement specific to this HSP. METHODS: We performed a retrospective radiological qualitative analysis of 15 subjects with SPG76 (4 previously unreported) initially screened for white matter involvement. Moreover, we performed quantitative analyses in our proband with available longitudinal studies. RESULTS: We observed bilateral, periventricular white matter involvement in 12 subjects (80%), associated with multifocal subcortical abnormalities in 5 of them (33.3%). Three subjects (20%) presented only multifocal subcortical involvement. Longitudinal quantitative analyses of our proband revealed increase in multifocal white matter lesion count and increased area of periventricular white matter involvement over time. DISCUSSION: SPG76 should be added to the list of HSPs with associated white matter abnormalities. We identified periventricular white matter involvement in subjects with SPG76, variably associated with multifocal subcortical white matter abnormalities. These findings, in the presence of progressive spastic paraparesis, can mislead the diagnostic process towards an acquired white matter disorder.

Assessment of CSF flow dynamics using PC-MRI in spontaneous intracranial hypotension.

AIM: Spontaneous intracranial hypotension (SIH) is caused by spontaneous cerebrospinal fluid (CSF) leaks and is known to cause orthostatic headaches. Phase-contrast magnetic resonance imaging (PC-MRI) is a non-invasive technique that can be used to quantify variation in CSF flow. The aim of this study was to assess CSF flow dynamics using PC-MRI in SIH. MATERIALS AND METHODS: Twenty-five patients with a definitive diagnosis of SIH and 25 healthy subjects were evaluated with PC-MRI. Magnetic resonance (MR) images were acquired using a 1.5-T unit with an 8-channel head coil. Differences between SIH patients and control subjects were assessed statistically using Wilcoxon's rank sum test, Spearman's rho test, or Pearson's chi-square test, as appropriate. RESULTS: CSF flow volumes toward the third ventricle, CSF flow volumes toward the fourth ventricle, the absolute stroke volume, the peak systolic velocity, and the peak diastolic velocity in SIH patients were significantly smaller than those in control subjects (P < .0001). On the other hand, the net CSF flow volume (P = .9227) and the net CSF flow direction (P = .2472) for SIH patients and control subjects were not significantly different. CONCLUSIONS: The results obtained by CSF flow analysis were directly related to values of CSF opening pressure, determined by lumbar puncture, and clinical findings, such as headache scores. Thus, CSF flow analysis with PC-MRI, which has a short performance time and is non-invasive, may contribute to assessment of SIH patients.

Diffusion-weighted imaging findings in brain death.

INTRODUCTION: The purpose of the present study was to determine the role of diffusion-weighted imaging (DWI) and to investigate the use of DWI in the diagnosis of brain death (BD). METHODS: We prospectively evaluated 22 patients diagnosed with clinical BD (9 women, 13 men; mean age, 39.63 ± 15.1 years; age range, 9-66 years). All clinical criteria for BD were present in all 22 patients before magnetic resonance imaging, including a positive apnea test. For all cases, DW images, T2-weighted images, and fluid-attenuated inversion recovery were obtained. Thirteen distinct neuroanatomical structures were selected for analysis in all the cases. For each region of interest, the mean, standard deviation, and range of the average apparent diffusion coefficient (ADCav) values were obtained. RESULTS: For BD patients, ADC values in all neuroanatomical structures were significantly lower than those for control subjects. We determined how ADC values in all structures were related to the diagnostic condition as well as the appropriate threshold ADC values to classify a subject as BD or control. The sensitivity, specificity, positive and negative predictive values, and correct classification rate of ADC cutoff values to distinguish BD from control groups were 100%. CONCLUSIONS: DWI might be used as a noninvasive confirmatory test for the diagnosis of BD in the future.

A case of primary Sjögren's syndrome presenting primarily with central nervous system vasculitic involvement.

Sjögren's syndrome is primarily a chronic systemic autoimmune disease that affects exocrine organs. Neurologic symptoms frequently present as peripheral neuropathy due to small vessel vasculitis. Type and prevalence of central nervous system involvement are still controversial. In this report, we present a 35-year-old woman with primary Sjögren's syndrome with central nervous system vasculitic involvement.

Magnetic resonance imaging of rheumatoid meningitis: a case report and literature review.

Rheumatoid meningitis is a rare and serious complication of rheumatoid arthritis (RA) with high mortality rate. Clinical importance of the disease is high because diagnosis is difficult, and the disease is treatable if diagnosed successfully. We present the clinical and cranial magnetic resonance imaging findings of 62-year-old female patient with RA who has been followed up for 4 years.

CT-guided percutaneous translaminar approach for blood patching: case report and technical note.

In this article the authors describe a novel technique for performing epidural blood patch (EBP) by percutaneous CT-guided translaminar approach in challenging cases where interlaminar approach is not possible. A 24-year-old woman with medical history of multiple spinal surgeries and instrumentations for the treatment of scoliosis, presented 3 months post-operatively with acute and severe orthostatic headaches that began 1 week after surgery. Neurological examination was normal. Brain magnetic resonance imaging (MRI) showed mild thickening and contrast enhancing in the bilateral dura. Computed tomography (CT) myelography revealed CSF leakage in the level of T3 vertebra. EBP was attempted using fluoroscopic and then CT guidance; however, despite multiple attempts, the epidural space could not be accessed through the interlaminar route due to extensive instrumentation of the spine and profound structural bony abnormalities. EBP was performed successfully via a CT-guided translaminar approach using an Ostycut trephine needle (Angiomed(®)/Bard, Karlsruhe), without complications.

Spontaneous intracranial hypotension due to intradural thoracic osteophyte with superimposed disc herniation: report of two cases.

Spontaneous intracranial hypotension (SIH) is a clinical syndrome in which absolute or relative hypovolemia of the cerebrospinal fluid (CSF) results in various neurological symptoms. The etiology of spontaneous CSF leaks often remains unknown. However, it is believed that the most common cause is the fragility of spinal meninges at the level of radicular nerve root sleeve. These tears can be spontaneous (primary) or secondary. Spinal pathologies can cause this tear with resultant CSF leak and SIH, which include spinal trauma, degenerative diseases and spinal surgery. Uncommonly, SIH is developed by osteophyte with disc herniation without any other pathology. In this article, we reported two cases of SIH secondary to spinal dural tear due to intradural thoracic osteophyte with superimposed disc herniation, with the absence of other pathologies, which were treated successfully with epidural blood patch (EBP).

Primary intracranial abscess localized in the basal ganglia: a case report.

Brain abscesses are serious infections that cause neurological problems and generally require antibiotic treatment and surgical drainage. Hematogenous brain abscesses are generally located in the region fed by the middle cerebral artery, but are occasionally found in deep tissues, such as the basal ganglia and thalamus.

Pathological contrast enhancement of the oculomotor and trigeminal nerves caused by intracranial hypotension syndrome.

The typical symptom of intracranial hypotension syndrome is orthostatic headache. The headache may also be accompanied by neck pain and stiffness, low backache, radicular symptoms, quadriplegia, interscapular pain, nausea/vomiting, and cranial nerve involvement symptoms (hearing and visual problems, face pain and numbness, hypogeusia). Radiologically, on cranial magnetic resonance imaging, intracranial hypotension syndrome is characterized by dural thickening and contrast enhancement, subdural effusion, engorgement of the venous structures, sagging or downward displacement of the brain, and pituitary hyperemia. Although clinical findings related to cranial nerves 3 and 5 have been described in intracranial hypotension, pathological contrast enhancement of these nerves has not. We present a 32-year-old patient whose cranial magnetic resonance imaging shows bilateral pathological contrast enhancement of cranial nerves 3 and 5 and describe a new imaging finding in intracranial hypotension syndrome.

In Vivo Evaluation of the Biomechanical Properties of Optic Nerve and Peripapillary Structures by Ultrasonic Shear Wave Elastography in Glaucoma.

BACKGROUND: Primary open-angle glaucoma is a multifactorial serious disease characterized by progressive retinal ganglion cell death and loss of visual field. OBJECTIVES: The purposes of this study were to investigate shear wave elastography (SWE) use in the evaluation of the optic nerve (ON) and peripapillary structures, and to compare the findings between glaucomatous and control eyes. PATIENTS AND METHODS: A case-controlled study, including 21 patients with primary open-angle glaucoma and 21 age-matched control subjects, was carried out. All of the participants had comprehensive ophthalmological exams that included corneal biomechanical measurements with ocular response analyzer. In vivo evaluation of the biomechanical properties of the ON and peripapillary structures were performed with SWE in all participants. The Kolmogorov-Smirnov test was used to analyze the normal distribution of data. Differences of parameters in ophthalmologic data and stiffness values of patients with and without glaucoma were evaluated using the Mann-Whitney U test. RESULTS: There were no statistically significant differences between the glaucoma and control groups in terms of age (P > 0.05) and gender (P > 0.05). Corneal hysteresis was lower in the glaucoma group (P < 0.05). Corneal compensated intraocular pressure and Goldmann correlated intraocular pressure were higher in the glaucoma group (P < 0.0001 for both). The mean stiffness of the ON and peripapillary structures were significantly higher in glaucoma patients for each measured region (P < 0.05). CONCLUSION: The study evaluated the biomechanical properties of the ON and peripapillary structures in vivo with SWE in glaucoma. We observed stiffer ON and peripapillary tissue in glaucomatous eyes, indicating that SWE claims new perspectives in the evaluation of ON and peripapillary structures in glaucoma disease.

Frequency of Nerve Root Sleeve Cysts in Autosomal Dominant Polycystic Kidney Disease.

BACKGROUND: There is sporadic data about the occurrence of spinal meningeal cysts in patients with autosomal dominant polycystic kidney disease (ADPKD). We suggest that there is a relationship with the frequency and size of spinal meningeal cysts and headache, intracranial aneurysms, and cerebrospinal fluid leakage in patients with ADPKD. AIM: To investigate the relationship with spinal meningeal cyst, cerebrospinal fluid leakage, and headache in patients with ADPKD. STUDY DESIGN: Cross-sectional study. METHODS: We enrolled 50 patients with ADPKD and 37 healthy volunteers. This cross-sectional study included patients with ADPKD and matched healthy volunteers. Magnetic resonance imaging myelography was performed using the 3D-T2 HASTE technique in an MRI scanner. We questioned our subjects regarding presence of headache and evaluated headache severity using a visual analog scale. The relationship between the number and size of spinal meningeal cysts with headache, intracranial aneurysms, and liver cysts was also investigated. RESULTS: Spinal meningeal cysts were more numerous and larger in patients than in controls (14.8±11.6 vs. 6.4±4.6 cysts respectively, p<0.001, 68.3±49.3 vs. 25.4±20.1 mm, p<0.001, respectively). Spinal cyst number and size were similar in APDKD patients with or without intracranial aneurysms. Headache score was correlated with the size and number of spinal meningeal cysts. This was valid only in patients with ADPKD. CONCLUSION: Abnormality involving the vessel wall in ADPKD may explain the increased number of spinal meningeal cysts in ADPKD. Moreover, leakage of cerebrospinal fluid secondary to spinal meningeal cyst may be responsible for recurrent severe headache by causing spontaneous intracranial hypotension in these patients.

Primary Psammomatous Melanotic Schwannoma of the Spine.

Schwannoma is an easily identifiable and frequently diagnosed lesion of the spinal column. However, if the schwannoma contains a melanin component, the diagnosis is challenging. Our purpose in this case report is to discuss the imaging and histopathologic findings of a rarely seen psammomatous type of melanotic schwannoma diagnosed in a 31-year-old woman.

Aneurysmal Bone Cyst of Sphenoid Bone and Clivus Misdiagnosed as Chordoma: A Case Report.

Aneurysmal bone cysts (ABCs) are benign and rapidly expanding bone destructive lesions of any bone. They are commonly localized in the metaphysis of long bones, whereas skull base ABCs are rare. We report a case of a 21-year-old man who had been misdiagnosed as chordoma and undergone surgery. However, histopathological examination revealed it to be an ABC.

The prevalence of pineal cyst in patients with cerebral palsy.

PURPOSE: Pineal cysts are common incidental findings during magnetic resonance imaging (MRI) examinations. The etiology of pineal cyst development is still unclear. We aimed to determine whether there is an association between periventricular leukomalacia and pineal cyst prevalence. METHODS: Clinical and MRI data of 201 patients with periventricular leukomalacia (110 female, 91 male; mean age, 6 years; range, 2-18 years) and 687 control patients (355 female, 332 male; mean age, 6 years¸ range, 2-18 years) who did not have any evidence of periventricular leukomalacia were independently evaluated by two radiologists for presence or absence of pineal cyst. RESULTS: Pineal cysts were detected in 32.3% of the study group (65/201) and 8.4% of the control group (58/687) (P < 0.001). Patients with periventricular leukomalacia were more likely to have a pineal cyst. In terms of pineal cyst detection on MRI, interobserver reliability was high between the two radiologists. CONCLUSION: The prevalence of pineal cysts is higher in patients with periventricular leukomalacia. We suggest that an ischemic process may have a role in the etiopathogenesis of pineal cyst development.

Primary diffuse leptomeningeal gliomatosis mimicking tuberculous meningitis.

Primary diffuse leptomeningeal gliomatosis is a disease with an aggressive course that can result in death. To date, 82 cases have been reported. Here, the case of a 3-year-old male patient presenting with strabismus, headache, and restlessness is reported. Physical examination revealed paralysis of the left abducens nerve, neck stiffness, and bilateral papilledema. Tuberculous meningitis was tentatively diagnosed, and antituberculosis treatment was initiated when cranial imaging revealed contrast enhancement around the basal cistern. Craniocervical magnetic resonance imaging (MRI) was performed when there was no response to treatment, and it revealed diffuse leptomeningeal contrast enhancement around the basilar cistern, in the supratentorial and infratentorial compartments, and in the spinal region. Primary diffuse leptomeningeal gliomatosis was diagnosed by a meningeal biopsy.

Multiple small hyperintense lesions in the subcortical white matter on cranial MR images in two Turkish brothers with cold-induced sweating syndrome caused by a novel missense mutation in the CRLF1 gene.

Cold-induced sweating syndrome (CISS) is a rare autosomal recessive disorder characterized by excess sweating induced by cold exposure, camptodactyly and kyphoscoliosis. CISS is genetically heterogeneous. Deficiency of the CRLF1 or the CLCF1 gene function results in one of two clinically indistuinguishable disorders called CISS1 and CISS2, respectively. We present two Turkish brothers (22 and 13 years old) who had excess sweating induced by cold exposure, severe dorsal scoliosis, camptodactyly, reduced pain sensitivity and marfanoid habitus. The patients were homozygous and their parents heterozygous for a novel missense mutation c.413C>T (p.Pro138Leu) in CRLF1 gene. The cranial magnetic resonance imaging (MRI) of two patients also showed multiple small hyperintense lesions in the subcortical white matter. Similar MRI finding has also been reported in a Japanese woman with CISS1 and marfanoid habitus. The lesions found in the present cases showed no characteristic features. However, multiple small hyperintense lesions in subcortical white matter on T2 weighted and fluid attenuation inversion recovery (FLAIR) images may support the clinical diagnosis of CISS.

Cavernous hemangioma of the cavernous sinus misdiagnosed as a meningioma: a case report and MR imaging findings.

Cavernous hemangioma (CH) is a benign vascular malformation. Intracranial CH is generally localized as an intracranial-intraaxial and responsible for 5-13% of all intracranial vascular malformations. Intracranial-extraaxial CHs are rare rather than intracranial-intraaxial CHs. Clinical findings, imaging characteristics, and surgical approach of extraaxial CHs are rather different than intraaxial CHs. Diagnosing cavernous sinus CH preoperatively is very important, but its radiological differential diagnosis is quite difficult. In this study, we present magnetic resonance imaging findings of a 48-year-old male who was considered preoperatively to have meningioma but was diagnosed with cavernous sinus CH during surgery by pathological examination.

Total agenesis of superior sagittal sinus and falx cerebri in a patient who has a subacute subdural hematoma crossing midline: case report.

BACKGROUND AND IMPORTANCE: Anatomic variations of the superior sagittal sinus (SSS) and falx cerebri (FC) are uncommon in that agenesis of these structures is extremely rare. We report an extremely rare anatomic variation, total agenesis of the SSS and FC, and briefly discuss it from the anatomical, embryological, radiological, and clinical perspectives. CLINICAL PRESENTATION: A 49-year-old woman presented with long-standing headache, gait disturbance, and nausea. Imaging studies showed a bilateral subdural hematoma crossing the midline, dilated venous structures, and perineural cysts, but SSS and FC. Following right-sided hemiparesis and consciousness disturbances, the subdural hematoma was evacuated from a left-sided parietal burr hole because of thick hematoma in this side. After the surgical evacuation, the hemiparesis and consciousness disturbances were regressed; however, she still had severe headache. On account of ongoing headaches and related imaging findings, it was thought that she had possible spontaneous intracranial hypotension. She was treated with autologous epidural blood patch and recovered well. CONCLUSION: Agenesis of the SSS and FC are extremely rare variations. Agenesis of the SSS results in development of alternative venous pathways and may lead to misdiagnosis as dural arteriovenous fistulas. Agenesis of FC may cause diagnostic confusion, because subdural pathologies such as hematomas can cross the midline in rare occasions.