T2 Signal Hyperintensity Research Articles

Magnetic resonance imaging features of parotid mucoepidermoid carcinoma: a retrospective analysis

Background: Parotid mucoepidermoid carcinoma (P-MEC) is the most common of malignant salivary gland tumors and its accurate preoperative diagnosis is crucial for better management and prognostication. This becomes even more critical because in most cases P-MEC is difficult to distinguish from other common benign parotid lesions including abscess both clinically and radiologicall, but where management is entirely different. MRI could prove a vital cog in the wheel in this whole endeavor. This study aims to analyse the various MRI features in parotid mucoepidermoid carcinoma and the control group of non-MEC benign parotid masses which will aid in better distinguish between the two entities thereby coming to a confident diagnosis which will aid in management planning and better prognosis. Methods: This retrospective study was conducted at Sree Uthradom Thirunal Academy of Medical Science (SUTAMS), Thiruvananthapuram, India from July 2021 to July 2022 after obtaining Institutional Ethical committee approval. An informed consent was obtained from all patients before conduct of MRI imaging. The various MRI findings was applied in 60 patients in age bracket of 45-90 yrs with HPE proven parotid gland tumors. The cohort was divided into 2 groups: MEC (n=30) and non–MEC (n=30) as a control group. Results: Logistic regression analysis identified presence of cystic component (p<0.001), T1 hypointense signal (p<0.001), T2 hyperintense signal (p<0.001), heterogeneous post contrast enhancement (p<0.001) and perineural invasion (p=0.002) as characteristic MRI features of parotid MEC that aided in its accurate distinction from other non-MEC lesions of parotid. Conclusions: MRI can be a valuable tool for reliably distinguishing parotid mucoepidermoid from other parotid lesions.

Epileptic Amnesia as an Alzheimer’s Disease Mimic (P2-6.004)

<h3>Objective:</h3> To present a case of amnestic dementia that was caused as a result of mesial temporal lobe epilepsy. <h3>Background:</h3> Memory impairments have been reported as a result of mesial temporal lobe epilepsy. Dementia as a result of long term uncontrolled seizures has been reported. Here we report a patient presenting for evaluation of dementia - diagnosed with uncontrolled mesial temporal lobe epilepsy. <h3>Design/Methods:</h3> A 76 year old male presented to the cognitive clinic for evaluation of amnestic problems that started approximately 4 years prior. The patient reported problems with both long-term and short-term memory–such as details of family vacations, weddings, names of acquaintances. He additionally reported mild word finding abnormalities and spatial difficulty. Neuropsychological testing revealed problems in non contextual verbal learning and memory. The patient notably reported stereotyped events which also started around 4 years prior.–which he described as getting a sense of deja vu followed by tingling in all 4 limbs, nausea and a changed sense of smell. These events occurred around 10 times a month. MRI brain performed 4 years prior had revealed some small vessel ischemic changes and diffuse volume loss. An EEG obtained at the time had been unremarkable. Repeat MRI brain was significant for left hippocampal atrophy with bilateral hippocampal T2 signal hyperintensities. Ambulatory EEG monitoring was significant for transient epileptiform discharges. Lumbar puncture was unremarkable with tau /amyloid profiles not consistent with Alzheimer9s pathology. The patient was started on Levetiracetam with cessation of his episodes of deja vu, tingling and nausea. <h3>Results:</h3> NA <h3>Conclusions:</h3> Uncontrolled mesial temporal lobe epilepsy can result in hippocampal atrophy and cause amnestic problems. Epilepsy should be a diagnostic consideration in patients who present for evaluation of dementia. <b>Disclosure:</b> Dr. Philip has nothing to disclose. Dr. Chabrashvili has nothing to disclose.

Atypical Posterior Reversible Encephalopathy Syndrome with Obstructive Hydrocephalus and Impending Tonsillar Herniation. (P6-5.030)

<h3>Objective:</h3> <h3>OBJECTIVE AND IMPORTANCE:</h3> Rhombencephalic edema with hydrocephalus has rarely been described as a manifestation of hypertensive emergency. We present an unusual case of brain edema with hydrocephalus secondary to hypertensive emergency. <h3>Background:</h3> <h3>CASE DESCRIPTION:</h3> A 33-year-old male with uncontrolled hypertension and CKD Stage IV presented to hospital with occipital headache and vomiting. His BP was 230/160. He was treated with Nicardipine infusion. CT head showed cerebellar vasogenic edema and mild tonsillar herniation with partial effacement of the fourth ventricle. MRI confirmed these findings, there was disproportionate dilatation of the lateral and third ventricles with periventricular T2 hyperintense signal consistent with transependymal CSF flow. The headache and neck pain were persistent. The patient was upgraded to Neurocritical Care Unit for monitoring and continued IV BP treatment. Given concerns for incipient tonsillar herniation, Mannitol and Hemodialysis [HD] were added to his medical treatment. After these interventions, his headache and blood pressure management improved. Two days later, CT showed significant interval improvement of hydrocephalus, cerebral edema and posterior fossa crowding. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> <h3>DISCUSSION:</h3> This was an unusual case of extension of PRES to the posterior fossa, leading to hydrocephalus and a vicious cycle of worsening rise in intracranial pressure, leading to worsening refractory systemic hypertension, end organ damage, need for hemodialysis, impending tonsillar herniation and risk of death. <h3>Conclusions:</h3> <h3>CONCLUSION:</h3> This presentation and improvement were compatible with the final diagnosis of an atypical presentation of Posterior Reversible Encephalopathy Syndrome. In this case, reduction of blood pressure, hyperosmolar therapy and dialysis resulted in improvement of the symptoms as well as radiological resolution of hydrocephalus. Aggressive medical treatment was the best approach in this case, but neurosurgical intervention would have been necessary if the patient had not responded to these measures. <b>Disclosure:</b> Dr. Solano has nothing to disclose. Dr. Zafar has nothing to disclose. Dr. Newman has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Clyde Bergstresser, LLC. Dr. Newman has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Maria Rubio LLC. The institution of Dr. Newman has received research support from Albert Einstein Society.

Teaching NeuroImages: Spinal xanthomatosis

A 57-year-old woman presented with a 20-year history of progressive lower extremity weakness, spasticity, and proprioception deficits. She was given a diagnosis of primary progressive multiple sclerosis at age 38. Her Achilles tendons were enlarged (figure 1). Brain MRI was normal. Spine MRI demonstrated T2-hyperintense signal involving the posterior and lateral columns (figure 2). Serum cholestanol level was elevated. CYP27A1 gene sequencing revealed 2 pathogenic variants, c.1183C>T(p.Arg395Cys) and c.410G>A(p.Arg137Gln), confirming the diagnosis of cerebrotendinous xanthomatosis (CTX). Spinal xanthomatosis is a rare variant of CTX presenting with progressive corticospinal and posterior column signs.1 Early treatment with chenodeoxycholic acid may improve outcomes.2

Teaching NeuroImages: Cerebrotendinous xanthomatosis

A 39-year-old previously healthy man presented with insidiously progressive paresthesia in his lower extremities and worsening of gait and balance. MRI demonstrated T2-hyperintense signal abnormalities involving the thalami, midbrain, dentate nuclei, and adjacent deep cerebellar white matter, which are characteristic of cerebrotendinous xanthomatosis (CTX) (figure). Magnetic resonance spectroscopy revealed typical lipid peaks at 0.9 and 1.3 ppm,1 and serology analysis indicated sterol 27-hydroxylase deficiency,2 confirming the diagnosis. CTX is a rare lipid storage disease with unique clinical and imaging findings, and effective treatment with positive outcomes if initiated early, raising the importance of awareness and early recognition of this disease.2

Variant Sciatic Nerve Anatomy in Relation to the Piriformis Muscle on Magnetic Resonance Neurography: A Potential Etiology for Extraspinal Sciatica

To assess the prevalence and clinical implications of variant sciatic nerve anatomy in relation to the piriformis muscle on magnetic resonance neurography (MRN), in patients with lumbosacral neuropathic symptoms. In this retrospective single-center study, 254 sciatic nerves, from 127 patients with clinical and imaging findings compatible with extra-spinal sciatica on MRN between 2003 and 2013, were evaluated for the presence and type of variant sciatic nerves, split sciatic nerve, abnormal T2-signal hyperintensity, asymmetric piriformis size and increased nerve caliber, and summarized using descriptive statistics. Two-tailed chi-square tests were performed to compare the anatomical variant type and clinical symptoms between imaging and clinical characteristics. Sixty-four variant sciatic nerves were identified with an equal number of right and left variants. Bilateral variants were noted in 15 cases. Abnormal T2-signal hyperintensity was seen significantly more often in variant compared to conventional anatomy (40/64 vs. 82/190; p = 0.01). A sciatic nerve split was seen significantly more often in variant compared to conventional anatomy (56/64 vs. 20/190; p < 0.0001). Increased nerve caliber, abnormal T2-signal hyperintensity, and asymmetric piriformis size were significantly associated with the clinically symptomatic side compared to the asymptomatic side (98:2, 98:2, and 97:3, respectively; p < 0.0001 for all). Clinical symptoms were correlated with variant compared to conventional sciatic nerve anatomy (64% vs. 46%; p = 0.01). Variant sciatic nerve anatomy, in relation to the piriformis muscle, is frequently identified with MRN and is more likely to be associated with nerve signal changes and symptomatology.

Primary synovial sarcoma on MRI - a case series and review of the literature.

Simple MRI features are mandatory to facilitate the diagnostics of synovial sarcomas, especially for radiolo-gists outside multidisciplinary sarcoma centres. In this case-series and review, we investigate the main appearance of synovial sarcoma on MRI. Fifteen histologically proven primary synovial sarcomas who underwent MRI at 2 different sarcoma centres were included in this case series. Patients and their primary synovial sarcomas were examined for age, localization, mean tumour size (in mm), histological grade (G - according to the Fédération Nationale des Centres de Lutte Contre Le Cancer [FNCLCC]), configuration, T2 signal intensity, presence/absence of "triple sign", heterogeneity/homogeneity, borders (well-defined or infiltrative), and intensity of contrast enhancement on MRI. Additionally, a comprehensive literature review to identify observational studies, reviews, and case-reports assessing MRI features of primary synovial sarcoma was performed. The mean age of the patients was 47.6 years (SD: 17.2). The mean size of primary synovial sarcoma was 59.3 mm (SD: 42). Primary synovial sarcomas were significantly most often multilobulated (n = 12, p < 0.01), heterogeneous (n = 10), and infiltrative (n = 9). Additionally, 3 other primary synovial sarcomas showed the following configurations: ovoid/nodular (n = 2) and fascicular (n = 1). Ovoid/nodular synovial sarcomas were solely depicted as homogeneous with well-defined borders. All tumours showed T2 hyperintense signal and presented with marked contrast enhancement. Primary synovial sarcomas are mainly multilobulated, heterogeneous, and infiltrative tumours. In minor cases, ovoid/nodular or fascicular configurations occur. Knowing the main appearance of synovial sarcoma can help facilitate the diagnostics of primary synovial sarcomas.

AN INHERITABLE STROKE DISORDER PRESENTING WITH UNIPOLAR PSYCHOTIC DEPRESSION: CASE REPORT OF A RARE ENTITY

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant rare hereditary angiopathy that causes stroke in young patients. Most frequent clinical presentations include ischemic strokes, recurrent transient ischaemic attacks and migraine with aura. Psychiatric manifestations are conspicuously rare and especially if present as the only presenting manifestation. Here is a case of a female patient in her fties who was diagnosed with major depressive disorder with psychotic features and was on medication for the same for one and a half years with no improvement in symptoms. She was subsequently admitted due to an increased tendency to self-harm and on day 6 of admission had an episode of seizures, which led us to look for organic causes. After a battery of tests and ruling out various differentials, she underwent an MRI which revealed T2 hyperintense signal in bilateral frontal, temporal, parieto-occipetal, and periventricular white matter. Based on MRI ndings, family history of her father passing away due to a stroke at age 68 and poor response to antidepressants, she was suspected to have CADASIL. She underwent genetic testing which conrmed the same. The clinical course and distinct facts of the case are divulged below.

Basal ganglia dysplasia and mTORopathy: A potential cause of postoperative seizures in focal cortical dysplasia.

Pathogenic somatic MTOR variants in the cerebral cortex are a frequent cause of focal cortical dysplasia (FCD). We describe a child with drug and surgery-resistant focal epilepsy due to FCD type II who developed progressive enlargement and T2 signal hyperintensity in the ipsilateral caudate and lentiform nuclei. Histopathology of caudate nucleus biopsies showed dysmorphic neurons, similar to those in resected cortex. Genetic analysis of frontal and temporal cortex and caudate nucleus identified a pathogenic somatic MTOR variant [NM_004958.4:c.4375G > C (p.Ala1459Pro)] that was not present in blood-derived gDNA. The mean variant allele frequency ranged from 0.4% to 3.2% in cerebral cortex and up to 5.4% in the caudate nucleus. The basal ganglia abnormalities suggest more widespread, potentially hemispheric dysplasia in this patient, consistent with the pathogenic variant occurring in early cerebral development. This finding provides a potential explanation for persistent seizures in some patients with seemingly complete resection of FCD or disconnection of a dysplastic hemisphere.

Clinical Characteristics of Methanol-Induced Optic Neuropathy: Correlation between Aetiology and Clinical Findings

To show the clinical characteristics, identify the magnetic resonance imaging (MRI) and optical coherence tomography (OCT) features, and observe the visual outcome of methanol-induced optic neuropathy. Clinical data were retrospectively collected from in-patients diagnosed with methanol-induced optic neuropathy in the Neuro-Ophthalmology Department of the Chinese People's Liberation Army General Hospital from January 2016 to January 2021. Eight patients were included in this study. The exposure time was 6-34 h for ingestion, 3-4 months for inhalation, and more than ten years for skin absorption. All patients demonstrated bilateral acute visual impairment. Seven of eight patients had other accompanying systemic symptoms. Seven of eight patients demonstrated optic nerve lesions in MRI, and five presented with a hyperintense T2 signal in a "central" type. OCT showed the macular ganglion cell layer and inner plexiform layer (mGCL-IPL) thinning before the peripapillary retinal nerve fiber layer (pRNFL) thinning. The visual improvement was achieved transiently for seven of eight patients after treatment. One patient with a mitochondrial DNA mutation maintained a bilateral no-light perception (NLP) from the onset to the last visit. All patients had poor visual prognoses, with either light perception or NLP. Methanol-induced optic neuropathy is a rare bilateral optic neuropathy with a poor visual outcome. A centrally hyperintense T2 signal of the optic nerve is common in methanol-induced optic neuropathy. The thinning of the mGCL-IPL is more sensitive than that of the pRNFL for early diagnosis. A mitochondrial genetic defect may be a predisposing factor for methanol-induced optic neuropathy.

Long term follow-up of uterine fibroids treated with microwave ablation: an up to 3-year observational study of volume, regrowth, and symptoms

Objective To study long-term effects in patients treated with microwave ablation (MWA) for symptomatic uterine fibroids and investigate fibroid characteristics predictive of successful treatment. Method Women who received MWA treatment for uterine fibroids in a previous study were included. A total of 16 patients underwent contrast enhanced MRI before treatment, postoperatively at 6 months and at long-term follow-up, to assess volumes of treated fibroids (n = 42). Long-term MRI was performed between 16 and 36 months after treatment [median 22 months, interquartile range (IQR) 18.5–27]. Validated questionnaires for evaluation of uterine fibroid symptoms and menstrual bleeding (UFS-QoL and PBAC) were used to assess long-term effects on symptoms. The degree of shrinkage was correlated to vascularization and T2 signal intensity (SI) at preoperative MRI and location of fibroids according to the FIGO classification, using the Mann–Whitney U test. Results Most patients (82%) reported improvement up to 3 years after treatment. Out of 42 treated fibroids, 35 (83%) continued to shrink over time with median relative volume reduction of 77% (IQR 39–95). For eight fibroids (19%) which showed low vascularization on the pretreatment MRI, there was less shrinkage compared to well-vascularized fibroids (p = 0.01). Most fibroids (79%) showed iso- to hyperintense T2 signal on preoperative MRI and showed a higher grade of shrinkage than hypointense fibroids (p = 0.02). Conclusion After microwave treatment improvement is maintained for most patients up to 36 months and most fibroids showed continuous shrinkage. Preoperative vascularization, high T2 SI and submucosal location predicted continuous volume reduction. However, to confirm this, larger studies are needed.

A Novel Variant of the Short-Chain Enoyl-CoA Hydratase-1 Gene Presenting with a Mild Phenotype: The Second Case Report from India

AbstractA 9-year-old girl presented with asymmetric abnormal twisting movements affecting her left side more than the right side, initially action induced, but later persistent. Examination revealed generalized persistent dystonia with choreoathetosis and right partial tonic ocular tilt reaction. Brain magnetic resonance imaging showed T1 and T2 fluid-attenuated inversion recovery (FLAIR) hypointense and T2 hyperintense signal changes in bilateral globus pallidi. Clinical exome sequencing revealed compound heterozygous variatnts in enoyl-CoA hydratase-1 (ECHS1) gene: a novel pathogenic variant in exon 6, chr10:g.133366045G &gt; A (p.Gln224Ter) and a likely pathogenic variant in exon 5, chr10:g.133366990G &gt; A (p.Ala173Val). Metabolic testing and arterial lactate levels were normal. She was treated with valine restricted diet, trihexiphenidyl, clonazepam, N-acetyl cysteine and mitochondrial cocktail, without significant improvement over the 6 months follow-up period.

Painless Rectal Bleeding in a Patient With Prostate Cancer

Painless Rectal Bleeding in a Patient With Prostate Cancer

073 Posterior reversible encephalopathy syndrome (PRES) complicating anti-NMDA receptor encephalitis (NMDARE) with ovarian teratoma

IntroductionAnti-N-methyl-D-aspartate receptor antibody-associated encephalitis (NMDARE) is a form of autoimmune encephalitis typically affecting young women, often associated with an ovarian teratoma. Clinical features include psychiatric disturbances, seizures, dyskinesia, dysautonomia, and cardiac dys- rhythmias. Here we present a case of NMDARE complicated by posterior reversible encephalopathy syndrome (PRES). This complication has not been reported previously.CaseA 17-year-old girl presented with acute psychosis. The neurological examination was unremark- able. MRI brain revealed left hippocampal T2 signal hyperintensity; there was a CSF lymphocytosis. NMDA-receptor antibodies in serum and CSF were detected and intravenous methylprednisolone was commenced. Progressive agitation lead to ITU admission. Plasma exchange and intravenous immuno- globulins were administered. MRI pelvis revealed a left ovarian cyst which was removed (laparoscopic salpingo-oophrectomy). Gross inspection and histopathology confirmed an ovarian teratoma. Persistently elevated blood pressure was noted for a week on ITU, associated with clinical deterioration. Rituximab was administered. A repeat MRI brain the next day revealed hyperintensities in temporo-parietal and occipital regions consistent with PRES. The second dose of rituximab was withheld. Clinical and radiologi- cal improvement was noted after a week, and the patient was discharged home.DiscussionEncephalitis mediated dysautonomia may lead to disruption of cerebral autoregulation cul- minating in PRES. The administration of rituximab may also have contributed. This case exemplifies the uncommon complications associated with NMDARE.dr.dvinayan@gmail.com

Brain magnetic resonance imaging radiomics features associated with hepatic encephalopathy in adult cirrhotic patients

PurposeHepatic encephalopathy (HE) is a potential complication of cirrhosis. Magnetic resonance imaging (MRI) may demonstrate hyperintense T1 signal in the globi pallidi. The purpose of this study was to evaluate the performance of MRI-based radiomic features for diagnosing and grading chronic HE in adult patients affected by cirrhosis.MethodsAdult patients with and without cirrhosis underwent brain MRI with identical imaging protocol on a 3T scanner. Patients without history of chronic liver disease were the control population. HE grading was based on underlying liver disease, severity of clinical manifestation, and number of encephalopathic episodes. Texture analysis was performed on axial T1-weighted images on bilateral lentiform nuclei at the level of the foramina of Monro. Diagnostic performance of texture analysis for the diagnosis and grading of HE was assessed by calculating the area under the receiver operating characteristics (AUROC) with 95% confidence interval (CI).ResultsThe final study population consisted of 124 patients, 70 cirrhotic patients, and 54 non-cirrhotic controls. Thirty-eight patients had history of HE with 22 having an HE grade > 1. The radiomic features predicted the presence of HE with an AUROC of 0.82 (95% CI: 0.73, 0.90; P < .0001; 82% sensitivity, 66% specificity). Radiomic features predicted grade 1 HE (AUROC 0.75; 95% CI: 0.61, 0.89; P < .0001; 94% sensitivity, 60% specificity) and grade ≥ 2 HE (AUROC 0.82; 95% CI: 0.71, 0.93; P < .0001, 95% sensitivity, 57% specificity).ConclusionIn cirrhotic patients, MR radiomic is effective in predicting the presence of chronic HE and in grading its severity.

Clinical and radiographic characteristics of sacral arteriovenous fistulas: a multicenter experience.

Available data on management of sacral arteriovenous fistulas (sAVFs) are limited to individual case reports and small series. Management includes observation, endovascular embolization, or surgical ligation, with no clear guidelines on the optimal treatment modality. The authors' objective was to report their multiinstitutional experience with management of sAVF patients, including clinical and radiographic characteristics and postprocedural outcomes. The electronic medical records of patients with a diagnosis of spinal arteriovenous fistula treated from January 2004 to December 2019 at the authors' institutions were reviewed, and data were summarized using descriptive statistics, including percentage and count for categorical data, median as a measure of central tendency for continuous variables, and interquartile range (IQR) as a measure of dispersion. A total of 26 patients with sAVFs were included. The median (IQR) age was 65 (57-73) years, and 73% (n = 19) of patients were male. Lower-extremity weakness was the most common presenting symptom (n = 24 [92%]), and half the patients (n = 13 [50%]) reported bowel and bladder sphincter dysfunction. The median (IQR) time from symptom onset to treatment was 12 (5.25-26.25) months. Radiographically, all patients had T2 hyperintensity at the level of the conus medullaris (CM) (n = 26 [100%]). Intradural flow voids were identified in 85% (n = 22) of patients. The majority of the lesions had a single identifiable arterial feeder (n = 19 [73%]). The fistula was located most commonly at the S1 level (n = 13 [50%]). The site where the draining vein connects to the pial venous plexus was seen predominantly at the lumbar level (n = 16 [62%]). In total, 29 procedures were performed: 10 open surgeries and 19 endovascular embolization procedures. Complete occlusion was achieved in 90% (n = 9) of patients after open surgery and 79% (n = 15) after endovascular embolization. Motor improvement was seen in 68% of patients (n = 15), and bladder and bowel function improved in 9 patients (41%). At last follow-up, 73% (n = 16) of patients had either resolution or improvement of the pretreatment intramedullary T2 signal hyperintensity. T2 hyperintensity of the CM and a dilated filum terminale vein are consistent radiographic signs of sAVF, and delayed presentation is common. Complete occlusion was achieved in almost all patients after surgery, and endovascular embolization was effective in 70% of the patients. Further studies are needed to determine the best treatment modality based on case-specific characteristics.

Progressive Myelopathy With Acute Worsening After Steroids and Lumbar Puncture.

We present the case of a 73-year-old woman with a 3-month history of non-traumatic thoracic myelopathy. Initial MRI showed a T6-conus T2 signal hyperintensity. Based on this presentation, and given a personal and family history of autoimmune disease, our patient was first managed as an inflammatory transverse myelitis. Subsequent worsening after lumbar puncture and steroids prompted re-evaluation, ultimately identifying the cause as a thoracic spinal dural AV fistula. Both investigation of possible transverse myelitis with lumbar puncture and empiric treatment with steroids may not only result in diagnostic delays but also precipitate venous infarction and irreversible harm. While the MRI often provides the initial diagnosis, clinical suspicion for this under-diagnosed cause of myelopathy should be raised in older patients with a more progressive thoracic myelopathy with worsening after lumbar puncture and/or steroids. Definitive and time-sensitive treatment by interventional neuroradiology or neurosurgery results in stabilization or improvement of disability in most cases.

Clinical and Molecular Correlates of Abnormal Changes in the Cerebellum and Globus Pallidus in Fragile X Premutation.

BackgroundFragile X premutation carriers (55–200 CGG triplets) may develop a progressive neurodegenerative disorder, fragile X-associated tremor/ataxia syndrome (FXTAS), after the age of 50. The neuroradiologic markers of FXTAS are hyperintense T2-signals in the middle cerebellar peduncle—the MCP sign. We recently noticed abnormal T2-signals in the globus pallidus in male premutation carriers and controls but the prevalence and clinical significance were unknown.MethodsWe estimated the prevalence of the MCP sign and pallidal T2-abnormalities in 230 male premutation carriers and 144 controls (aged 8–86), and examined the associations with FXTAS symptoms, CGG repeat length, and iron content in the cerebellar dentate nucleus and globus pallidus.ResultsAmong participants aged ≥45 years (175 premutation carriers and 82 controls), MCP sign was observed only in premutation carriers (52 vs. 0%) whereas the prevalence of pallidal T2-abnormalities approached significance in premutation carriers compared with controls after age-adjustment (25.1 vs. 13.4%, p = 0.069). MCP sign was associated with impaired motor and executive functioning, and the additional presence of pallidal T2-abnormalities was associated with greater impaired executive functioning. Among premutation carriers, significant iron accumulation was observed in the dentate nucleus, and neither pallidal or MCP T2-abnormalities affected measures of the dentate nucleus. While the MCP sign was associated with CGG repeat length >75 and dentate nucleus volume correlated negatively with CGG repeat length, pallidal T2-abnormalities did not correlate with CGG repeat length. However, pallidal signal changes were associated with age-related accelerated iron depletion and variability and having both MCP and pallidal signs further increased iron variability in the globus pallidus.ConclusionsOnly the MCP sign, not pallidal abnormalities, revealed independent associations with motor and cognitive impairment; however, the occurrence of combined MCP and pallidal T2-abnormalities may present a risk for greater cognitive impairment and increased iron variability in the globus pallidus.

Surgical management of spinal dural arteriovenous fistula – A single centre experience

IntroductionSpinal dural arteriovenous fistulas (sdAVF) are vascular malformations occurring in the spine which are commonly seen in elderly men in the sixth and seventh decade of life [1]. Patients initially present with non-specific symptoms, eventually progressing to motor, sensory, and autonomic disturbances. Due to delayed presentation and non- specific symptoms, sdAVF are often undiagnosed, leading to delay in treatment. AimWe report 4 cases of sdAVF who underwent microsurgical treatment. MethodsAll the patients presented with insidious onset, gradually progressive bilateral lower limb weakness, paraesthesia, and urinary incontinence. MRI revealed subarachnoid flow voids and spinal cord T2 signal hyper-intensities in all 4 patients. Spinal angiogram showed fistula at T10,11 level in one patient, L1 level in two patients and at L5 in one patient. In these patients the artery supplying the AV fistula share a common pedicle with either artery of Adamkiewicz, Anterior spinal artery (ASA) or posterior spinal artery(PSA). Hence, the use of embolic agent in these patients to occlude the fistula carries a high risk of cord ischemia. In all these patients microsurgical procedure was carried out with successful outcomes. ResultsAll the 4 patients improved neurologically after surgery. Lower limb weakness and sensations improved early, but improvement of bladder function was delayed. Functional outcome was reassessed 6 months after the procedure using Modified Aminoff and Logue’s scale, which was compared to pre-operative functional status of the individual. ConclusionTimely intervention of sdAVF is challenging because of its nonspecific presentation. MRI offers a clue to the diagnosis while spinal angiography confirms the diagnosis and angio-architecture will decide the suitable mode of treatment. Though endovascular treatment is a viable option surgery is the gold standard [14].

Intralabyrinthine Hemorrhage After General Anesthesia: Case Report

Several cases of intralabyrinthine hemorrhage are described in the literature. The causes are mainly anticoagulants and blood disorders. The diagnosis is suspected on clinical examination (sudden cochleo-vestibular syndrome) and confirmed by inner ear magnetic resonance imaging (MRI). We report the case of a 58-year-old woman with a history of neuro-syphilis with motor sequelae, ischemic heart disease under antiplatelet therapy, rheumatoid arthritis and colonic adenocarcinoma treated by surgery and adjuvant chemotherapy. This patient presented a unilateral cochleovestibular syndrome immediately after a short general anesthesia. MRI showed a spontaneous T1 signal hyperintensity confirming a left labyrinthine hemorrhage. The clinical course was marked by regression of balance disorders without auditory recovery. Intralabyrinthine hemorrhages are rare and often secondary to anticoagulants or blood diseases. Through this article, we report a case of intralabyrinthine hemorrhage secondary to a brief general anesthesia.