Spinal Cord Magnetic Resonance Imaging Research Articles

Downbeat Nystagmus Elicited by Eyelid Closure

We describe a patient with downbeat nystagmus (DBN) evoked only by eye closure. Brain and spinal cord magnetic resonance imaging revealed a T2 paramedian lesion in the left lower basis pontis and other white matter lesions consistent with multiple sclerosis. One potential mechanism for DBN in this case involves transverse ephaptic spread of excitation from areas that subserve coordinated lid closure to the decussating ventral tegmental tract.

Eosinophilic CNS vasculitis can mimic demyelinating disease of the brain and spinal cord

A previously healthy 61year-old Filipino man developed progressive ascending sensory loss up to the T8 dermatome and paraparesis over a period of four weeks. Spinal cord magnetic resonance imaging (MRI) revealed a T2-hyperintense lesion from the T7 to the T10 spinal segment with varying degrees of gadolinium enhancement. Brain MRI imaging revealed several ovoid T2-hyperintense lesions in juxtacortical and periventricular areas. Cerebrospinal fluid analysis only showed a mild lymphocytic pleocytosis without atypical cells.

Normal Cerebellar Metabolism in a Patient with Superficial Siderosis

Normal Cerebellar Metabolism in a Patient with Superficial Siderosis

Specific pattern of gadolinium enhancement in spondylotic myelopathy.

To highlight a specific under-recognized radiological feature of spondylotic myelopathy often resulting in misdiagnosis. Patients evaluated between January 1, 1996 and December 31, 2012 who met the following criteria were included: (1) spondylotic myelopathy was suspected, (2) gadolinium enhancement was detected, and (3) spinal surgery was performed. Fifty-six patients (70% men) whose median age was 53.5 years (range = 24-80) were included. Spinal cord magnetic resonance imaging (cervical in 52; thoracic in 4) revealed longitudinal spindle-shaped T2-signal hyperintensity (100%) and cord enlargement (79%) accompanied by a characteristic pancakelike transverse band of gadolinium enhancement in 41 (73%), typically immediately caudal to the site of maximal spinal stenosis. Forty (71%) patients were initially diagnosed with neoplastic or inflammatory myelopathies, and decompressive surgery was delayed by a median of 11 months (range = 1-64). Spinal cord biopsy in 6 did not reveal any alternative diagnosis. Ninety-five percent were stable or improved. Gadolinium enhancement persisted in 75% at 12 months, raising concern about the accuracy of the initial diagnosis. Twenty patients required a gait aid (36%) at last follow-up (median = 60 months, range = 10-172). The need for a gait aid preoperatively (p = 0.005), but not delay of surgery, predicted the need for gait aid at final follow-up. Transverse pancakelike gadolinium enhancement associated with and just caudal to the site of maximal stenosis and at the rostrocaudal midpoint of a spindle-shaped T2 hyperintensity suggests that spondylosis is the cause of the myelopathy. Persistent enhancement for months to years following decompressive surgery is common. Recognition is important to prevent inappropriate interventions or delay in consideration of a potentially beneficial decompressive surgery.

Primary natural killer/T-cell lymphoma presenting as leptomeningeal disease

IntroductionPrimary central nervous system natural killer/T-cell lymphoma (primary-CNS-NK/TCL) is a rare non-Hodgkin's lymphoma. To our knowledge, only five patients have been described previously, all of whom were male, with brain parenchymal involvement and previous Epstein–Barr virus infection, it has never been reported to present as leptomeningeal disease as our case. Our objective is to report a rare case of primary-CNS-NK/TCL presenting as leptomeningeal disease and to share our diagnostic/therapeutic approach to this rare disease. MethodsWe report a rare case of primary-CNS-NK/TCL presenting as leptomeningeal disease. The patient was diagnosed and treated at The University of Texas MD Anderson Cancer Center. ResultsThe patient presented with multiple cranial neuropathies and gait ataxia. Brain and spinal cord magnetic resonance imaging demonstrated leptomeningeal enhancement of the cerebellar folia/vermis, spinal cord dura, and both temporal lobes as well as adjacent brain parenchymal disease. Cerebrospinal fluid (CSF) revealed atypical lymphoma cells of NK/T-cell lineage by flow cytometric immunophenotyping. Molecular analysis using real-time quantitative polymerase chain reaction did not detect Epstein–Barr virus DNA in the lymphoma cells. Bone marrow biopsy revealed no morphologic, flow cytometric, or immunohistochemical evidence of B-, T- or NK-cell lymphoma. Slit-lamp examination demonstrated no evidence of intraocular lymphoma. Whole-body PET scan showed no evidence of malignancy other than CNS disease. The patient was given systemic chemotherapy with high-dose methotrexate, vincristine, and procarbazine, along with intrathecal therapy with free cytarabine. The patient showed clinicoradiographic improvement and CSF cytology became negative. ConclusionThis case highlights an atypical presentation of primary-CNS-NK/TCL with a potentially successful treatment regimen.

Hereditary spastic paraplegia type 5: a potentially treatable disorder of cholesterol metabolism

Spastic paraplegia type 5 (SPG5) is an autosomal, recessive, hereditary spastic paraparesis (HSP) caused by mutations in CYP7B1, which is responsible for a key step in the alternative pathway of bile acid synthesis [1]. Both pure and complicated clinical forms are possible, and brain magnetic resonance imaging (MRI) might show periventricular and subcortical white matter involvement [2]. Electrophysiology reveals abnormal conduction along the central pathways and peripheral nervous system sparing [3]. SPG5 patients have increased levels of 27-hydroxycholesterol (27OHC) in plasma and cerebrospinal fluid (CSF) [4]. Cerebral accumulation of 27OHC may be an important pathogenetic event in SPG5, with relevant therapeutic implications: indeed, since the substrate availability is a limiting factor for CYP27A1 activity [5], HMG CoA reductase inhibitors might reduce 27OHC, thus preventing neurological impairment. Here we describe two SPG5 patients and report the preliminary follow-up data including evaluation of response to statin therapy in one. Patient 1 was a 29-year-old woman with a 2-year history of gait disturbances, whereas her 24-year-old brother referred walking difficulties and lower limb stiffness since he was age 20. Past medical history revealed prolonged neonatal jaundice in both siblings. Neurological examination disclosed a pure clinical phenotype with spastic paraplegia rating scale (SPRS) [6] values of 15/52 and 10/52 in patients 1 and 2, respectively. Neuropsychological assessment did not detect cognitive impairment. Brain MRI showed slight FLAIR hyper-intense signal in the periventricular and centrum ovale white matter, and spectroscopy (1H MRS) on those areas showed a mild reduction of the relative ratio N-acetyl aspartate/creatine and the presence of a small lipid peak in both patients (Fig. 1a, b). Spinal cord MRI was normal. A motor evoked potentials (MEPs) study uncovered abnormal central motor conduction times (CMCTs) from both upper and lower limbs, and electromyography was normal. Gene analysis detected two compound heterozygous mutations (c.333_334delTC and c.806delA) in CYP7B1. Serum 27OHC levels were elevated 5.5 times in patient 1 (88 lg/ dl) and 10 times in patient 2 (159 lg/dl), respectively (normal 16 ± 3 lg/dl). Both patients underwent clinical, biochemical, and instrumental follow-up over a period of 9 months. Patient 1 was put on oral simvastatin (20 mg/day at months 0–3, 40 mg/day at months 4–6, and 60 mg/day at months 7–9), whereas her younger brother manifested cramps and marked hyperCKemia and discontinued simvastatin 3 days after introduction. When compared to baseline levels, serum 27OHC values were decreased at months 3, 6, and 9 (10, 34, and 33 %, respectively) in patient 1, and A. Mignarri A. Malandrini F. Ginanneschi A. Federico M. T. Dotti (&) Unit of Neurology and Neurometabolic Disorders, Department of Medicine, Surgery and Neurosciences, University of Siena, Siena, Italy e-mail: dotti@unisi.it

Groupwise multi-atlas segmentation of the spinal cord’s internal structure

The spinal cord is an essential and vulnerable component of the central nervous system. Differentiating and localizing the spinal cord internal structure (i.e., gray matter vs. white matter) is critical for assessment of therapeutic impacts and determining prognosis of relevant conditions. Fortunately, new magnetic resonance imaging (MRI) sequences enable clinical study of the in vivo spinal cord's internal structure. Yet, low contrast-to-noise ratio, artifacts, and imaging distortions have limited the applicability of tissue segmentation techniques pioneered elsewhere in the central nervous system. Additionally, due to the inter-subject variability exhibited on cervical MRI, typical deformable volumetric registrations perform poorly, limiting the applicability of a typical multi-atlas segmentation framework. Thus, to date, no automated algorithms have been presented for the spinal cord's internal structure. Herein, we present a novel slice-based groupwise registration framework for robustly segmenting cervical spinal cord MRI. Specifically, we provide a method for (1) pre-aligning the slice-based atlases into a groupwise-consistent space, (2) constructing a model of spinal cord variability, (3) projecting the target slice into the low-dimensional space using a model-specific registration cost function, and (4) estimating robust segmentation susing geodesically appropriate atlas information. Moreover, the proposed framework provides a natural mechanism for performing atlas selection and initializing the free model parameters in an informed manner. In a cross-validation experiment using 67 MR volumes of the cervical spinal cord, we demonstrate sub-millimetric accuracy, significant quantitative and qualitative improvement over comparable multi-atlas frameworks, and provide insight into the sensitivity of the associated model parameters.

Mucopolysaccharidosis type IVA: Evidence of primary and secondary central nervous system involvement

Mucopolysaccharidosis type IVA is a rare lysosomal storage disease caused by a deficiency of N-acetylgalactosamine 6-sulfatase. Studies usually focus on skeletal abnormalities and their consequences. This study explores the neurological manifestations in a cohort of mucopolysaccharidosis type IVA patients, with a detailed focus on brain and spinal magnetic resonance imaging (MRI) findings. We performed a cross-sectional study involving nine patients with a biochemical confirmation of mucopolysaccharidosis type IVA. The protocol consists of a comprehensive clinical examination and brain and spinal cord MRI analysis for all subjects. The mean age was 16.4 years (±5.7) and the mean onset of symptoms was 11.5 months (±6.3). Overall, cognition was spared in all but one patient and motor weakness was a constant finding in all patients. Deep sensation impairment was found in six patients. The brain MRIs showed non-specific white matter changes in two patients. Other abnormalities such as clival hypoplasia, basilar invagination, and arachnoid cists appeared in seven of the nine patients. Eight patients presented spinal cord compression, and in three of them, two spinal levels were compromised. Odontoid hypoplasia and degenerative features in the neuroaxis were present in all patients. Our experience with mucopolysaccharidosis type IVA patients supports the evidence of central nervous system involvement. We emphasize the importance of regular clinical assessments with complete MRI studies, as an attempt to detect the early signs of spinal cord compression. This evaluation may be especially important before surgical interventions, as occult lesions may become symptomatic and promote postoperative unfavorable outcomes.

Sensitivity and specificity of spinal cord Magnetic Resonance Imaging in the diagnosis of HTLV-1 associated myelopathy

Since 1997, the Interdisciplinary Research Group on HTLV-1/2 (GIPH) has been following individuals infected with HTLV in Minas Gerais, Brazil, in an open prospective cohort study. The HAM diagnosis is based on clinical parameters, with support of complementary exams to exclude other possible causes of myelopathy. The objective of this study was to evaluate the sensitivity and specificity of spinal cord MRI in HAM diagnosis. This was a cross-sectional study, conducted between March 17 th and September 28 th , 2012. The 120 HTLV-1 seropositive were included in the study. HAM was diagnosed on the basis of the World Health Organization diagnostic criteria. Cervical and thoracic MRIs were performed in sagittal and axial sequences fast spin-echo T2-weighted, without gadolinium. Blinded interpretation of MRIs was performed by radiologists who did not know clinical neurologic status of the participants. The mean age of the 120 individuals was 48.0 ± 12.8 years (range: 16.4 to 72.6 years) and 77 (64.2%) were women. Among all patients, 19 (15.8%) had a diagnosis of HAM. There was no statistically significant difference between the mean ages of the HAM and asymptomatic groups (p = 0.7). In the HAM group, 7 (36.8%) of them showed changes in MRIs compatible with the disease. On the other hand, 93 (83.0%) asymptomatic individuals have had normal MRIs. Spinal cord MRI demonstrated to be a diagnostic method with good specificity but low sensitivity in the diagnosis of HAM. It is possible that time evolution of disease, as well as the degree of neurological impairment is directly related to the sensitivity of the method.

A case of pediatric paraparesis secondary to an idiopathic acute transverse myelitis

Introduction: Acute transverse myelitis (AtM) refers to a frequently idiopathic, segmental spinal cord inflammation. It is a rare condition, in particular in children, and not previously reported in a family retinitis pigmentosa (rP) clinical setting. case report: An 11-year-old previously healthy girl, with a family history of rP, presented with a subacute flaccid paraparesis, with bilateral, up to the fourth dorsal level, mixed sensory hypoesthesia and autonomic dysfunction. brain and spinal cord magnetic resonance imaging (MrI) showed an extensive, t2-hyperintense, non-contrast enhancing lesion from the second to fifth dorsal levels. cerebrospinal fluid (csF) and lab studies were normal, as the ophthalmologic observation. treated with high-dose corticosteroids and intensive physical therapy, a significant recovery could be seen. conclusion:

痛みをともなわず1日で横断性脊髄症を生じ広範な脊髄病変を呈した神経Behçet病の1例

A 64-year-old man had transverse myelopathy that rapidly progressed without pain over the course of 1 day. The cerebrospinal fluid interleukin-6 (CSF IL-6) level was extremely high (1,120 pg/dl). Spinal cord magnetic resonance imaging (MRI) showed a longitudinal extensive lesion extending from Th8 to the conus medullaris. Despite treatment with steroids and cyclophosphamide, the symptoms worsened, and the range of sensory disturbance spread. MRI showed that the lesion expanded to Th3 over the course of 2 months. Neuro-Behçet disease (NBD) was diagnosed on basis of the high CSF IL-6 level and HLA-B51 positivity, and treatment with infliximab was begun. The sensory disturbance improved slightly, and the CSF IL-6 level fell to the normal range (7.0 pg/ml). It is important to include NBD, which rarely presents with extensive spinal cord lesion, in the differential diagnosis when patients present with acute transverse myelopathy without pain.

Propiospinal myoclonus at sleep onset (PSM) after spinal anesthesia in a patient with restless legs syndrome (RLS) and periodic limb movements during sleep (PLMS): a case report

Introduction PMS is a rare disorder that consist of repetitive axial jerks arousing mainly during drowsiness preceding falling asleep. The last revision of the International Classification of Sleep Disorders (ICDS-2) has considered PMS into the apparently normal variants and unresolved issues. PMS have been occasionally reported to occur in idiopathic RLS. Our objetive is to expose the results of the study of a patient with RLS who started with axial jerks resembling PSM after spinal anesthesia. Materials and methods A 56-year-old woman had a 6-year history of uncomfortable sensations involving the legs and sometimes also the arms when sitting or laying down, worsening in bed and impending sleep. Her husband also reported frequent limb movements during sleep. In the last 2 years sudden jerks of her trunk and abdomen had additionally appeared during relaxed wakefulness. Apparently the axial jerks began immediately after spinal anesthesia for a left lower extremity orthopedic procedure. The patient underwent a clinical interview, physical and neurological examination, video electroencephalographic recording (VEEG), video-polysomnographic recording (VPS), jerk-related EEG-EMG back averaging, electroneurography (ENG), somatosensory-evoked potentials (SEPs), magnetic stimulation motor evoked potentials (TMS) and brain and spinal cord magnetic resonance imaging (MRI). Results VPS showed repetitive jerks with variable regularity during relaxed wakefulness, intra sleep wakefulness and even during light sleep involving axial and leg muscles. On the other hand, isolated PLMs during sleep involving both anterior tibial muscles were registered. Spinal cord MRI showed degenerative changes present in the lower cervical and lumbar spine without apparent medullar compromise. TMS however revealed a slight but significant delay of central conduction to left lower limb. Jerk – related EEG – EMG back- averaging did not disclose any preceding cortical potential. No significant findings were found in the rest of the tests. Conclusion From our knowledge this is the first description of PMS after spinal anesthesia in a patient with SPI and PLMS. The causal relationship remains unclear. Acknowledgements I must thank all the contributors: Dr. Fernandez – Torre and Dr. Orizaola Balaguer of the Neurophysiology Department. All medical staff, nurses and technicians of our Multidisciplinary Marques de Valdecilla Sleep Unit.

Clinical Manifestations and Spinal Cord Magnetic Resonance Imaging Findings in Chinese Neuromyelitis Optica Patients

Neuromyelitis optica (NMO) is a disease distinct from multiple sclerosis in terms of clinical and magnetic resonance imaging (MRI) manifestations. Antibody to aquaporin-4 (AQP4) has been identified as a specific biomarker and part of the diagnostic criteria for NMO. Although it is relatively common in Asia, a comprehensive clinical and imaging evaluation of NMO has not been reported in Chinese patients. Here, we reviewed data from 57 Chinese cases. The patients had an obvious female preponderance (female/male = 8.5:1), and transverse myelitis (82.5%) and optic neuritis (56.1%) were the most common manifestations. In MRI, longitudinally extensive transverse myelitis (6.9 ± 2.3 segments) dominated the spinal cord lesions, which were mainly (69.7%) distributed in cervical and thoracic cord. However, the length of the lesions was not correlated with onset age, paralysis severity, relapse rate, or duration. Among 29 patients who underwent AQP4 antibody assay, 17 (58.6%) were positive. There was no difference between seropositive and seronegative patients in terms of female preponderance, onset age, relapse rate, and Expanded Disability Status Scale score. However, seropositive patients had significantly more damaged segments (8.3 ± 3.5) than did seronegative patients (4.5 ± 1.6) (p < 0.001). The data revealed the clinical and MRI characteristics and AQP4 antibody status of NMO in Chinese patients and the correlations between them, which may have important implications for the diagnosis of the disease.

PROBING THE POTENTIAL FOR FUNCTIONAL RECOVERY IN PATIENTS WITH NEUROMYELITIS OPTICA USING VISUOMOTOR PRACTICE

ObjectiveNeuromyelitis optica (NMO) is characterised by episodes of optic neuritis and transverse myelitis. Despite therapeutic advances, treatments aimed at reducing disease activity have little impact on disabilities and much of...

Recurrent tethered cord: radiological investigation and management

Recurrent tethered cord (RTC) is almost the rule after the repair of myelomeningocele and quite frequent after the repair of lipomyelomeningocele, resulting from the adhesions of the placode within a too narrow spinal canal. About one-third of patients with myelomeningocele and 10 % of those with spinal lipoma develop symptomatic RTC, mainly caused by the ischemic-metabolic injuries due to the cord stretching. The goal of this review is to provide information about the pathophysiology, the radiological picture, and the management of RTV according to the pertinent literature and the authors' experience. The magnetic resonance imaging (MRI) picture is characterized by a low position of the conus and by tethering of the spinal cord to the subcutaneous scar or to the inner surface of the spinal canal. The radiological work-up always includes brain MRI, to rule out other possible causes of late neurological deterioration (as shunt malfunction), and MRI of the whole spinal cord, to detect possible associated lesions (syringomyelia, dermoids, etc.). X-rays and/or computed tomography scan of the spine is required for the assessment of scoliosis or other bony malformations. The surgical treatment is planned after a multidisciplinary neurological, urological, orthopedic, physiatric, and radiological evaluation. The surgical detethering is carried out cautiously, possibly with electrophysiological intraoperative monitoring. Surgery ensures improvement or stability of the clinical picture in 70-80 % of cases, the remaining 20-30 % of patients needing multiple operations for their recovery. Complications may affect up to one-third of operated patients, being mainly represented by CSF leak, pseudomeningocele, and shunt malfunction.

New insights in mucopolysaccharidosis type VI: Neurological perspective

Objective: Mucopolysaccharidosis type VI is a rare autosomal recessive storage disorder, caused by deficiency of arylsulfatase B. Data on neurological involvement in mucopolysaccharidosis type VI patients under enzyme-replacement therapy are limited. This study explores the neurological and magnetic resonance imaging findings in a sample of mucopolysaccharidosis type VI patients receiving enzyme-replacement therapy. Methods: We performed a cross-sectional study including six patients with biochemical confirmation of mucopolysaccharidosis type VI and at least 105 consecutive weeks (two years) receiving intravenous enzyme-replacement therapy. The protocol included a comprehensive clinical examination, brain and spinal cord magnetic resonance imaging for all subjects. Results: Overall, cognition was spared, while we found presence of hearing impairment, increasing in deep tendon reflexes and deep sensation reduction in three patients. In addition to the classical abnormalities related to other types of mucopolysaccharidosis, imaging studies demonstrated morphological changes in anatomy of middle cranial fossa and sella shape. Even in asymptomatic or mild compromised patients, spinal cord compression was found. In four patients we noticed atlantoaxial joint subluxation and three had cervical spinal stenosis. Degenerative processes involving vertebral column, including discal protrusion and axis abnormalities, were present in all patients. Conclusions: Neuroaxis involvement was a universal finding and neurological examination might not predict the severity of the disease in course. Image studies should not be performed according exclusively clinical parameters for these patients, once we have demonstrated that neurological involvement may be silent in these patients.

Pediatric onset multiple sclerosis: McDonald criteria 2010 and the contribution of spinal cord MRI

Background: Diagnostic magnetic resonance imaging (MRI) criteria have not been sufficiently validated in pediatric multiple sclerosis (MS) despite differences in epidemiologic data and clinical disease courses between pediatric and adult MS. Objective: The objective of this paper is to evaluate the diagnostic applicability and validity of the revised McDonald diagnostic criteria 2010 in a large cohort of pediatric-onset MS patients (POMS) and compare them to previously recommended MRI-based classifications. Furthermore, we aimed to investigate the contribution of spinal cord lesions to the revised McDonald criteria 2010. Methods: A cohort of 85 patients with definite MS, age at onset 2.8–18 years, was analyzed in a retrospective multicenter study. Number and regional distribution of T2w and contrast-enhancing T1w lesions at initial and follow-up MRIs were main outcome measures. Results: In 62% of POMS the initial MRI within four weeks after symptom onset was sufficient to diagnose MS according to the revised McDonald criteria 2010. In a subcohort of patients with spinal MRI at first presentation, 10% reached the dissemination in space (DIS) and dissemination in time (DIT) criteria only by the inclusion of contrast-enhancing spinal lesions. Conclusions: The revised McDonald criteria 2010 facilitate the diagnosis of POMS already at first presentation. The addition of a spinal cord MRI was helpful only in selected cases.

Magnetic resonance imaging correlates of physical disability in relapse onset multiple sclerosis of long disease duration

Background: Understanding long-term disability in multiple sclerosis (MS) is a key goal of research; it is relevant to how we monitor and treat the disease. Objectives: The Magnetic Imaging in MS (MAGNIMS) collaborative group sought to determine the relationship of brain lesion load, and brain and spinal cord atrophy, with physical disability in patients with long-established MS. Methods: Patients had a magnetic resonance imaging (MRI) scan of their brain and spinal cord, from which we determined brain grey (GMF) and white matter (WMF) fractional volumes, upper cervical spinal cord cross-sectional area (UCCA) and brain T2-lesion volume (T2LV). We assessed patient disability using the Expanded Disability Status Scale (EDSS). We analysed associations between EDSS and MRI measures, using two regression models (dividing cohort by EDSS into two and four sub-groups). Results: In the binary model, UCCA (p < 0.01) and T2LV (p = 0.02) were independently associated with the requirement of a walking aid. In the four-category model UCCA (p < 0.01), T2LV (p = 0.02) and GMF (p = 0.04) were independently associated with disability. Conclusions: Long-term physical disability was independently linked with atrophy of the spinal cord and brain T2 lesion load, and less consistently, with brain grey matter atrophy. Combinations of spinal cord and brain MRI measures may be required to capture clinically-relevant information in people with MS of long disease duration.

16 Syndrome in a Patient With Multiple Sclerosis

We read with interest the report of Connors et al (1) of a case of 16 syndrome with complete horizontal gaze paralysis and facial diplegia caused by a pontine hemorrhage. We describe a patient with multiple sclerosis (MS) who experienced an evolving eye movement disorder, which included internuclear ophthalmoplegia, one-and-a-half syndrome, eight-and-a-half syndrome, fifteen-and-a-half syndrome, and finally 16 syndrome. A 44-year-old man with a history of hypertension, heavy smoking, and a positive family history of cardiovascular disease complained of double vision upon awakening. Neurological examination revealed bilateral internuclear ophthalmoplegia (INO), which was attributed to a brainstem infarction given the sudden onset and patient’s vascular risk factors. Magnetic resonance imaging (MRI) of the brain and the intracranial vessels revealed several periventricular white matter lesions but no brainstem abnormality. Three days later, the patient developed drooping of the right corner of his mouth and complained of drooling. Examination revealed a right lower motor neuron facial nerve paresis in addition to bilateral INO. Repeat MRI revealed a nonenhancing lesion in the midline of the dorsal pons (Fig. 1). Two days later, he developed a complete right conjugate horizontal gaze palsy which, coupled with his left INO, produced a one-and-a-half syndrome. Concurrently, he also experienced bilateral facial paresis.FIG. 1: Axial fluid-attenuated inversion recovery (A) and sagittal T2 (B) MRI show an area of high signal (arrows) in the dorsal pons ventral to the fourth ventricle.Evaluation for stroke including extensive hematological tests, Holter cardiac monitoring, and transesophageal echocardiography was normal. Cerebrospinal fluid analysis for IgG, oligoclonal bands, and aquaporin-4 antibodies as well as visual evoked responses were normal. MRI of the cervical spinal cord revealed no lesions. On the 11th day of hospitalization, the patient developed bilateral horizontal gaze palsies with worsening of his facial diplegia. His clinical course in combination with the periventricular white matter lesions was highly suggestive of demyelinating disease. Treatment with methylprednisolone was begun, and 3 weeks later, he had recovered completely. Several follow-up MRIs were performed. The most recent MRI, 1.5 years after the onset of symptoms, revealed an increase in number and extent of the white matter hyperintensities (Fig. 2). Glatiramer acetate therapy was initiated.FIG. 2: Axial fluid-attenuated inversion recovery (A) and sagittal T2 (B) scans reveal several areas of high signal intensity in the periventricular region and corpus callosum.On occasion, a one-and-a-half syndrome can be accompanied by a facial paresis if the fascicle or nucleus of the seventh cranial nerve in the lower part of the dorsal pontine tegmentum is also affected. Eggenberger (2) designated this as eight-and-a-half (1.5 + 7) syndrome. As mentioned by Connors et al (1), variations of this syndrome caused by pathology of the dorsal pontine tegmentum have since been described including a combination of a one-and-a-half syndrome and a bilateral peripheral facial paresis which Bae and Song (3) designated fifteen-and-a-half (1.5 + 7 + 7) syndrome. Only 3 cases of isolated eight-and-a-half syndrome caused by MS have been described in the literature (4,5). In one of these cases (4), the eight-and-a-half syndrome was, as in our patient, the initial symptom of MS. A 16 syndrome caused by MS has not been reported previously.

Nonanaplastic Pleomorphic Xanthoastrocytoma with Meningeal Dissemination Presenting with Bilateral Visual Loss

Pleomorphic xanthoastrocytoma (PXA) is a brain neoplasm included in the astrocytic group, exceptionally manifesting with meningeal dissemination. We described a 27-year-old patient presented with acute bilateral visual loss and papilledema with normal brain computed tomography scan, initially mimicking idiopathic intracranial hypertension (IIH). Brain and spinal cord magnetic resonance imaging (MRI) study revealed a subtle area of hyperintensity of the gyri surrounding the left central sulcus, and contrast enhancement of the thoracic leptomeninges. Brain biopsy of the parietal lesion revealed nonanaplastic PXA. Treatment with temozolomide was given. Yearly control MRI demonstrated new brain lesions and marked progression of leptomeningeal spinal enhancement. In spite of this, the patient has remained stable with no new symptoms. Nonanaplastic PXA may present with widespread meningeal dissemination with acute visual loss and papilledema mimicking IIH, and no clinical progression at 3 years.