Bilateral High Signal Research Articles

Correlation of Endolysmphatic Duct Signal Intensity With Clinical Features in Otological Diseases.

Bilateral high signal intensity (SI) in the endolymphatic duct (ED) on magnetic resonance imaging (MRI) has been reported as a common characteristic in ears with large vestibular aqueduct syndrome (LVAS). However, the significance of bilateral high SI in the ED remains unknown. The present study aimed to compare the correlation between SI in the ED and the clinical manifestations in various otological disorders and consider the significance of the MRI findings. Retrospective study. University hospital. The study included 2,450 ears from 1,225 patients with various otological disorders. All ears underwent 3T enhanced MRI and were evaluated for the degree of endolymphatic hydrops (EH) and the SI ratios (SIRs; i.e., the calculation between SIs in the ED and those in the cerebellum). The imaging findings were compared with their clinical symptoms. Ears with bilateral high SIRs in the ED tended to have considerably less occurrence of EH in both the cochlea and vestibule than those with bilateral low SIRs. Ears with SIR ≥8 showed significantly elevated hearing thresholds at lower frequencies on pure-tone audiometry, although they exhibited a markedly lower incidence of cochlear EH than those with SIR <8. Moreover, ears with vertigo exhibited notably higher SIRs than those without vertigo. Bilateral high SI in the ED on MRI may reflect pathophysiology underlying sensorineural hearing loss and vestibular symptoms, which are not associated with EH formation.

Stroke-like presentation of acute toxic leukoencephalopathy due to capecitabine treatment with extensive intramyelinic edema.

Stroke-like presentation of acute toxic leukoencephalopathy due to capecitabine treatment with extensive intramyelinic edema.

Hyperperfused bilateral high signal intensity on diffusion‐weighted imaging of the parietotemporal cortices in echovirus 25 encephalitis

Authors declare no Conflict of Interest for this article.

Distribution of Deep Gray Matter Lesions on Magnetic Resonance Imaging in Lymphomatosis Cerebri.

We herein report the distribution of gray matter lesions on magnetic resonance imaging (MRI) in two patients with lymphomatosis cerebri (LC). In our patients, the fluid-attenuated inversion recovery sequence of brain MRI demonstrated a bilateral and diffuse high signal intensity, not only in the white matter but also in the thalamus, globus pallidus, putamen, and hippocampus. Among the deep gray matter, the caudate head and putamen (striatum) were relatively spared when compared with the globus pallidus, thalamus, and hippocampus. Interestingly, we found seven previous reports of similar MRI findings, with relative sparing of the striatum, in patients with LC. This finding may be characteristic of LC and help facilitate its diagnosis. Further investigations of a larger number of LC patients are necessary to confirm these findings.

A case report of adult-onset Alexander disease clinically presenting as Parkinson\u2019s disease: is the comorbidity associated with genetic susceptibility?

BackgroundAlexander disease is a rare neurological disease characterized by progressive spastic quadriparesis and bulbar palsy. Moreover, certain patients with adult-onset Alexander disease were often misdiagnosed as other neurodegenerative disorders.Case presentationHerein, we report an adult a 58-year-old woman presented with typical parkinsonism with good levodopa-responsiveness. The patient’s dopamine transporter scanning showed significant striatal depletion, while her brain magnetic resonance imaging revealed bilateral tadpole shape of medulla oblongata and bilateral high signal intensity at both cerebellar dentate nuclei in T2-weighted images, suggesting the possibility of a genetic disorder beyond Parkinson’s disease. The patient’s genetic test resulted in known pathogenic glial fibrillary acidic protein variant, indicating Alexander disease.ConclusionThis unique case highlights genetically diagnosed Alexander disease may present with clinical Parkinson’s disease.

Acute Hippocampal Encephalopathy in Heavy Cannabis Users: About 2 Cases

BackgroundCannabis use is increasing worldwide despite the various health effects of this substance. MethodsWe report 2 cases of acute hippocampal encephalopathy in heavy cannabis users (>10 joints/d). ResultsIn both male patients, acute encephalitis was suspected. Brain magnetic resonance imaging (MRI) diffusion-weighted sequences showed bilateral high signal abnormalities in hippocampal regions. Patients had renal dysfunction, rhabdomyolysis, and inflammatory syndrome. Investigations showed no evidence of infectious or autoimmune encephalitides. Repeated electroencephalograms revealed no epileptic activity. Clinical, biological, and magnetic resonance imaging acute abnormalities improved within weeks. New exposure to cannabis yielded a new episode of encephalopathy. In both patients, severe long-lasting episodic memory impairment associated with hippocampal atrophy were observed several months later. ConclusionsHealth professionals should be aware of this cannabis-related syndrome given its severe and long-lasting effects.

Capecitabine-induced acute toxic leukoencephalopathy

A 45-year-old woman was treated by Capecitabine (Xeloda®) during 6days for breast cancer with metastatic bone lesions when she presented with nausea, headaches, muscle cramps, dysarthria and swallowing disorders. A stroke was first suspected. Brain CT was normal. MRI showed bilateral and symmetric high signal intensities of deep white matter, corpus callosum and corticospinal tracts on diffusion-weighted imaging and T2 fluid-attenuated inversion recovery (FLAIR) sequence, similar to 5-FU acute leukoencephalopathy. An acute toxic leukoencephalopathy was diagnosed prompting to discontinue capecitabine, which allowed a regression of the symptoms. Though acute toxic leukoencephalopathies with pseudo-stroke presentation have been reported with other chemotherapy agents such as methotrexate or 5-fluorouracil (5-FU), cases of leukoencephalopathy induced by capecitabine are less reported and less well known. This oral precursor of 5-FU is commonly used to treat colorectal, stomach or breast cancers. Neurotoxicity of other 5-FU derivates like cormafur and tergafur have rarely been depicted as well.Although 5-FU-induced leukoencephalopathy is known, the potential toxicity of its precursor should be acknowledged as well. Early detection of chemotherapy-induced toxicity by MRI is crucial as symptoms may be reversible to the condition that chemotherapy is immediately discontinued.

Metronidazole-Induced Encephalopathy Diagnosed by Follow-Up Brain Magnetic Resonance Imaging

Background: Metronidazole-induced encephalopathy (MIE) is caused by consumption of an excessive dose or prolonged use of metronidazole. Case Report: An 83-year-old female presented with left side ataxia and dysarthria. Initial brain magnetic resonance imaging (MRI) showed subtle high signal intensity in the splenium of corpus callosum. Three days later, she developed confused mental status. Follow-up brain MRI showed bilateral high signal intensities in the dentate nuclei of the cerebellum, splenium, insula, and subcortical white matter. She had infectious spondylitis and was taking metronidazole 1 g/day for 44 days. After discontinuation of metronidazole, her mental status and ataxia improved. Conclusion: Although initial brain MRI does not show typical lesions in a patient with clinically suspected MIE, follow-up brain MRI is recommended. Key Words: Metronidazole; Encephalopathy; Brain MRI

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

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). 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. 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. 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.

Acute Myositis of the Tibialis Anterior Muscle after Performance of 108 Prostrations

Exercise-induced muscle damage (EIMD) can be caused by novel or unaccustomed exercise resulting in a temporary decrease in muscle function, increased muscle soreness and swelling, and an increase in muscle proteins in blood. A 38-year-old female presented with a 2-week history of bilateral lower leg pain and swelling that developed suddenly after performing 108 prostrations. Fat-suppressed contrastenhanced T1-weighted magnetic resonance imaging showed bilateral symmetric high signal intensity of the tibialis anterior muscles. Our patient was diagnosed with acute myositis and treated with naproxen. History of physical exertion and acute onset of typical clinical findings of myositis were key elements in the diagnosis. Acute myositis related to exercise is a common and self-limiting condition which fully resolves after 2 to 3 weeks. However, other causes of myositis should be excluded in patients with atypical clinical features.

LRPPRC mutations cause early-onset multisystem mitochondrial disease outside of the French-Canadian population.

Mitochondrial Complex IV [cytochrome c oxidase (COX)] deficiency is one of the most common respiratory chain defects in humans. The clinical phenotypes associated with COX deficiency include liver disease, cardiomyopathy and Leigh syndrome, a neurodegenerative disorder characterized by bilateral high signal lesions in the brainstem and basal ganglia. COX deficiency can result from mutations affecting many different mitochondrial proteins. The French-Canadian variant of COX-deficient Leigh syndrome is unique to the Saguenay-Lac-Saint-Jean region of Québec and is caused by a founder mutation in the LRPPRC gene. This encodes the leucine-rich pentatricopeptide repeat domain protein (LRPPRC), which is involved in post-transcriptional regulation of mitochondrial gene expression. Here, we present the clinical and molecular characterization of novel, recessive LRPPRC gene mutations, identified using whole exome and candidate gene sequencing. The 10 patients come from seven unrelated families of UK-Caucasian, UK-Pakistani, UK-Indian, Turkish and Iraqi origin. They resemble the French-Canadian Leigh syndrome patients in having intermittent severe lactic acidosis and early-onset neurodevelopmental problems with episodes of deterioration. In addition, many of our patients have had neonatal cardiomyopathy or congenital malformations, most commonly affecting the heart and the brain. All patients who were tested had isolated COX deficiency in skeletal muscle. Functional characterization of patients' fibroblasts and skeletal muscle homogenates showed decreased levels of mutant LRPPRC protein and impaired Complex IV enzyme activity, associated with abnormal COX assembly and reduced steady-state levels of numerous oxidative phosphorylation subunits. We also identified a Complex I assembly defect in skeletal muscle, indicating different roles for LRPPRC in post-transcriptional regulation of mitochondrial mRNAs between tissues. Patient fibroblasts showed decreased steady-state levels of mitochondrial mRNAs, although the length of poly(A) tails of mitochondrial transcripts were unaffected. Our study identifies LRPPRC as an important disease-causing gene in an early-onset, multisystem and neurological mitochondrial disease, which should be considered as a cause of COX deficiency even in patients originating outside of the French-Canadian population.

Psychiatric aspects of adult-onset hallervorden-spatz syndrome: a report of two cases

Psychiatric aspects of adult-onset hallervorden-spatz syndrome: a report of two cases Hallervorden-Spatz Syndrome (HSS) is a rare, familial, progressive, and ultimately fatal disorder characterized by extrapyramidal rigidity, dysarthria, pyramidal tract involvement, and dementia, with pathological evidence of iron deposition in the globus pallidus and substantia nigra. The diagnosis is usually made using magnetic resonance imaging (MRI), where bilateral high signal intensity in the globus pallidus surrounded by low signal intensity areas is found. This is called an “eye-of-the-tiger” appearance and is considered specific to this disorder. Psychiatric aspects of this syndrome are common and include cognitive decline, personality changes with impulsivity and violent outbursts, depression and emotional

YOU CANNOT BE SIRIUS!

An 18 year old man was admitted following a suspected seizure after inhalation of the legal high ‘Sirius’. The following day he was still suffering from mild disorientation and impaired short term memory (scoring 20/30 on Montreal Cognitive Assesment). An MRI brain revealed marked bilateral medial temporal lobe high signal which enhanced following contrast. Blood tests were normal and he gradually improved to his normal self over the three days following admission. A repeat MRI is pending to investigate whether the imaging changes have resolved. Sirius is a synthetic cannabinoid receptor agonist that mimics the effects of cannabis. It is available on the internet or in head shops where it is sold as herbal incense. Legal highs (or ‘Novel Psychoactive Substances’) are recreational drugs not covered by current misuse of drugs laws, so are legal to possess or use. With the increase in number, availability, and consumption of these products neurologists need to be aware of the potentially serious neurological risks associated with their use. The novel MRI findings reported in this case suggest that they should also be considered in the differential diagnosis of patients presenting with suspected viral and immune-mediated encephalitides.

A rare cause of bilateral and symmetric high intensity T2 signal of corticospinal tracts at brain MRI: Neurolyme

Neurolyme is defined as the neurological involvement secondary to systemic infection by the spirochete Borrelia. Acute meningoradiculitis is the most common presentation of neurolyme whereas meningoencephalitis is less frequent. We report a case of borreliosis meningo-encephalitis with corticospinal tract involvement.

Reversible Posterior Leukoencephalopathy Syndrome After Blood Transfusion in a Pediatric Patient With Sickle Cell Disease

Children with sickle cell disease are at increased risk for neurological complications. Reversible posterior leukoencephalopathy syndrome is a clinico-radiological syndrome characterized by headache, vomiting, seizures, and visual disturbances along with the radiological findings of posterior leukoencephalopathy. We report a 13-year-old female with sickle cell disease presenting with a headache and two isolated episodes of partial seizures with secondary generalization after blood transfusion. The brain MRI revealed bilateral high signal areas on T2 images in parietal, occipital, and frontal lobes; in the cerebellum; midbrain; and within the right caudate nucleus. Follow-up MRI 2weeks later showed almost complete resolution of the previously detected findings. The clinical presentation along with the reversible aforementioned neuroimaging findings indicated reversible posterior leukoencephalopathy syndrome as the most appropriate diagnosis. Although the syndrome has been described previously in various clinical conditions, this is the first time that it is recognized after blood transfusion in an adolescent with sickle cell disease. Neurological deterioration in children with sickle cell disease after blood transfusion should raise high suspicion for reversible posterior leukoencephalopathy syndrome, especially in the setting of elevated blood pressure.

Progressive striatal necrosis associated with anti-NMDA receptor antibodies

BackgroundWe report a case of childhood onset, generalized dystonia due to slowly progressive bilateral striatal necrosis associated with anti-N-methyl-D-aspartate receptor (NMDAR) antibodies. This clinical phenotype has not been previously associated with NMDA receptor autoimmunity.Case presentationAn eighteen year old man presented with a history of childhood-onset, progressive generalized dystonia. Clinical examination revealed a pure generalized dystonia with no cognitive or other neurological findings. Magnetic resonance imaging showed bilateral high T2 signal striatal lesions, which were slowly progressive over a period of nine years. New parts of the lesion showed restricted water diffusion suggesting cytotoxic oedema. Positron emission tomography of the brain showed frontal hypermetabolism and cerebellar hypometabolism. Antibodies against the NR1 subunit of the NMDA receptor were detected in the patient’s serum and cerebrospinal fluid. There was no neoplasia or preceding infection or vaccination.ConclusionThis is the first report of chronic progressive bilateral striatal necrosis associated with anti-NMDAR antibodies. Our findings expand the clinical spectrum of disease associated with anti-NMDAR antibodies and suggest that these should be included in the work-up of dystonia with striatal necrosis.

P2-041 - A Cese of Colorectal Cancer which was Safely Treated with Folfiri, Although Complicated by Reversible Posterior Leukoencephalopathy Syndrome after Bevacizumab + FOLFOX

ABSTRACT We report a rare case which was safely treated with FOLFIRI, although complicated by reversible posterior leukoencephalopathy syndrome (RPLS) after bevacizumab (Beva) + FOLFOX. A 67-year-old woman had right half colorectomy for cecal carcinoma in July 2011. It had been already shown peritoneal dissemination, and m-FOLFOX6 was carried out from September. She was hospitalized for the purpose of adding on Beva in November, and Beva + m-FOLFOX6 was carried out. Complications of hypertension were seen on day 4, and headache, convulsions and disturbance of consciousness on day 5 after chemotherapy. MRI of the brain revealed bilateral high signal intensities of posterior lobes on T2 weighted and FLAIR images without enhancement. She was diagnosed as RPLS and referred to our department. She was treated with antihypertensive and anticonvulsive therapy. Her symptoms entirely disappeared after 12 days. The causing agent of RPLS was not clear, but we administered a single agent, CPT-11 which is one of the key drags for colorectal cancer and then possible to treat with FOLFIRI safely. Medical oncologists should be aware that multidrug chemotherapies may increase the risk of fatal neurological complications like RPLS.

Hirayama disease: An uncommon cause of distal upper extremity deficit

Hirayama disease is a myelopathy related to flexion movements of the neck that produce ischemic damage in the anterior horn of the cervical cord. The disease affects young people and is characterized by a distal upper extremity deficit that develops gradually. The diagnosis is confirmed by cervical magnetic resonance imaging (MRI) in flexion demonstrating a forward shift of the posterior dural sac and spinal cord compression. On radiology, there should be suggestive signs in neutral position such as cord atrophy, intramedullary bilateral high signal intensity on T2-weighted images, straightening of the cervical spine, and loss of attachment between the posterior dural sac and subjacent lamina. Exploration should be completed by an MR study in neck flexion. We report here on two typical cases of the disease and also include a review of the literature.

Ingestion of gasoline in a suicide attempt: an uncommon cause of bilateral basal ganglia T1 hyperintensities

A 70-year-old man with a 27-year history of psychosis and hypertension was brought comatose to our Emergency Room following a suicide attempt with oral unleaded gasoline ingestion that was witnessed and described by his neighbors. His neurological examination disclosed a Glasgow Coma Scale Score of 8 (eye opening response 2 points, verbal response 2 points, motor response 2 points), with normal brainstem reflexes and spontaneous breathing. The subject was treated with a gastric lavage (gastric content, almost 2 l of gasoline) and activated charcoal. A baseline brain CT was unremarkable, while his liver function tests were normal. His neurological status did not improve and was transferred to the Intensive Care Unit of our institution where severe aspiration pneumonia was diagnosed. A brain MRI was performed at 16 days following his suicide attempt. Bilateral hyperintensities affecting the globus pallidus and the caudate nucleus were shown on axial T1, T2 and FLAIR sequences (Fig. 1). The patient expired 3 weeks later because of his aspiration pneumonia. Lesions with high signal intensity on spin-echo Tl-weighted MR images are unusual and are associated with relatively few entities, including lipids, calcification, high protein count, laminar necrosis in cerebral infarction, neoplasms and paramagnetic substances such as methemoglobin, melanin, copper and manganese [1]. Manganese, in particular, is considered a neurotoxic agent causing movement and psychological disorders characterized by rigidity, tremor and hypokinesia in workers involved in the alloy industry (miners, welders, smelters), in patients receiving long-term total parenteral nutrition and in hepatic encephalopathy patients with portal liver shunts [1–3]. Since the introduction of MRI in clinical practice, bilateral symmetric high signal intensities at the globus pallidus and substantia nigra have been well described in patients with manganese neurotoxicity. The most plausible explanation for the increased T1 signal intensity is a rise in manganese concentration that has paramagnetic properties in the central nervous system (CNS), with preferential deposition in the globus pallidus. Methylcyclopentadienyl manganese tricarbonyl (MMT) is an octane-improving additive to unleaded gasoline, which has recently attracted attention with regard to the prospect of worldwide manganese exposure as increases in environmental manganese concentrations have been recorded relative to traffic density [4]. We postulate that in the present case oral ingestion of large quantities of gasoline resulted in MMT absorption, which in turn resulted in increased CNS manganese concentrations leading to bilateral T1 basal ganglia hyperintensities. However, it should be noted that manganese concentrations were not measured in blood, while we have no neuropathological confirmation of manganese deposition in globus pallidus. In conclusion, the present case highlights bilateral T1 basal ganglia hyperintensities related to oral ingestion of gasoline in a suicide case and underscores manganese neurotoxicity as the potential underlying cause of this uncommon neuroimaging presentation. G. Tsivgoulis (&) I. Heliopoulos K. Vadikolias C. Piperidou Department of Neurology, Democritus University of Thrace, University Hospital of Alexandroupolis, Kapodistriou 3, Nea Xili, 68100 Alexandroupolis, Greece e-mail: tsivgoulisgiorg@yahoo.gr

POC18 An encephalopathy worth its salt

A 67-year-old female was admitted to hospital with acute cholecystitis, she was treated with gentamicin and cefotaxime. She became confused and complained of flashing coloured lights in both visual fields....