Identifying patients with neuronal intranuclear inclusion disease in Singapore using characteristic diffusion-weighted MR images.

PURPOSE: Adult-onset neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disorder described mainly in the Japanese population, with characteristic DWI abnormalities at the junction between gray and white matter. We identify possible cases of NIID in the picture archive and communication system (PACS) of a tertiary neurological referral hospital in Singapore and describe their radiological features. METHODS: The neuroradiology imaging database was reviewed using keyword search of radiological reports to identify patients who had "subcortical U fibre" abnormalities on DWI. MRI were retrospectively reviewed, and those fulfilling inclusion criteria were invited for skin biopsy to detect nuclear inclusions by light and electron microscopy. RESULTS: Twelve Chinese patients (nine female; median age 70.5 years) were enrolled. Seven patients were being assessed for dementia and five for other neurological indications. In all patients, DWI showed distinctive subcortical high signal with increased average apparent diffusion coefficient (ADC), involving frontal, parietal, and temporal more than occipital lobes; the corpus callosum and external capsule were affected in some patients. On T2-weighted images, cerebral and cerebellar atrophy and white matter hyperintensity of Fazekas grade 2 and above were seen in all patients. Three patients underwent skin biopsy; all were positive for intranuclear hyaline inclusion bodies on either p62 staining or electron microscopy, which are pathognomonic for NIID. CONCLUSION: Previously undiagnosed patients with NIID can be identified by searching for abnormalities at the junction between gray and white matter on DWI in PACS and subsequently confirmed by skin biopsy. Radiologists should recognize the distinctive neuroimaging pattern of this dementing disease.

Diffusion-weighted MRI abnormalities in an outbreak of Streptococcus agalactiae Serotype III, multilocus sequence type 283 meningitis.

PURPOSE: In 2015, an outbreak of group B streptococcal (GBS) infection caused by Streptococcus agalactiae Serotype III, multilocus sequence type 283, related to consuming infected raw freshwater fish, affected more than 200 patients in Singapore. We describe the clinical, laboratory, and neuroimaging features of a subgroup of adults with central nervous system (CNS) infections caused by GBS. MATERIALS AND METHODS: The database of the Singapore Neurologic Infections Program (SNIP), a national multicenter study for surveillance of infectious neurologic disease, was reviewed to select patients with GBS CNS infection during the outbreak. Cases were diagnosed on the basis of clinical features, cerebrospinal fluid (CSF) findings and identification or isolation of Streptococcus agalactiae in the blood or CSF. Demographic, clinical and neuroradiological information was obtained prospectively and retrospectively abstracted. RESULTS: Fourteen patients (6 male, 8 female; median age, 58 years) presented with fever, meningism, headache, encephalopathy, focal neurological deficits, and/or seizures. All except two were previously healthy. Diffusion-weighted imaging (DWI) on admission was abnormal in 13 patients, showing tiny hyperintensities in the subarachnoid space (7 patients), ventricles (6 patients) and brain parenchyma (8 patients); 5 patients had cerebellar abnormalities. CONCLUSION: Among healthy non-pregnant adults infected with Serotype III, multilocus sequence type 283 GBS meningitis linked to eating infected raw freshwater fish, DWI detected small pus collections and unusual cerebellar involvement. A collaborative national surveillance system that includes MRI can be helpful during unusual food-borne zoonotic infectious disease outbreaks. LEVEL OF EVIDENCE: 4 J. Magn. Reson. Imaging 2017;45:507-514.

Clandestine charisma of the charm needles: a radiologist's challenge.

A 94-year-old Korean woman was seen in the Emergency Department after a fall. CT examination of the maxillofacial region found multiple small linear metallic densities in the subcutaneous soft tissues of the face. The appearance of these densities was unchanged when compared to a study performed 2 years prior; however, the imaging interpretations of these densities were markedly different. Additional imaging during the course of her hospital stay demonstrated numerous similar densities in the breasts, abdomen, hips, and legs-finally diagnosed as "charm needles." Although common practice in Southeast Asia, with ever increasing globalization, these needles, or "susuks," are being seen with greater frequency in North America. Here, we review the imaging appearance of a bizarre case of these charm needles so as to raise awareness of this potential diagnostic challenge and help the radiologist avoid confusion when interpreting images.

Differential diagnosis for bilateral abnormalities of the basal ganglia and thalamus.

The basal ganglia and thalamus are paired deep gray matter structures that may be involved by a wide variety of disease entities. The basal ganglia are highly metabolically active and are symmetrically affected in toxic poisoning, metabolic abnormalities, and neurodegeneration with brain iron accumulation. Both the basal ganglia and thalamus may be affected by other systemic or metabolic disease, degenerative disease, and vascular conditions. Focal flavivirus infections, toxoplasmosis, and primary central nervous system lymphoma may also involve both deep gray matter structures. The thalamus is more typically affected alone by focal conditions than by systemic disease. Radiologists may detect bilateral abnormalities of the basal ganglia and thalamus in different acute and chronic clinical situations, and although magnetic resonance (MR) imaging is the modality of choice for evaluation, the correct diagnosis can be made only by taking all relevant clinical and laboratory information into account. The neuroimaging diagnosis is influenced not only by detection of specific MR imaging features such as restricted diffusion and the presence of hemorrhage, but also by detection of abnormalities involving other parts of the brain, especially the cerebral cortex, brainstem, and white matter. Judicious use of confirmatory neuroimaging investigations, especially diffusion-weighted imaging, MR angiography, MR venography, and MR spectroscopy during the same examination, may help improve characterization of these abnormalities and help narrow the differential diagnosis.

Severe hypoglycemia associated with an illegal sexual enhancement product adulterated with glibenclamide: MR imaging findings.

PURPOSE: To describe the magnetic resonance (MR) imaging findings associated with severe hypoglycemia after consumption of an illegal sexual enhancement product (Power 1 Walnut) adulterated with glibenclamide, an oral hypoglycemic agent used to treat diabetes mellitus. MATERIALS AND METHODS: Institutional review board approval was obtained for this retrospective study. Records in eight male patients with severe hypoglycemia of unknown cause, without prior treatment for diabetes, and with positive blood toxicology results for glibenclamide were reviewed. MR imaging included diffusion-weighted imaging and, in some patients, MR angiography, dynamic contrast material-enhanced perfusion MR imaging, and MR spectroscopy. RESULTS: In seven patients, there were hyperintense abnormalities on diffusion-weighted and T2-weighted images in the hippocampus and cerebral cortex, sparing the subcortical white matter and cerebellum. Three patients had abnormalities of the splenium of the corpus callosum, and one had widespread involvement, including the caudate nucleus, basal ganglia, and internal capsule bilaterally. In three patients, unilateral cortical involvement, which did not conform to the typical cerebral arterial territories, was noted. In one patient, perfusion MR imaging showed slightly increased relative cerebral blood volume, and MR spectroscopy revealed no evidence of abnormal lactate in the affected cerebral cortex. CONCLUSION: Diffusion-weighted MR imaging findings in patients with severe hypoglycemia showed typical lesions in the hippocampus and cerebral cortex, but the caudate nucleus and basal ganglia were involved in only the most severely affected patient. The splenium of the corpus callosum and internal capsule were also abnormal in three patients, and unilateral cortical lesions could be distinguished from acute ischemic stroke by the pattern of involvement and MR angiographic, perfusion, and spectroscopic findings.

Hyperglycemic choreoathetosis: role of the putamen in pathogenesis.

Hyperglycemic choreoathetosis (HC) is an uncommon syndrome often associated with hyperintensity of the basal ganglia on MRI. We performed a retrospective review of cases with HC to characterize the clinical, biochemical, and neuroimaging (CT, MRI, and MR spectroscopy) findings and to propose a mechanism for this syndrome. Seven HC patients with a mean age of 75.1 years, mean blood glucose of 27.4 mmol/L, and mean plasma osmolarity of 313.4 mmol/L were studied. All had MR-T1 hyperintensity of the putamen on the side contralateral to the choreoathetosis. Two patients had additional hyperintensity of the globus pallidus while one also had involvement of the caudate. On MR-T2, 2 patients showed hyperintensity, 2 isointensity, and 3 hypointensity in the putamen. MR spectroscopy showed elevated choline and reduced N-acetylaspartate; two patients also had elevated myoinositol levels. Our findings suggest that the putamen has a central role in HC, and MR spectroscopy supports neuronal dysfunction in the putamen. Biochemical and neuroimaging findings support hyperviscosity as the most plausible mechanism.

Territorial arterial spin labeling in the assessment of collateral circulation: comparison with digital subtraction angiography.

BACKGROUND AND PURPOSE: Collateral circulation plays a vital role in patients with steno-occlusive disease, in particular for predicting stroke outcome. Digital subtraction angiography (DSA) is the gold standard for the assessment of collateral circulation, despite its invasive nature. Recently, the development of a new class of arterial spin labeling (ASL) methods allowed independent measurement of territorial flow information without the need for contrast media injection. Here, we compared combined territorial ASL (TASL) and MR angiography (MRA) against DSA in the assessment of collateral circulation. METHODS: Eighteen patients presenting with extra- or intracranial arterial steno-occlusive disease were recruited. All DSA studies were performed using a biplane angiography unit. MR imaging consisted of time-of-flight MRA and TASL, performed at 3T. Collateral circulation on both modalities was evaluated in consensus in a double-blinded manner by 3 neuroradiologists. RESULTS: Good agreement was found between DSA and TASL in the assessment of collateral flow: Cramer coefficient, V=0.53 (P<0.0001) and Contingency coefficient, C=0.67, with kappa=0.70 and kappa=0.72 in the assessment of flow and collaterals, respectively. TASL and DSA successfully evaluated 89% and 98% of the vessels, respectfully. Failure was linked to motion-related artifacts in TASL, and highly tortuous vessels in DSA. Generally, combined MRA-TASL was comparable to DSA in diagnostic quality. CONCLUSIONS: TASL provided radiological information comparable to DSA on collateral flow, with the advantage that it could be performed during routine MRI studies. TASL may provide insight on collateral perfusion in patients who may not otherwise be candidates for DSA, and may potentially replace it.

MRI in cerebral schistosomiasis: characteristic nodular enhancement in 33 patients.

OBJECTIVE: The purpose of our study was to describe the characteristic MRI appearance of cerebral infection with Schistosoma japonicum. CONCLUSION: Cerebral infection with S. japonicum can cause a characteristic MRI pattern of a large mass comprising multiple intensely enhancing nodules, sometimes with areas of linear enhancement. The typical appearance may be useful for diagnosis in endemic regions and may potentially be useful in cases imported into countries in which the disease is not endemic.

Central neurocytoma: typical magnetic resonance spectroscopy findings and atypical ventricular dissemination.

PURPOSE: Central neurocytomas (CNCs) are rare neuronal tumors that have a favorable prognosis and lower rate of recurrence compared with other intraventricular neoplasms. Although it may be difficult to distinguish CNC on conventional neuroimaging, typical MR spectroscopy (MRS) features have been reported. We describe the MRI and MRS features of CNC. MATERIALS AND METHODS: Eight patients with CNC were reviewed. Three patients underwent presurgical in vivo single-voxel MRS at short echo time (TE, 35 ms) and multi-voxel MR spectroscopic imaging at long TE (144 ms). The surgically resected tumor specimen of one of these patients was also studied ex vivo using high-resolution magic angle spinning (HRMAS) nuclear magnetic resonance. RESULTS: All eight tumors were located in the lateral ventricles. In six patients, CNC extended into the third ventricle, and in two patients the tumor showed further contiguous intraventricular dissemination into the fourth ventricle. In all three patients who underwent MRS, a characteristic metabolite peak was detected at 3.55 parts per million (ppm) at both long and short TE. HRMAS confirmed the presence of elevated glycine (Gly) at 3.55 ppm, without increase in the concentration of myo-inositol found at the same chemical shift. Elevated choline (at 3.2 ppm) was also seen in all three patients. CONCLUSION: On MRS, CNCs have a typical appearance with a metabolite peak at 3.55 ppm due to increased Gly, and this feature may be helpful in presurgical diagnosis. Although they are rare benign intraventricular tumors, in atypical cases, CNCs can show extensive intraventricular dissemination into the fourth ventricle.

Superior sagittal sinus thrombosis: subtle signs on neuroimaging.

INTRODUCTION: The aim of this study was to review the clinical, computed tomography (CT) and magnetic resonance imaging (MRI) diagnosis and the frequency of positive neuroimaging findings in patients with cerebral venous thrombosis (CVT) involving the superior sagittal sinus. MATERIALS AND METHODS: A clinical and radiological database of patients with final diagnosis of CVT was compiled from the inpatient hospital information service of a tertiary neurological hospital over 5 years. CT and MRI studies in 22 patients were retrospectively examined for direct signs of venous sinus thrombosis and for complications of CVT. The diagnosis of CVT before and after CT and MRI was reviewed. RESULTS: Clinical diagnosis of possible CVT was suspected in only 1 patient. When the diagnosis was not suspected, CT diagnosis was difficult and there was a high false negative rate of 52.6%. MRI fared better, but the false negative rate was still 11%. Directs signs of venous sinus thrombosis such as the triangle sign, empty delta sign on CT and loss of the normal flow voids on MRI, could be retrospectively detected in 57.9%, 100% and 100% of patients respectively. Although 4 patients presented with subarachnoid haemorrhage, these direct signs were present in 3 patients. CONCLUSION: Clinical diagnosis of CVT is rarely suspected before CT and MRI, and although subtle positive signs are often present, these may not be appreciated unless there is a high index of suspicion or image review at multidisciplinary team meetings.

Corticospinal tract degeneration in amyotrophic lateral sclerosis: a diffusion tensor imaging and fibre tractography study.

INTRODUCTION: Motor neuron damage and cortical spinal tract (CST) degeneration in amyotrophic lateral sclerosis (ALS) are difficult to visualise and quantify on conventional magnetic resonance imaging (MRI). CLINICAL PICTURE: We studied 8 ALS patients and 12 normal volunteers using diffusion tensor imaging (DTI) and fibre tractography using fibre assignment by continuous tracking (FACT) to study the fibres of the CST and the posterior thalamic radiation (PTR), a nonmotor tract. OUTCOME: Fibre tractography was successfully performed in all normal volunteers and all patients except 1. The fibre bundles of the CST, but not the PTR, were significantly reduced (P <0.05) in patients compared to normal volunteers. CONCLUSION: Fibre tractography can visualise axonal degeneration in the CST and may provide supplementary information about upper motor neuron disease in ALS patients.

Magnetic resonance spectroscopy in adult-onset citrullinemia: elevated glutamine levels in comatose patients.

BACKGROUND: Adult-onset type II citrullinemia is an inborn error of urea cycle metabolism that can lead to hyperammonemic encephalopathy and coma. However, type II citrullinemia is rare outside Japan, and diagnosis and treatment can be delayed. Magnetic resonance spectroscopy may be a useful adjunct to magnetic resonance imaging, and has been applied to noninvasively study chemical metabolism in the human brain. PATIENTS: We describe 2 patients with type II citrullinemia who presented with episodic postprandial somnolence and coma. Diffusion-weighted magnetic resonance imaging showed bilaterally symmetrical signal abnormalities of the insular cortex and cingulate gyrus. On magnetic resonance spectroscopy, glutamine and glutamate levels were elevated, and choline and myo-inositol levels were decreased. The diagnosis of citrullinemia was confirmed based on elevated plasma ammonia and citrulline levels. CONCLUSION: Characteristic features found at the time of magnetic resonance imaging and magnetic resonance spectroscopy may be helpful for early diagnosis of type II citrullinemia in adult patients who present with hyperammonemic encephalopathy and coma.

Decompression sickness: MRI of the spinal cord.

Decompression sickness (DCS) typically causes changes in the white matter of the spinal cord on MR imaging. We present a case of DCS in a scuba diver with dorsal white matter lesions typical of venous infarction. In addition, some central gray matter involvement was noted. Characteristic features of venous spinal cord infarction can be recognized on MR imaging in DCS but may be more extensive in severe cases.

Elevated thyroid peroxidase antibodies with encephalopathy in MELAS syndrome.

Both the syndrome of mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS syndrome) and Hashimoto's encephalopathy can present with nonspecific encephalopathy. Hashimoto's encephalopathy is an association of steroid-responsive encephalopathy with elevated thyroid peroxidase antibodies. Steroid-responsive encephalopathy, however, is not characteristic of the MELAS syndrome, which typically presents with stroke-like episodes and lactic acidosis in cerebrospinal fluid and blood. Here, a patient is described with goiter, recurrent encephalopathy and elevated thyroid peroxidase antibodies who apparently responded to steroid therapy; however, magnetic resonance imaging was atypical for Hashimoto's encephalopathy, and she was diagnosed with MELAS syndrome. This syndrome can present with apparent steroid-responsive encephalopathy and elevated thyroid peroxidase antibodies, mimicking Hashimoto's encephalopathy, and should be suspected if lactic acidosis is present and typical features are detected on magnetic resonance imaging.

Multivoxel MR spectroscopic imaging--distinguishing intracranial tumours from non-neoplastic disease.

INTRODUCTION: Multi-voxel MR spectroscopic imaging (MRSI) provides chemical metabolite information that can supplement conventional MR imaging in the study of intracranial neoplasia. Our purpose was to use a robust semi-automated spectroscopic analysis to distinguish intracranial tumours from non-neoplastic disease. MATERIALS AND METHODS: Twenty intracranial tumours and 15 patients with non-neoplastic disease confirmed on histological examination or serial neuroimaging were studied with 2-dimensional MRSI using point-resolved spectroscopic (PRESS) imaging localisation. Using semi-automated post-processing software, spectra were analysed for peak heights of choline (Cho), creatine (Cr), N-acetyl aspartate (NAA), lactate (Lac) and lipid (Lip). Normalised Cho (nCho) ratios, computed by dividing maximum Cho in the lesion by the normal-appearing brain, were compared between intracranial tumours and non-neoplastic disease. RESULTS: Meningiomas displayed homogeneously elevated Cho. Malignant tumours, especially large glioblastoma multiforme, displayed inhomogeneity of metabolites within the tumour. All tumours had elevation of nCho >1 (mean 1.91 +/- 0.65), and non-neoplastic diseases had tumour nCho <1 (mean 0.91 +/- 0.46), which was significantly lower (P <0.05). Two patients with non-neoplastic lesions, one with subacute cerebral infarction and the other with cryptococcoma, had elevated Cho compared to normal tissue (false positive rate 13%). CONCLUSION: Using semi-automated MRSI method, a simplified normalised Cho algorithm provides a method to distinguish intracranial tumours from non-neoplastic disease.