Abnormal Signal Intensity Research Articles

Detection of clinically manifest and silent synovitis in the hands and wrists by fluorescence optical imaging

ObjectivesThe correct identification of synovitis is critical for achieving optimal therapy results. Fluorescence optical imaging (FOI) is a novel modality based on the use of an intravenous fluorophore, which enables...

Predicting developmental outcomes in premature infants by term equivalent MRI: systematic review and meta-analysis.

BackgroundThis study aims to determine the prognostic accuracy of term MRI in very preterm born (≤32 weeks) or low-birth-weight (≤1500 g) infants for long-term (>18 months) developmental outcomes.MethodsWe performed a systematic review searching Central, Medline, Embase, and PsycInfo. Two independent reviewers performed study selection, data extraction, and quality assessment. We documented sensitivity and specificity for three different MRI findings (white matter abnormalities (WMA), brain abnormality (BA), and diffuse excessive high signal intensity (DEHSI)), related to developmental outcomes including cerebral palsy (CP), visual and/or hearing problems, motor, neurocognitive, and behavioral function. Using bivariate meta-analysis, we estimated pooled sensitivity and specificity and plotted summary receiver operating characteristic (sROC) curves for different cut-offs of MRI.ResultsWe included 20 papers published between 2000 and 2013. Quality of included studies varied. Pooled sensitivity and specificity values (95 % confidence interval (CI)) for prediction of CP combining the three different MRI findings (using normal/mild vs. moderate/severe cut-off) were 77 % (53 to 91 %) and 79 % (51 to 93 %), respectively. For prediction of motor function, the values were 72 % (52 to 86 %) and 62 % (29 to 87 %), respectively. Prognostic accuracy for visual and/or hearing problems, neurocognitive, and/or behavioral function was poor. sROC curves of the individual MRI findings showed that presence of WMA provided the best prognostic accuracy whereas DEHSI did not show any potential prognostic accuracy.ConclusionsThis study shows that presence of moderate/severe WMA on MRI around term equivalent age can predict CP and motor function in very preterm or low-birth-weight infants with moderate sensitivity and specificity. Its ability to predict other long-term outcomes such as neurocognitive and behavioral impairments is limited. Also, other white matter related tests as BA and DEHSI demonstrated limited prognostic value.Systematic review registrationPROSPERO CRD42013006362Electronic supplementary materialThe online version of this article (doi:10.1186/s13643-015-0058-7) contains supplementary material, which is available to authorized users.

MRI of a syrinx: is contrast material always necessary?

The workup of a syrinx often includes contrast-enhanced MRI to exclude the presence of an underlying mass. The diagnostic yield of performing these additional contrast-enhanced sequences is not well defined in the literature. We hypothesized that T2-weighted imaging alone could reliably exclude the presence of a syrinx-associated mass without the need for contrast-enhanced imaging sequences in all cases. Two independent readers retrospectively analyzed contrast-enhanced MRI studies of 87 consecutive patients with syringes. The presence or absence of an associated spinal cord mass was determined using only T2-weighted imaging. The imaging features considered positive for a possible syrinx-associated lesion on T2-weighted imaging were syrinx nodularity, syrinx septations, and a spinal cord signal intensity abnormality or a mass separate from the syrinx. The size of the syrinx was also recorded. Using contrast-enhanced sequences as the reference standard, statistical analysis was performed to determine the accuracy of T2-weighted imaging in detecting a syrinx-associated mass. Of the 87 cases of syrinx, there were 23 mass lesions, 11 Chiari malformations, three spinal cord contusions, and 50 idiopathic syringes. Using T2-weighted imaging alone, readers detected no findings suspicious for a syrinx-associated mass in 55 cases and detected findings suspicious for a mass in 32 of 87 cases. Reader sensitivity for an underlying mass lesion was 100%; specificity, 86%; positive predictive value, 72%; and negative predictive value, 100%. Interreader agreement was excellent (κ = 0.88). Syrinx size showed a positive correlation with the presence of a mass lesion (p < 0.0001). T2-weighted imaging alone appears to have a high sensitivity and high negative predictive value in evaluating for a syrinx-associated mass, and contrast-enhanced imaging may not be required for the workup of a syrinx.

Hyperintense Optic Nerve due to Diffusion Restriction: Diffusion-Weighted Imaging in Traumatic Optic Neuropathy.

Abnormal signal intensity of the optic nerve due to diffusion restriction may be seen in traumatic optic neuropathy. In addition to evaluating optic nerve hyperintensity on diffusion-weighted imaging, we compared the group differences of ADC values between the injured and uninjured contralateral nerve and identified the relation between measured ADC values and admission visual acuity. We retrospectively evaluated 29 patients with traumatic optic neuropathy who underwent MR imaging with DWI. Uninjured contralateral optic nerves were used as controls. Two attending radiologists, blinded to the side of injury, independently reviewed the DWI for the presence of signal-intensity abnormality and obtained ADC values after manually selecting the ROI. Hyperintensity of the optic nerve was demonstrated in 8 of the 29 patients, with a sensitivity of 27.6% (95% CI, 12.8-47.2) and a specificity of 100% (95% CI, 87.9-100). ADC values were obtained in 25 patients. The mean ADC in the posterior segment of the injured nerve was significantly lower than that in the contralateral uninjured nerve (Welch ANOVA, F = 9.7, P = .003). There was a moderate-to-strong correlation between low ADC values and poor visual acuity in 10 patients in whom visual acuity could be obtained at admission (R = 0.7, P = .02). Patients with optic nerve hyperintensity presented with worse visual acuity. Hyperintensity of the optic nerve due to diffusion restriction can serve as a specific imaging marker of traumatic optic neuropathy. When paired with reduced ADC values, this finding may be an important surrogate for visual acuity.

Comparison of Self-Expanding Stents With Distal Embolic Protection to Balloon-Expandable Stents Without a Protection Device in the Treatment of Symptomatic Vertebral Artery Origin Stenosis

Purpose: To compare the angiographic and clinical outcomes of self-expanding stents (SES) with distal embolic protection devices (EPD) vs balloon-expandable stents (BES) without EPD in the treatment of symptomatic atherosclerotic vertebral artery ostial stenosis (VAOS). Methods: Between July 2011 and March 2013, a prospective randomized trial was conducted involving 127 patients (mean age 67.3±10.2 years; 94 men) with symptomatic VAOS randomly assigned to treatment with SES + EPD (Precise RX or RX Acculink stent + Spider FX EPD; n=61) or BES (Palmaz Blue or Resolute RX; n=66) without EPD. In-stent restenosis (ISR) >50% detected by duplex ultrasound was the primary endpoint. Technical success, clinical success, complications within 30 days, and signal intensity abnormalities on diffusion weighted imaging (DWI) after stenting were compared. Results: The 30-day technical success rate was 95.5% (63/66) for SES+EPD vs 100% (70/70) for BES without EPD (p=0.072). DWI at 24 hours poststenting showed 2 hyperintense lesions in 2 (3.3%) SES + EPD cases and 15 hyperintense lesions in 13 (18.6%) BES patients (p<0.01). At a mean 18-month follow-up, the clinical success rate was 93.9% (62/66) for the SES + EPD group vs 85.7% (60/70) for the BES group (p=0.115). The ISR was seen in 16/70 (22.9%) arteries in the BES group and 2/66 (3.1%) arteries in SES + EPD group (p<0.01). Target vessel revascularization was performed in 7 (10.0%) BES arteries vs none in the SES + EPD group (p<0.01). Conclusion: SES with EPD in the treatment of symptomatic VAOS is technically feasible and safe, with low rates of ISR and significantly reduced thromboembolic events on imaging when compared to BES without EPD.

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EARLY POSTOPERATIVE MAGNETIC RESONANCE IMAGING FINDINGS IN FIVE DOGS WITH CONFIRMED AND SUSPECTED BRAIN TUMORS.

Early postoperative neuroimaging has been performed in people for over 20 years to detect residual brain tumor tissue and surgical complications. The purpose of this retrospective study was to describe characteristics observed using early postoperative magnetic resonance imaging in a group of dogs undergoing craniotomy for brain tumor removal. Two independent observers came to a consensus opinion for presence/absence of the following MRI characteristics: residual tumor tissue; hemorrhage and ischemic lesions; abnormal enhancement (including the margins of the resection cavity, choroid plexus, meninges) and signal intensity changes on diffusion-weighted imaging. Five dogs were included in the study, having had preoperative and early postoperative MRI acquired within four days after surgery. The most commonly observed characteristics were abnormal meningeal enhancement, linear enhancement at margins of the resection cavity, hemorrhage, and a thin rim of hyperintensity surrounding the resection cavity on diffusion-weighted imaging. Residual tumor tissue was detected in one case of an enhancing tumor and in one case of a tumor containing areas of hemorrhage preoperatively. Residual tumor tissue was suspected but could not be confirmed when tumors were nonenhancing. Findings supported the use of early postoperative MRI as a method for detecting residual brain tumor tissue in dogs.

1,2-Dichloroethane-induced toxic encephalopathy: A case series with morphological investigations

1,2-Dichloroethane (DCE) is commonly used as an industrial organic solvent and causes occupational diseases. Toxic encephalopathy is the most common and serious disorder resulting from DCE intoxication. Five patients who worked in shoemaking, pipemaking or a paint factory were identified as suffering from severe encephalopathy due to DCE intoxication. DCE-induced toxic encephalopathy manifests as various neurological deficits, with changes observable by neuroimaging. The main clinical manifestation is headache accompanied by intracranial hypertension. The typical cranial CT/MR scan of DCE toxic encephalopathy shows extensive brain edema and diffuse, symmetric, abnormal signal intensities in the cerebellar dentate nucleus, basal ganglia, and white matter in the bilateral cerebral hemispheres. The diagnosis of DCE toxic encephalopathy can be confirmed by the typical clinical and neuroimaging characteristics together with a history of exposure to substances contaminated with DCE. Dehydrating agents and glucocorticoids are the primary treatments. With early diagnosis and prompt treatment, promising results and recovery can be achieved. Effective prevention is expected to reduce the incidence of DCE toxic encephalopathy.

Characterization of both myocardial extracellular volume expansion and myocyte hypertrophy by CMR in heart transplantation recipients without active rejection: implications for early cardiac remodeling

Background Left ventricular hypertrophy (LVH) after heart transplant (HTx) is multifactorial, associations include hypertension, chronic immune injury and the intrinsic effects of immunosuppression. Its consequences are significant and potentially provide an explanation for the development of diastolic dysfunction and exercise intolerance, as well as the limited life expectancy after HTx. Both expansion of myocardial extracellular volume (ECV) and myocyte hypertrophy (MH) coexist in this setting. Cardiac biopsies have limitations and may be non-representative to assess global myocardial remodeling. The goal of this pilot study was to characterize both ECV and MH by CMR in cohort of HTx recipients without active rejection. Methods T1 relaxation times were measured before and after gadolinium contrast. The intracellular lifetime of water (τic), a cell size-dependent parameter, and extracellular volume fraction, a marker of interstitial fibrosis, were determined with a model for transcytolemmal water exchange. Results Nineteen HTx recipients (mean age 50±0, 6 female, BSA 1,70±0,16m 2 , median follow-up after HTx 11±13 months) without acute rejection and 20 age matched health volunteers (mean age 51±14) underwent CMR (1.5T) includingmeasurement of LV function, T2, T1 mapping pre- and post-gadolinium and LGE, and a echocardiogram for measurement of diastolic function. HTx recipients demonstrated normal LVEF (68±11%) with a significant increased in LVMass in comparison with age-matched volunteers (LVMass 114±19g vs. 80±5g; p<0.05). Both groups (HTx and controls) did not show LGE or abnormal signal intensity in T2 images. ECV was substantially higher in HTx patients (0,43 ±0,14) compared with volunteers (0.29±0.03, p<0.0001). Both ECV, a marker of interstitial fibrosis, and τic ,a new validated maker of myocyte hypertrophy, were significantly associated with LV mass (r=0.72 and r=0.68 respectively , both p<0.05). ECV and τic also demonstrated a strong association with E wave deceleration time (EDT) by TTE (r=0.77 and 0.74 respectively, both p<0.05). ECV maintained the positive association with EDT indexed to E wave. (r=0.66, p<0.01). by ROC curve analysis, the ECV was able to predict diastolic dysfunction using EDT by ETT with AUC 0.97 (ECV cut value 0.37, Sens 100%, Spec 86%, p<0.01).

Neuromyelitis Optica: Atypical Clinical and Neuroradiological Presentation

The extreme variability of clinical and MRI findings in the suspicion of Devic's disease always requires the detection of specific antibodies (AQP4). MRI scans were performed with a high-field MRI scanner (1.5T General Electric Signa Horizon): the MRI protocol of the brain employed axial DP, T2, T1, FLAIR and DWI weighted images (wi) and coronal T2-wi. After intravenous administration of contrast medium axial and sagittal T1-weighted images of the brain were repeated. The spine protocol employed after contrast medium included sagittal T2-wi, T2-wi with fat suppression and T1-wi. In May 2004, a 64-year-old healthy Caucasian woman began to suffer loss of motor and thermal sensitivity in the left lower limb. MRI showed non-specific areas of abnormal signal intensity on the deep left frontal and right frontoparietal white matter with no pathological enhancement after contrast medium and a small intramedullary area of altered signal at metameric level C2-C4 with a diagnosis of post-viral transverse myelitis. The patient had two similar episodes years later so the neurologist decided to search for circulating IgG AQP4 with the definitive diagnosis of neuromyelitis optica. In this case, compared to a clinical presentation of atypical deficit neurological involvement, the neuroradiological results of a progressive diffuse involvement of the white matter were atypical.

Epstein-Barr Virus Encephalitis: A Case Report

Many neurologic manifestations of Epstein-Barr virus (EBV) infection have been documented, including encephalitis, aseptic meningitis, transverse myelitis, and Guillain-Barre syndrome. These manifestations can occur alone or coincidentally with the clinical picture of infectious mononucleosis. EBV encephalitis is rare and is indicated as a wide range of clinical manifestations. We report a 10-year-old girl presented with fever, gait disturbance, and bizarre behavior for one week. The results of the physical examination were unremarkable. The diagnosis of EBV encephalitis was made by changes in titers of EBV specific antibodies and MRI findings. A cranial MRI demonstrated abnormal high signal intensities in the basal ganglia and the striatal body, especially in the putamen and caudate nucleus. EBV infection should be considered when lesions are localized to the basal ganglia.

Imaging findings and morphology classification of intraductal papillary mucinous neoplasm of the bile duct

Objective To investigate the CT and MRI features and morphology classification of intraductal papillary mucinous neoplasm of the bile duct (IPMN-B). Methods A total of 18 patients with IPMN-B proved by pathology were retrospectively analyzed. Out of 18 patients, 16 patients underwent enhanced and non-enhanced CT, 13 underwent contrast enhanced MR, and 11 out of 13 underwent both CT and MRI. IPMN-B was classified into 4 types: typical IPMN-B, cystic-forming IPMN-B, non-tumor IPMN-B and invasive IPMN-B, according to imaging findings and gross pathological findings. Results Typical IPMN-B (9 cases): tumors were distributed along the bile ducts, both upstream and downstream bile ducts were obviously dilated. Cystic-forming IPMN-B (5 cases): single or multiple tumors were found in aneurysmal dilatation of bile ducts. Non-tumor IPMN-B (2 cases): no mass was found in the widely dilated bile ducts with smooth bile duct wall. Invasive IPMN-B (2 cases): tumors protruded into the dilated bile ducts causing jagged wall of bile duct, with accompanied abnormal density or signal intensity outside the bile ducts. Bile duct dilatations were shown in all 18 cases, and tumors were shown in 16 cases. In 2 cases no mass was displayed in widely dilated bile ducts. CT density of the tumor was lower than that of liver parenchyma, and higher than that of the bile and intraductal mucin. Signal intensity of the tumor was higher than that of stones, and lower than that of bile and intraductal mucin at MR T2WI. All tumors showed high intensity on DWI. Tumors showed mild to moderate enhancement after injection of contrast agent, CT density or signal intensity of the tumors were lower than that of the liver parenchyma during all three phases of contrast-enhanced CT or MRI. Conclusion IPMN-B has some specific CT and MR imaging features, which are helpful for the diagnosis and classification of IPMN-B. Key words: Bile duct neoplasms; Tomography, X-ray computed; Magnetic resonance imaging

GM2-Gangliosidosis, AB Variant: Clinical, Ophthalmological, MRI, and Molecular Findings.

GM2-gangliosidosis, AB variant is a very rare form of GM2 gangliosidosis due to a deficiency of GM2 activator protein, associated with autosomal recessive mutations in GM2A. Less than ten patients, confirmed by molecular analysis, have been described in the literature.A 12-month-old Hmong girl presented to the neurometabolic clinic for evaluation of global developmental delay, hypotonia, and cherry red spots. The parents were not known to be consanguineous. Her examination was remarkable for hypotonia with hyperreflexia and excessive startling. The head circumference was normal. An extensive neurometabolic evaluation was negative.Developmental regression began at 14 months of age. Retinal examination at 16 months of age disclosed 4+ cherry red/black spots with "heaped up" ring of whitish infiltrate surrounding both foveae but no evidence of optic atrophy or peripheral retinal abnormalities. Repeat magnetic resonance imaging (MRI) scan at 17 months of age revealed delayed but interval myelination associated with abnormal signal intensity of the bilateral thalami presenting as T2 hyperintensity of the posterior thalami in the region of the pulvinar nuclei and T2 hypointensity in the anterior thalami. Sequencing of the GM2A gene revealed a homozygous c.160 G>T mutation, predicted to result in a premature protein termination p. Glu54*.

Marchiafava-Bignami disease with asymmetric extracallosal lesions.

Marchiafava-Bignami disease (MBD) is a rare disease in patients with chronic alcoholism and is characterized by symmetric demyelination and necrosis of the corpus callosum. Clinical features include neuropsychiatric disorders, dysarthria, tetraparesis, astasia-abasia, impaired consciousness and symptoms of interhemispheric disconnection. The lesions in MBD are not restricted to the corpus callosum, but also occur in extracallosal regions. However, no case of MBD with extracallosal lesions confined to one side of the brain has been reported. We present a patient with MBD showing asymmetric extracallosal lesions and complete resolution of magnetic resonance imaging (MRI) abnormalities.

Mise au point sur le syndrome de Susac. À propos d’un cas pédiatrique

Susac's syndrome (SS) is a rare immune disorder. It combines encephalopathy, retinopathy and hearing loss. We report a new pediatric case, revealed by an occlusive retinal arteritis. A 12-year-old girl presented to the emergency room because of sudden onset of photopsias in her right temporal visual field. One year previously, she had developed an acute disseminated encephalomyelitis, resolving with steroids. Visual acuity was 10/10-P2 in both eyes. Fundus examination revealed a thin right inferior nasal artery. Fluorescein angiography revealed an occlusion of this artery and occlusive peripheral arterial vasculitis. An IV steroid bolus was administered urgently. Brain MRI showed high signal intensity abnormalities in the corpus callosum. The clinical triad was rapidly completed by a bilateral hearing loss. Intravenous immunoglobulins were initiated. The occurrence of contralateral occlusive retinal arteritis two months later led to treatment with cyclophosphamide. After 6infusions, visual acuity was stable, but the visual field remained, and regression of the vasculitis remained incomplete. Three hundred cases of SS have been reported with predominance in young women. Pediatric cases are rare. Diagnosis is difficult, because the typical clinical triad is often incomplete. Occurrence of relapses is unpredictable. Visual prognosis depends on the location of the occluded retinal territories. Treatment, based on a combination of steroids, immunosuppressive and antiplatelet agents is not always able to prevent relapse, as in our case. Unpredictable progression of SS requires early diagnosis and treatment as well as close monitoring. It must always be considered in the case of any occlusive retinal arteritis, even in children.

Hippocampal volume, early cognitive decline and gait variability: Which association?

BackgroundIn contrast to its prominent function in cognition, the involvement of the hippocampus in gait control is still a matter of debate. The present study aimed to examine the association of the hippocampal volume with mean values and coefficients of variation (CoV) of spatio-temporal gait parameters among cognitively healthy individuals (CHI) and patients with mild cognitive impairment (MCI). MethodsA total of 90 individuals (47 CHI with a mean age of 69.7±3.6years and 48.9% women, and 43 MCI individuals with a mean age of 70.2±3.7years and 62.8% women) were included in this cross-sectional study. The hippocampal volume was quantified from a three-dimensional T1-weighted MRI using semi-automated software. Mean values and CoV of stride time, swing time and stride width were measured at self-selected pace with a 10m electronic portable walkway (GAITRite®). Age, gender, body mass index, number of drugs daily taken, Mini-Mental State Examination (MMSE) score, history of falls, walking speed and white matter signal-intensity abnormality scoring with Manolio scale were used as covariates. ResultsPatients with MCI had a lower MMSE score (P<0.001), a higher CoV of stride time (P=0.013) and a lower hippocampal volume (P=0.007) compared with CHI. Multiple linear regression models showed that CoV of stride time was specifically associated with higher hippocampal volume among CHI (P<0.05) but not among patients with MCI (P>0.650). ConclusionsOur findings revealed a positive association between a greater (i.e., better morphological structure) hippocampal volume and a greater (i.e., worse performance) stride time variability among CHI, but not among MCI individuals.

The Clinical Outcomes of Anterior Cruciate Ligament Reconstruction Surgery Based on Iatrogenic Damage

Objectives:To examine the degenerative changes of adjacent cartilage and bony structure, after anterior cruciate ligament reconstruction surgery.Methods:27 subjects, who had anterior cruciate ligament reconstruction surgery due to various reasons at least 12 months ago, were admitted to study. MRI data of patients were acquired retrospectively. Pre-operative and post-operative MRI images were compared according to retropatellar cartilage thickness, evidence of any new osteophytes formation or any signal intensity change on bony elements. Paired statistical analysis was applied.Results:The MRI revelation showed that; there were 14 patients with decrement of retropatellar cartilage thickness, 9 patients with new osteophytes formation and 21 patients with abnormal signal intensity change on bony elements; which were statistically significant (P<0.001).Conclusion:These results provide basic information about the degenerative effect of anterior cruciate ligament reconstruction surgery, on adjacent cartilage and bony structure.

NI-67 * THE ROLE OF FLAIR VOLUME AS A PROGNOSTICATING FACTOR IN PATIENTS WITH GLIOBLASTOMA

BACKGROUND: Volumetric analysis on T1-weighted post-gadolinium (T1-PG) magnetic resonance imaging (MRI) sequence is central in managing patients with glioblastoma. It is unclear how the contrast enhancing tumor volume relates to abnormal findings in other MRI sequences such as fluid attenuated inversion recovery (FLAIR) and whether additional prognosticating information can be derived from these supplementary radiographic techniques. OBJECTIVES: To determine the relationship between volumetric measurements (T1-PG, FLAIR) and survival in patients with newly diagnosed GBM using rapid volumetric measurement (MacDonald ellipsoid method) for estimation of tumor volume. We will also compare the accuracy of rapid volumetric measurement to a manual tracing method for determination of tumor volume. METHODS: Tumor volumes of 30 patients (10 females, mean age 63 years) with diagnosis of glioblastoma were retrospectively measured using pre-operative MRI. Enhancing tumor volume on the T1-PG sequence was calculated using both a manual tracing method and the MacDonald ellipsoid method while volume of abnormal signal intensity on the FLAIR sequence was measured using the MacDonald ellipsoid method. The relationship between volumetric analyses and duration of survival were examined. RESULTS: Mean volume of enhancing tumors in the T1-PG sequence was 43.93 cm3 and 48.30 cm3 (manual and MacDonald ellipsoid method respectively), while mean volume of abnormal signal intensity in the FLAIR sequence was 147.60 cm3. There was moderate correlation between the T1-PG and FLAIR volumes (r= 0.65). Average duration of survival from surgery was 341 days. Neither tumor volume measurements correlated with duration of survival. CONCLUSIONS: Rapid measurement of tumor volume is a reasonable and accurate estimate of manually traced tumor volumes. Both T1-PG and FLAIR alone do not correlate with survival in this small group of patients.

Neurodegeneration With Brain Iron Accumulation Disorder Mimics Autism

This 15-year-old Thai girl who was diagnosed with autism by a child psychiatrist at 3 years of age. She was born to healthy, nonconsanguineous parents. Pregnancy, labor, and delivery were normal, and her neonatal period was uneventful. The patient had normal development until 3 months of age and had a global delay of 12 months compared with normal milestones. When she was one year old, she began to crawl. Her parents noticed her walking on the tips of her toes with ataxia and frequent falls. At 18 months, she spoke only a few words. When she entered the prekindergarten level, her teacher reported that she had a poor attention span, remarking that she could finish her work quickly but that the work was filled with mistakes. The child often played by herself and demonstrated repetitive and stereotypic behavior without imaginative play. Her speech development was slow. She began talking in broken sentences with poor pronunciation and comprehension. At age 3 years, a child psychiatrist diagnosed autism. Six months later, a pediatric neurologist diagnosed autism with spastic diplegia as spasticity increased in her lower extremities. Normal investigations included electroencephalography, auditory brainstem responses, and thyroid function tests. She received physical, occupational, and speech therapy and along with botulinum toxin injections for spasticity. Her first in a series of magnetic resonance imaging (MRI) examinations was performed 18 months after her presentation; it demonstrated abnormally low signal intensity in the globus pallidus, posterior limb of the internal capsule, substantia nigra, and pontine corticospinal tract (Fig 1). The findings were consistent with iron deposition in the brain. The diagnosis of neurodegeneration with brain iron accumulation (NBIA) was made in spite of a lack of the “eye-of-the-tiger” sign. At 5 years 10 months, her spasticity increased, and she lost her ability to walk. Two years later, she lost her sitting balance and developed dysphonia and dysphagia. At 10 years of age, she developed dystonic movements and tonic seizures. At 14 years, her condition FIGURE 1. The initial T2 brain magnetic resonance imaging demonstrated abnormal low signal intensity in globus pallidus and posterior limb of internal capsule.

Long-term clinical and radiological improvement of chronic acquired hepatocerebral degeneration after obliteration of portosystemic shunt: Report of a case

Neurological manifestations are common in patients with decompensated cirrhosis. The majority of these patients show hepatic encephalopathy or chronic acquired (non-Wilsonian) hepatocerebral degeneration (CAHD). They characteristically present with dysarthria, ataxia, involuntary movements, and altered mental status. Neuroradiological examination in patients with hepatic encephalopathy often shows abnormal signals in multiple regions of the brain, such as the pallidum, putamen, caudate nucleus, hemispheric white matter, and ventral midbrain. The pathogenesis of hepatic encephalopathy and CAHD is poorly understood and the response to conventional therapies is often poor.We report a male patient with cirrhosis of unknown cause, who developed slowly progressive cerebellar truncal and limb ataxia and slurred speech. Magnetic resonance imaging (MRI) showed focal T2 hyperintensity in bilateral dentate nuclei and middle cerebellar peduncles (MCPs). After treatment by obliteration of the portosystemic shunt, clinical manifestations and MRI abnormalities were dramatically improved. He was followed for six years until he died of uncontrollable bleeding due to hepatocellular carcinoma. At the last examination 9months before death, he showed no apparent aggravation of neurological symptoms, and no abnormal signal intensities in the MCPs and supratentorial compartment. The clinical course and changes of brain MRI findings of this case are extremely rare, suggesting that obliteration of the portosystemic shunt may be effective for CAHD over long term.