Decrease in cerebral and cerebellar gray matter in essential tremor: a voxel-based morphometric analysis under 3T MRI.

BACKGROUND AND PURPOSE: Though routine neuroimaging is usually normal in essential tremor (ET) there is clinical evidence of widespread involvement of central nervous system. This study aimed at determining morphological changes in brain of patients with ET using voxel-based morphometry (VBM) analysis and also compare the subtypes of ET. METHODS: Clinical and imaging data of 20 patients (5 women, 15 men; mean age 38.2 ± 16.5 years) of ET were collected prospectively. To determine areas of gray matter (GM) atrophy, T1W volumetric MRI data of patients were compared with 17 matched control subjects using VBM analysis with Statistical Parametric Mapping 5. RESULTS: Compared to controls, patients showed scattered areas of cerebral and cerebellar atrophy. Voxel-wise analysis of patients with and without head tremor showed widespread areas of GM atrophy in cerebral hemispheres of patients with head tremor. No difference was observed between the familial and nonfamilial subgroups of ET. Multiple regression analysis revealed relationship between GM atrophy and tremor severity score. CONCLUSIONS: Patients with ET showed widespread areas of atrophy in both cerebellum and cerebral GM, which supports the current concept of the progressive and diffuse nature of ET. Patients with additional head tremor may represent a distinct subgroup of ET.

Cognitive profile and structural findings in Wilson's disease: a neuropsychological and MRI-based study.

BACKGROUND: Systematic studies on neuropsychological profile in patients with Wilson's disease (WD) are far and few. AIM: To examine the profile of cognitive deficits and their magnetic resonance imaging (MRI) findings in patients with WD. PATIENTS AND METHODS: Twelve confirmed patients of WD (age at onset and evaluation, 13.7±11.2 and 21.7±5.3 years, respectively; M-F ratio, 7:5) on de-coppering therapy constituted the study sample. Battery of neuropsychological tests measuring mental speed, motor speed, sustained attention, focused attention, verbal category fluency, verbal working memory, response inhibition, planning, concept formation, set-shifting ability, verbal and visual learning and memory were administered. Phenotypic details and observations on MRI of brain carried out within six months of neuropsychological assessment were documented. RESULTS: Neuropsychological assessment elicited cognitive deficits in multiple domains in all but one patient, who had normal MRI. Percentage of patients in the deficit range in various domains included: motor speed: 73%; verbal working memory, sustained and focused attention: 50%; verbal learning: 42%; visuo-constructive ability, verbal memory, mental speed: 33%-34%; verbal fluency, set-shifting ability, visual memory, verbal memory: 25%-27%; and verbal recognition: 17%. MRI was normal in three patients, and revealed variable abnormalities in the remaining: cerebral atrophy in 3; brainstem atrophy in 2; signal changes in basal ganglia in 9; and brainstem signal changes in 5. None had subcortical white matter changes. Two patients with normal MRI showed cognitive deficits. CONCLUSION: This study provides insight into the complex cognitive and brain changes observed on MRI in WD. Use of advanced MRI techniques in a larger cohort may improve understanding regarding functional and structural brain changes observed in similar disorders.

Tuberculous meningitis presenting as isolated interhemispheric exudates.

The total number of tuberculosis cases in the world is increasing, and less common forms of tuberculous meningitis (TBM) with varying imaging manifestations are being encountered more often. We describe anterior interhemispheric variety of TBM, which has not been previously described to the best of our knowledge in the literature. Common imaging findings in these five patients include predominant involvement of the meninges in the anterior interhemispheric fissure with relatively little enhancement of the basal cisterns. Knowledge of uncommon radiological findings is vital in early diagnosis and treatment of this common disease.

Do MRI features distinguish Wilson's disease from other early onset extrapyramidal disorders? An analysis of 100 cases.

Magnetic resonance imaging (MRI) is frequently used in the evaluation of various extrapyramidal disorders. Among the plethora of MRI features in Wilson's disease (WD), only "face of the giant panda" sign has been recognized to distinguish WD from other early onset extrapyramidal disorders (EOEPD). To ascertain the value of various MRI features in differentiating neuropsychiatric form of WD from other EOEPD. This retrospective analysis included 100 patients (M:F = 56:44) of EOEPD (5-40 years), who had undergone MRI during Jan'03 to Nov'08. Their clinical features were recorded and the following MR sequences were analyzed: T1WI, T2WI, FLAIR. Fifty-six patients had WD (M:F = 28:30, age at onset: 14 +/- 6.8 years) and 44 had other EOEPD (M:F = 27:17, age at onset: 19 +/- 9.8 years) that included Huntington's disease--4, young-onset Parkinson's disease--7, mitochondrial disorders--2, Hallervorden-Spatz disease--8, non-Wilsonian hepatolenticular degeneration--2, toxic/metabolic disorder--1, and others--20. The duration of illness at the time of MRI was comparable (WD: 3.1 +/- 4.9 years; Other EOEPD: 2.8 +/- 2.4 years). MR signal characteristics varied in topography and severity in both the groups. All the patients of WD had signal abnormalities in MRI, as against 16/44 of the other EOEPD group. The following MR observations were noted exclusively in WD: "Face of giant panda" sign (14.3%), tectal plate hyperintensity (75%), central pontine myelinolysis (CPM)-like abnormalities (62.5%), and concurrent signal changes in basal ganglia, thalamus, and brainstem (55.3%). Besides "Face of giant panda" sign, hyperintensities in tectal-plate and central pons (CPM-like), and simultaneous involvement of basal ganglia, thalamus, and brainstem are virtually pathognomonic of WD.

Spectrum of epilepsy in Wilson's disease with electroencephalographic, MR imaging and pathological correlates.

BACKGROUND: Seizures are uncommon in Wilson's disease. OBJECTIVE: To analyze profile of seizures in WD and to correlate with EEG and MR imaging observations. SUBJECTS AND METHODS: 41/490 patients (8.3%) of WD were documented to have seizures. Autopsy observations were available in 3 cases. RESULTS: The age at onset of seizures was 12.8+/-5.7years. Seizure - preceded the onset of characteristic features of WD (19.5%); occurred concurrently (46.3%); or, followed de-coppering therapy (29.2%) and occurred as terminal event (4.8%). The types of seizures were: generalized tonic-clonic - 29, simple partial - 8, complex partial - 6, partial seizures with secondary generalization - 2 and periodic myoclonus - 1. Six patients had multiple seizure types and 4 had status epilepticus. EEG abnormalities were frequent (19/24) consisting of background slowing and epileptiform discharges. MRI (n=20) revealed varying degree of atrophy and signal changes involving basal ganglia, brainstem and white matter. The duration of follow-up was 8.1+/-9.2years. The outcome of seizure was: no recurrence - 68.3%, breakthrough seizures - 17.1%, poor control - 9.7% and no follow-up - 4.9%. Two of them succumbed following cluster attacks. Autopsy revealed cavitatory lesions in white matter in frontal, temporal and parietal areas with varying involvement of cortical ribbon. Patients with seizures had more often white matter changes than those without. It was also noted that patients whose seizures were not controlled had MRI suggestive of cavitation of white matter, though the reverse was not true. CONCLUSIONS: This is the largest series regarding epilepsy in WD. Seizures are not uncommon and could occur at any stage. Deafferentation of white matter tracts from cortex may contribute for seizure in WD.

Wilson's disease: (31)P and (1)H MR spectroscopy and clinical correlation.

INTRODUCTION: Proton ((1)H) magnetic resonance spectroscopy (MRS) changes are noted in Wilson's disease (WD). However, there are no studies regarding membrane phospholipid abnormality using (31)P MRS in these patients. We aimed to analyze the striatal spectroscopic abnormalities using (31)P and (1)H MRS in WD. METHODS: Forty patients of WD (treated, 29; untreated,11) and 30 controls underwent routine MR image sequences and in vivo 2-D (31)P and (1)H MRS of basal ganglia using an image-selected technique on a 1.5-T MRI scanner. Statistical analysis was done using Student's t test. RESULTS: The mean durations of illness and treatment were 6.2 ± 7.4 and 4.8 ± 5.9 years, respectively. MRI images were abnormal in all the patients. (1)H MRS revealed statistically significant reduction of N-acetyl aspartate (NAA)/choline (Cho) and NAA/creatine ratios in striatum ((1)H MRS) of treated patients compared to controls. The mean values of phosphomonoesters (PME) (p < 0.0001), phosphodiesters (PDE) (p < 0.0001), and total phosphorus (TPh) (p < 0.0001) were elevated in patients compared to controls. Statistically significant elevated levels of ratio of PME/PDE (p = 0.05) observed in the striatum were noted in treated patients as compared to controls in the (31)P MRS study. The duration of illness correlated well with increased PME/PDE [p < 0.001], PME/TPh [p < 0.05], and PDE/TPh [p < 0.05] and decreased NAA/Cho [p < 0.05] ratios. There was correlation of MRI score and reduced NAA/Cho ratio with disease severity. The PME/PDE ratio (right) was elevated in the treated group [p < 0.001] compared to untreated group. CONCLUSIONS: There is reduced breakdown and/or increased synthesis of membrane phospholipids and increased neuronal damage in basal ganglia in patients with WD.

Giant axonal neuropathy: a pictorial essay and review of literature.

Giant axonal neuropathy (GAN) is a neurodegenerative disorder classified within the hereditary motor and sensory neuropathies, affecting both the peripheral and central nervous systems. GAN typically presents in early childhood before the age of five years and progresses to death usually by early adulthood. Various imaging findings in giant axonal neuropathy have been described and documented in literature in the form multiple case reports. We here present a pictorial essay of all the major imaging GAN findings described in the literature. In addition, involvement of the dentate nucleus hitherto not described in the literature was noted in the present case.

Comparative Study Evaluating the In Vivo Characteristics of Ruptured and Unruptured Aneurysms Using serial Digital Subtraction Angiography.

Over the past decade preventive endovascular treatment is increasingly being considered for intracranial aneurysms irrespective of whether ruptured or unruptured. Few studies have dealt with in-vivo characteristics of intracranial aneurysms. We compare the angiographic morphology of ruptured and unruptured intracranial aneurysms using short interval serial DSA. 37 patients with intracranial aneurysms and who underwent at least two digital subtraction angiograms were included in the study. Based on the clinical presentation there were two subgroups of patients, Group A patients presenting acutely with Sub arachnoid haemorrhage (SAH) and Group B patients who had no clinical or imaging features suggestive of bleed. Clinical and serial angiographic data were correlated. Aneurysms in Group A (1.04 mm(3)) were significantly (p=0.010) smaller than in Group B (4.53 mm(3)). Aneurysms in group A showed increase in size and those in Group B showed a decrease in size (p=0.019). Hypertensive patients in both the groups showed a tendency for a decrease in the size of the aneurysms. Aneurysms having stasis at the time of initial angiogram had significantly reduced in size on follow up (p=0.013) at a faster rate (p=0.017). Presence of spasm in adjacent vessels was associated with increase in size of aneurysm on follow up in both Groups. There are significant differences between a ruptured aneurysm and an unruptured one. Ruptured aneurysms are small and show rapid increase in size. The presence of spasm increased the size of the aneurysm in the post rupture period and anti hypertensive medication and stasis were associated with decrease in size.

Intrathecal gadolinium-enhanced magnetic resonance cisternography in cerebrospinal fluid rhinorrhea: road ahead?

Accurate localization of cerebrospinal fluid (CSF) fistula in a patient with CSF rhinorrhea is challenging and often involves multiple imaging studies with associated expense and patient discomfort. Intrathecal contrast-enhanced computed tomography (CT) and unenhanced magnetic resonance (MR) cisternography using constructive interference in steady state (CISS-3D) sequences are currently being used in attempt to localize the leakage site but with varying degrees of success. Our purpose is to evaluate the utility of intrathecal gadolinium-enhanced MR cisternography (GdMRC) in patients with CSF rhinorrhea. Ten consecutive patients of CSF rhinorrhea (five spontaneous, four post-traumatic, and one post-operative) were evaluated with GdMRC. Nine of the patients underwent intrathecal contrast CT and CISS-3D examinations in addition. Each of studies was reviewed independently by three neuroradiologists blinded to results of other modalities. Surgery was planned after consensus and surgical correlation obtained in nine cases. The fistulous site was clearly demonstrated by using GdMRC in eight patients, CISS-3D in six, and intrathecal CT in three patients. The site of leakage was confirmed surgically in all the patients. One patient was found to be false negative both by intrathecal CT and GdMRC, and in one patient no fistulous site was demonstrated by any of the modalities and surgery was not offered. No adverse reaction was seen in any of the patients. GdMRC is a novel method of confirmation and localization of CSF fistula with potential for routine clinical application. Diagnosis and localization of fistulous site is better demonstrated due to its high-contrast resolution, absence of bony artifacts, and direct multi-planar imaging.

Sequential MRI changes in Wilson's disease with de-coppering therapy: a study of 50 patients.

Wilson's disease (WD) is clinically and radiologically a dynamic disorder. However, there is a paucity of studies involving sequential MRI changes in this disease with or without therapy This study looked at serial MRI changes and their clinical correlate in patients with WD The severity of MRI changes using 1.5 T MRI in 50 patients with WD was graded based on alteration in signal intensity of focal lesions and atrophy. Details of clinical manifestations, Schwab and England Activities of daily living (MSEADL) score, Neurological Symptom Score (NSS) and Chu staging were recorded. Clinical severity and disability scores were correlated with MRI scores using SPSS v10 The mean age at onset of illness and diagnosis was 12.8+/-5.6 years and 14.4+/-6.0 years, respectively. At the time of first MRI, patients had been treated for 49.0+/-77.3 months. At a follow-up of 24.2+/-12.2 months, clinically 36 patients had improved, 9 remained the same and 5 had worsened. Serial imaging revealed an improvement in MRI parameters in 35 patients, no significant changes in 10, worsening in 4 and an admixture of resolving and evolving changes in 1. The overall MRI score improved from 8.2+/-5.7 to 5.9+/-6.6. There was an improvement in measures of disability and impairment in all: Chu stage, 11.5+/-0.7 to 1.3+/-0.6; MSEADL score (%), 79.7+/-27.6 to 88.0+/-25.4; NSS, 10.6+/-11.2 to 8.0+/-11.6, with good clinico-radiological correlation. Patients with extensive changes, white-matter involvement and severe diffuse atrophy had a poor prognosis In conclusion, the majority of patients with WD showed variable improvement in clinical and MRI features when treated.

Quantitative magnetic resonance techniques in the evaluation of intracranial tuberculomas.

PURPOSE: To evaluate intracranial tuberculomas using quantitative magnetic resonance (MR) techniques such as T2 relaxometry, magnetization transfer (MT), and diffusion-weighted imaging (DWI). MATERIAL AND METHODS: Thirty-three patients with intracranial tuberculomas (histologically confirmed in 22) were evaluated using proton density/T2-weighted, T1-weighted (with and without MT), and echo-planar diffusion-weighted imaging sequences. T2 relaxation times, MT ratios (MTR), and apparent diffusion coefficient (ADC) values were calculated from the center of the lesion, the periphery, perilesional edema, and contralateral normal white matter. The mean and standard deviation values of each variable were calculated and correlated using Pearson's test (P = 0.05). RESULTS: The measured mean values of T2 relaxation time, MTR, and ADC in the center of lesions were 155.5 ms, 14.1, and 1.27 x 10(-3) mm(2)/s, respectively, compared to 117 ms, 23.72, and 0.74 x 10(-3) mm(2)/s in normal white matter, and a T2 relaxation time of 187.45 ms in normal gray matter. Significant inverse correlations were noted between T2 relaxation values and MTR (P<0.001) and between MTR and ADC (P = 0.046). Significant positive correlation was seen between T2 relaxation and ADC values (P = 0.03). CONCLUSION: Intracranial tuberculomas are characterized by relatively short T2 relaxation times (compared to normal gray matter), decreased MTR, and mostly no restriction of diffusion. A combination of these quantitative parameters could be of help in the noninvasive diagnosis of tuberculomas.

Middle meningeal arteriovenous fistula and its spontaneous closure. A case report and review of the literature.

SUMMARY: Middle meningeal artery pseudo-aneurysms and arteriovenous fistulas are usually post-traumatic, although occasional iatrogenic cases have been reported. The treatment has been obliteration of the fistula by surgical or endovascular means. Spontaneous closure of fistula is uncommon. We report a case of non-traumatic middle meningeal arteriovenous fistula in a patient with alcoholism, which resolved spontaneously without treatment.

CT Perfusion in Spinal Disease: an Adjunct Tool to Vertebral Body Biopsy.

Routine diagnostic techniques are inadequate for diagnosis of spinal diseases. The purpose of this study was to determine whether CT perfusion can differentiate inflammatory diseases like tuberculosis from neoplastic diseases of spine. Thirty-two patients with vertebral body lesions associated with paraspinal mass underwent CT guided bone biopsy and histopathological evaluation. CT perfusion was done in all patients before doing biopsy. Perfusion parameters like blood volume (BV), blood flow (BF) and time to peak (TTP) were calculated and correlated with histopathology. Statistical analysis was done using Mann-Whitney test. p value <0.05 was considered significant. Of 32 cases, 20 had tuberculous osteomyelitis and 12 neoplastic disease (seven metastasis, three plasmacytoma, one each lymphoma and chordoma). Mean rBF was [inflammatory lesions, 1.459 and neoplastic lesions, 18.080 (p<0.000). Mean rBV was (inflammatory disease, 2.8589 and neoplastic lesions, 12.2133 (p<0 .000)). Mean rTTP was [inflammatory pathology, 1.041 and neoplastic pathology, 0.703(p<0.079)]. This shows the deconvolution-based CTP technique's potential for noninvasive diagnosis of at least all inflammatory lesions affecting the spine that are associated with paraspinal mass. Validation of the use of deconvolution CTP parameters for differentiation of inflammatory from neoplastic pathology may permit this technique to be used as an adjunct tool when biopsy when routine imaging findings are inconclusive.

Spontaneous resolution of bilateral superior ophthalmic vein and cavernous sinus thrombosis. A case report.

Superior ophthalmic vein thrombosis is uncommon, and bilateral superior ophthalmic vein thrombosis is rarer still. The resolution of bilateral superior ophthalmic vein thrombosis takes a long time. The spontaneous resolution of bilateral superior ophthalmic vein thrombosis is not known and not reported in the literature so far. Here we present MRI of bilateral superior ophthalmic vein thrombosis and its spontaneous resolution.

Wilson's disease: cranial MRI observations and clinical correlation.

INTRODUCTION: Study of MRI changes may be useful in diagnosis, prognosis and better understanding of the pathophysiology of Wilson's disease (WD). We aimed to describe and correlate the MRI abnormalities of the brain with clinical features in WD. METHODS: MRI evaluation was carried out in 100 patients (57 males, 43 females; mean age 19.3+/-8.9 years) using standard protocols. All but 18 patients were on de-coppering agents. Their history, clinical manifestations and scores for severity of disease were noted. RESULTS: The mean duration of illness and treatment were 8.3+/-10.8 years and 7.5+/-7.1 years respectively. MRI of the brain was abnormal in all the 93 symptomatic patients. The most conspicuous observations were atrophy of the cerebrum (70%), brainstem (66%) and cerebellum (52%). Signal abnormalities were also noted: putamen (72%), caudate (61%), thalami (58%), midbrain (49%), pons (20%), cerebral white matter (25%), cortex (9%), medulla (12%) and cerebellum (10%). The characteristic T2-W globus pallidal hypointensity (34%), "Face of giant panda" sign (12%), T1-W striatal hyperintensity (6%), central pontine myelinosis (7%), and bright claustral sign (4%) were also detected. MRI changes correlated with disease severity scores (P<0.001) but did not correlate with the duration of illness. CONCLUSION: MRI changes were universal but diverse and involved almost all the structures of the brain in symptomatic patients. A fair correlation between MRI observations and various clinical features provides an explanation for the protean manifestations of the disease.

Multi-slice computed tomography appearance of abscess of cavum septum pellucidum: a case report and review of the literature.

Abscesses involving the cavum septum pellucidum are rare and, owing to their location, detection may be difficult with routine conventional computed tomography (CT). Only a few isolated cases have been reported and mostly in children. We report a case of abscess involving the cavum septum pellucidum in an adult and its appearance on multi-slice spiral CT.

Unilateral Moyamoya Phenomenon Due to MCA Occlusion in a Child Presenting with Intracerebral Hemorrhage.

SUMMARY: Spontaneous middle cerebral artery (MCA) occlusion leading to moyamoya phenomenon is different from classical moyamoya disease. Previous studies have reported such phenomena in adults with ischemic lesions, except for a solitary case in a child.We report a case of a ten-year old girl who presented with a deep intracerebral hematoma and a normal ipsilateral middle cerebral artery on initial evaluation by CT. Subsequently, on follow-up angiography, the ipsilateral MCA was occluded with evidence of unilateral basal lenticulostriate and transdural collateral supply causing a 'moyamoya' phenomenon. This report discusses a rare and interesting disease from the perspective of 'moyamoya' phenomenon and angiogenesis.

Prognostic factors in patients presenting with severe neurological forms of Wilson's disease.

BACKGROUND: Wilson's disease (WD), a metabolic disorder, is believed to be potentially reversible, even in its severe form. However, some patients do not respond to treatment. AIM: To analyse prognostic factors in severe WD. DESIGN: Retrospective audit. METHODS: A total of 140 patients were regularly followed from February 2002 to May 2004. Twenty-nine (18 males, 11 females) had severe disease, as defined by Modified Schwab and England Activities of Daily Living score (MSEADL) of < or=50% or Chu stage of 3. We analysed their clinical, laboratory and MRI features with respect to prognosis. RESULTS: For the severe form, mean age at symptom onset was 11.5 +/- 6.4 years, and at diagnosis, 13.3 +/- 7.0 years. Mean Neurological Symptom Score (NSS), Chu stage, and MSEADL were 26.5 +/- 8.2, 2.7 +/- 0.5 and 24.8 +/- 17.4, respectively. Twenty-one patients underwent MRI; 14 had repeat MRI. Following treatment, 14 (group A) had progressive worsening, including death in two, while 15 (group B) had sustained clinical improvement. Baseline demographic, clinical and laboratory features and MRI scores did not significantly differ between the two groups. However, diffuse white-matter abnormalities were more extensive in group A. Full-dose initial penicillamine therapy could have contributed to worsening in four patients. Drug compliance was poor in both groups but resumption of treatment did not benefit patients in group A. Serial MRI showed regression of lesions only among patients with clinical improvement. DISCUSSION: Severe WD remains a therapeutic challenge, with early diagnosis and treatment are essential. Specific MRI observations, a 'start low-go slow' regimen for penicillamine, and compliance may have prognostic significance. In absence of clinical predictors, genetic attributes need to be explored.