Disseminated Takayasu arteritis with neurovascular small and medium vessel involvement

@#Takayasu arteritis is a rare granulomatous vasculitis that is commonly classified as a large vessel disease. Small and medium vessel involvement are extremely rare, with only a few case reports describing neurovascular, ocular and cutaneous involvement. We describe a 21 year old Malaysian woman with pre-existing Takayasu arteritis confined to the large vessels, presenting one year later to our centre with status epilepticus. Extensive radiologic studies revealed diffuse encephalopathic changes and multifocal neurovascular involvement, with the vasculitis progressing to encompass the large, medium and small vessels. The patient was treated with high dose steroid therapy and discharged well with long term steroid and immunosuppressive therapy. Follow up MRI with vessel wall imaging sequence (VW-MR) and arterial spin labelling (ASL) perfusion imaging demonstrated intra and extracranial vessel wall inflammation, with generalized reduction in left sided cerebral blood flow. This case demonstrates that Takayasu arteritis is not exclusively a large vessel vasculitis, and that small and medium vessel involvement does not preclude its diagnosis.

Moyamoya disease in a young woman with intraand extracranial vessels involvement on vessel wall imaging

@#Moyamoya disease (MMD) was first described in 1957 as “bilateral hypoplasia of internal carotid arteries (ICAs)”.1 Aside from involving the intracranial arteries, MMD can also affect extracranial ICAs and external carotid arteries (ECAs).2-4 High resolution magnetic resonance (MR) vessel wall imaging (VWI) is increasingly being used to help with the diagnosis and characterization of the condition focusing mainly on intracranial vessels and extracranial ICAs.5-9 We present a case of a young woman with MMD, demonstrating vessel wall enhancement of nonstenotic maxillary branches of bilateral ECAs.

Tuberculous optochiasmatic arachnoiditis and optochiasmatic tuberculoma in Malaysia

@#Background & Objectives: Arachnoiditis which involves the optic chiasm and optic nervecan rarely occurs in the patients with tuberculous meningitis (TBM). The primary objective of this study was to determine the incidence, assess the clinical and neuroimaging findings, and associations, understand its pathogenesis of these patients, and determine its prognosis. Methods: The patients admitted with TBM in the neurology wards of two tertiary care hospitals from 2009 to 2017 in Kuala Lumpur, Malaysia were screened. The patients with OCA and optochiasmatic tuberculoma were included in this study. We assessed the clinical, cerebrospinal fluid (CSF), imaging findings of the study subjects and compared with other patients without OCA or optochiasmatic tuberculoma. Results: Eighty-eight patients with TBM were seen during the study period. Seven (8.0%) had OCA and one (1.1%) had optochiasmatic tuberculoma. Five out of seven (71.4%) patients with OCA were newly diagnosed cases of TBM. The other two (28.6%) had involvement while on treatment with antituberculous treatment (paradoxical manifestation). The mean age of the patients with OCA was 27.3 ± 11.7. All the OCA patients had leptomeningeal enhancement at other sites. All had hydrocephalus and cerebral infarcts on brain neuroimaging. Three (42.9%) patients had cerebral tuberculoma at sites other than suprasellar and optic chiasm areas. On univariate analysis, the presence of OCA and optochiasmatic tuberculoma was associated with raised CSF opening pressure (p=0.014), younger age (p=0.024), cerebral infarcts (p=0.018) and hydrocephalus (p= 0.046). There was no statistically significant association on logistic regression. Only one (14.3%) patient had visual impairment. Conclusion: OCA and optochiasmatic tuberculoma were seen in 9% of a cohort of Malaysian TBM patients. They were more likely to be younger, have raised CSF opening pressure, cerebral infarcts and hydrocephalus, suggesting the association with a more severe exudative disease.

Role of cytokines in the assessment of clinical outcome and neuroimaging findings in patients with tuberculous meningitis

Background: Tuberculous meningitis is a life-threatening manifestation resulting from infection by Mycobacterium tuberculosis, especially in the developing countries. The molecular aspects of pathogenesis of tuberculous meningitis remain poorly understood. We evaluated the correlation of cerebrospinal fluid (CSF) and serum cytokine levels with the clinical outcome of 15 HIV-negative patients with tuberculous meningitis. We also assessed the association of CSF and serum cytokines with neuroimaging of brain findings in the patients. Methods: The prospective longitudinal study was conducted at the University Malaya Medical Centre between 2012 and 2014. Neuroimaging of the brain was performed and the findings of leptomeningeal enhancement, hydrocephalus, tuberculoma, infarcts and vasculopathy were recorded. The CSF and serum specimens were analyzed for IL-1ß, IL-8, IL-10, IL-18, IP-10, IFN-γ, MCP-1, TGF-ß, VEGF, TNF- α, IL-18BPa and MMP-9. The clinical outcome was graded at 3 months based on Modified Rankin scale (mRS). Results: On admission and at one month of anti-tuberculosis treatment, the CSF levels of IL-8, IL-1β, IP-10, IFN-γ and VEGF were elevated in all of the patients. Serum IP-10, MCP-1, IL-1β and IL-8 levels were increased on admission and at one month of anti-tuberculosis treatment. There were statistically significant differences between good and poor outcome (mRS at 3 months) for CSF IFN-γ (p=0.033), CSF IL-10 (p=0.033) and serum VEGF (p=0.033) at one month of treatment. None of the patients showed any association between CSF and serum cytokines on admission and at one month of anti-tuberculosis treatment with neuro-radiological findings. Conclusion: The CSF cytokine levels were not related to TBM disease severity on admission, and changes on MRI/CT scans. CSF levels of IFN-γ and IL-10 at one month of anti-tuberculosis treatment were associated with clinical outcome at 3 months. CSF cytokine levels on admission were not associated with the clinical outcome.

Level 4 comprehensive epilepsy program in Malaysia, a resource-limited country

Background and Objective: There is a great challenge to establish a level 4 epilepsy care offeringcomplete evaluation for epilepsy surgery including invasive monitoring in a resource-limited country.This study aimed to report the setup of a level 4 comprehensive epilepsy program in Malaysia and theoutcome of epilepsy surgery over the past 4 years. Methods: This is a retrospective study analyzingcases with intractable epilepsy in a comprehensive epilepsy program in University Malaya MedicalCenter (UMMC), Kuala Lumpur, from January 2012 to August 2016. Results: A total of 92 caseshad comprehensive epilepsy evaluation from January 2012 till August 2016. The mean age was 35.57years old (range 15-59) and 54 (58.7%) were male. There were 17 cases having epilepsy surgeryafter stage-1 evaluation. Eleven cases had mesial temporal sclerosis and 81% achieved Engel classI surgical outcome. Six cases had lesionectomy and 60% had Engel class I outcome. A total of 16surgeries were performed after stage-2 evaluation, including invasive EEG monitoring in 9 cases.Among those with surgery performed more than 12 months from the time of data collection, 5/10(50%) achieved Engel I outcome, whereas 2 (20%) had worthwhile improvement (Engel class III)with 75% and 90% seizure reduction.Conclusion: Level 4 epilepsy care has an important role and is possible with joint multidisciplinaryeffort in a middle-income country like Malaysia despite resource limitation.

Spinal tuberculous disease is common in tuberculous meningitis

Background: Tuberculous disease of spine (spinal TB) is under-recognized in tuberculous (TB) meningitis.The objective of the study was to evaluate the frequency, clinical and neuroimaging changes, andoutcome in the patients with spinal TB. Methods: All the patients with spinal TB admitted in the twolargest tertiary hospitals in Kuala Lumpur from 2009 to 2017 were recruited, the clinical features weredocumented, the magnetic resonance imaging (MRI) of the spine was performed. Clinical outcome wasassessed with Modified Rankin scale (MRS). Results: Twenty two patients were recruited. This wasout of 70 TB meningitis patients (31.4%) seen over the same period. Eighteen (81.8%) patients hadconcomitant TB meningitis. The clinical features consisted of systemic symptoms with fever (63.6%),meningitis symptoms with altered sensorium (45.5%), myelopathy with paraparesis (36.4%). Thefindings on spinal MRI were discitis (36.4%), spinal meningeal enhancement (31.8%), spinal cordcompression (31.8%), psoas abscess (27.3%), osteomyelitis (22.7%), and cord oedema (22.7%). Allexcept two patients (90.9%) had involvement in psoas muscle, bone or leptomeningeal enhancement,features that can be used to differentiate from myelopathy that affect the parenchyma only, such asdemyelination. Unusual manifestations were syringomyelia and paradoxical manifestations seen in 3patients each. The outcome were overall poor, with 68% having MRS 3 or more.Conclusion: Spinal TB is common in TB meningitis. The outcome is overall poor. A heightenedawareness is crucial to enable early diagnosis and treatment.

Neuroimaging findings are sensitive and specific in diagnosis of tuberculous meningitis

Objective: The primary objective of this study was to describe the neuroimaging changes of tuberculous meningitis (TBM), and to determine the role of neuroimaging in the diagnosis of TBM. Methods: Between January 2009 and July 2015, we prospectively recruited TBM patients in two hospitals in Malaysia. Neuroimaging was performed and findings were recorded. The control consists of other types of meningo-encephalitis seen over the same period. Results: Fifty four TBM patients were recruited. Leptomeningeal enhancement was seen in 39 (72.2%) patients, commonly at prepontine cistern and interpeduncular fossa. Hydrocephalus was observed in 38 (70.4%) patients, 25 (46.3%) patients had moderate and severe hydrocephalus. Thirty four patients (63.0%) had cerebral infarction. Tuberculoma were seen in 29 (53.7%) patients; 27 (50.0%) patients had classical tuberculoma, 2 (3.7%) patients had “other” type of tuberculoma, 18 (33.3%) patients had ≥5 tuberculoma, and 11 (20.4%) patients had < 5 tuberculoma. Fifteen (37.2%) patients had vasculitis, 6 (11.1%) patients had vasospasm. Close to nine tenth (88.9%) of the patients had ≥1 classical neuroimaging features, 77.8% had ≥ 2 classical imaging features of TBM (basal enhancement, hydrocephalus, basal ganglia / thalamic infarct, classical tuberculoma, and vasculitis/vasospasm). Only 4% with other types of meningitis/encephalitis had ≥1 feature, and 1% had two or more classical TBM neuroimaging features. The sensitivity of the imaging features of the imaging features for diagnosis of TBM was 88.9% and the specificity was 95.6%. Conclusion: The classic imaging features of basal enhancement, hydrocephalus, basal ganglia/thalamic infarct, classic tuberculoma, and vasculitis are sensitive and specific to diagnosis of TBM.

Evaluation of solid breast lesions with power Doppler: value of penetrating vessels as a predictor of malignancy

<p><b>INTRODUCTION</b>This study aimed to evaluate the vascular pattern of solid breast lesions using power Doppler ultrasonography (PDUS) and assess whether the presence of intratumoural penetrating vessels can predict breast cancer malignancy.</p><p><b>METHODS</b>Greyscale ultrasonography (US) and PDUS were prospectively performed on 91 women in Malaysia with histopathologically proven breast lesions. The diagnostic accuracy of greyscale US, PDUS, and both greyscale US and PDUS was calculated and compared.</p><p><b>RESULTS</b>The 91 women had 102 breast lesions (55 benign, 47 malignant). Of the 47 malignant lesions, 36 demonstrated intratumoural penetrating vessels. The sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of greyscale US findings in diagnosing malignancy were 100.0%, 71.4%, 74.1% and 100.0%, respectively. The presence of calcification in the breast lesion and the margin, shape and posterior acoustic features of the lesion were significant parameters in predicting malignancy (p < 0.01). The sensitivity, specificity, PPV and NPV of the presence of intratumoural penetrating vessels in predicting malignancy were 76.5%, 80.0%, 76.5% and 80.0%, respectively. When both greyscale US and PDUS were used, there was a significant correlation in predicting malignancy (p < 0.05). The specificity and PPV values of the combined greyscale US and PDUS method (89.0% and 85.7%, respectively) were higher than those of greyscale US or PDUS alone.</p><p><b>CONCLUSION</b>Flow patterns revealed by PDUS can be useful for differentiating benign and malignant breast lesions. The visualisation of penetrating vessels in solid breast lesions can be used to complement greyscale US findings in predicting malignancy.</p>

Bilateral thalamic internal medullary lamina involvement in a case of dengue encephalitis

There are 50-100 million dengue infections each year, but dengue encephalitis is relatively uncommon. The aetiology of neuronal injury is proposed to be due to direct viral neurotropism or host immune response-mediated inflammation causing neuronal damage. We report a case of severe dengue encephalitis, presenting during the acute viraemic phase of the disease. This was associated with inflammation and haemorrhage of the internal medullary lamina of both thalami which, to our knowledge, has not yet been reported in other infections of the central nervous system.

Imaging findings of an isolated deep cerebral venous thrombosis in the absence of superficial sinus thrombosis

Thrombosis of the deep cerebral venous system in the absence of superficial sinus thrombosis is a very rare disease. The clinical and radiological findings can be diagnostically challenging due to the subtle appearances on computed tomography (CT) scan. Magnetic resonance imaging (MRI) examination is a preferred imaging modality to complement the CT findings for an accurate diagnosis of venous sinus thrombosis. We present a case of this unusual condition which present as unilateral thalamic lesion on CT scan and the role of contrast enhanced MRI with fast spoiled gradient echo (FSPGR) sequence and 3D reconstruction which led to the diagnosis of thrombosis in the deep cerebral venous system.

Mimics and diagnostic pitfalls of intracranial lesions in conventional MRI: Clues on advanced MRI

Objective: To delineate and differentiate between late subacute hemorrhage and intracranial lipomas in clinically available conventional and advanced MR sequences. Methods: Two cases of late subacute hemorrhage and two cases of intracranial lipoma were reviewed with CT scans and 3.0T scanner MRI. The sequences evaluated in MRI were T1-weighted (T1W) fast spin echo (FSE), T2-weighted (T2W) FSE, gradient echo T2*-weighted (GRE T2*W) images, diffusion weighted (DWI), apparent diffusion coefficient (ADC) and multivoxel spectroscopy. Results: Late subacute hemorrhage and intracranial lipoma have similar imaging features on T1W, T2W FSE with blooming artefact at the margins on GRE T2*W. However on GRE T2*W sequence, the central area of lipoma demonstrates low signal; while hemorrhage demonstrates high signal. In DWI, late subacute hemorrhage shows hyperintensity; while in lipoma there is loss of signal. Conclusion: Awareness of the potential pitfalls in standard sequence are important, as these entities appear to have similar T1W/ T2W characteristic with blooming artefact on T2*W. Knowing the distinctive central signal intensity pattern on GRE T2W* and DWI is therefore essential to differentiate between these lesions as there are differences to their clinical management.

Primary angiitis of the central nervous system with myelopathy as initial clinical presentation

Primary angiitis of the central nervous system (PACNS) is a rare vasculitis restricted to the central nervous system without systemic involvement. Delay in diagnosis and treatment is common due to its non-specific symptoms and lack of non-invasive diagnostic tests. Myelopathy can occur in PACNS, during the clinical course of the illness, with or without cerebral symptoms. We describe here a 51 year-old ethnic Chinese woman who presented initially with paraparesis without cerebral symptoms. The diagnosis of PACNS was eventually made from brain biopsy when she subsequently developed cerebral involvement. Despite aggressive treatment, the patient developed progressive neurological deterioration and died. This patient demonstrates the rare occurrence of myelopathy as the sole initial presentation of PACNS.

Leptomeningeal glioblastoma multiforme presenting as cauda equina syndrome

Glioblastoma multiforme (GBM) is the commonest primary cerebral malignancy consisting of 12- 20% of intracranial brain tumours.1 We report here a patient with GBM with very unusual marked and widespread leptomeningeal GBM.

Lymphocytic Mastitis Mimicking Breast Carcinoma, Radiology and Pathology Correlation: Review of Two Cases

Lymphocytic mastitis, or diabetic mastopathy, is an unusual finding in early-onset and long-standing diabetes. It can presents as a non-tender or tender palpable breast mass. Mammogram and ultrasound frequently demonstrate findings suspicious of malignancy, thus biopsy and histological confirmation is usually required. We reviewed two cases of lymphocytic mastitis with characteristics findings on mammogram, ultrasound, and histopathology. Diagnoses were confirmed with excision biopsy.

Evaluation of bowel distension and mural visualisation using neutral oral contrast agents for multidetector-row computed tomography

<p><b>INTRODUCTION</b>We compared the effectiveness of different types of non-commercial neutral oral contrast agents for bowel distension and mural visualisation in computed tomographic (CT) enterography.</p><p><b>METHODS</b>90 consecutive patients from a group of 108 were randomly assigned to receive water (n = 30), 3.8% milk (n = 30) or 0.1% gastrografin (n = 30) as oral contrast agent. The results were independently reviewed by two radiologists who were blinded to the contrast agents used. The degree of bowel distension was qualitatively scored on a four-point scale. The discrimination of bowel loops, mural visualisation and visualisation of mucosal folds were evaluated on a 'yes' or 'no' basis. Side effects of the various agents were also recorded.</p><p><b>RESULTS</b>3.8% milk was significantly superior to water for bowel distension (jejunum, ileum and terminal ileum), discrimination of bowel loops (jejunum and ileum), mural visualisation and visualisation of mucosal folds (ileum and terminal ileum). It was also significantly superior to 0.1% gastrografin for bowel distension, discrimination of bowel loops, mural visualisation and visualisation of mucosal folds (jejunum, ileum and terminal ileum). However, 10% of patients who received 3.8% milk reported immediate post-test diarrhoea. No side effects were documented for patients who received water and 0.1% gastrografin.</p><p><b>CONCLUSION</b>3.8% milk is an effective and superior neutral oral contrast agent for the assessment of the jejunum, ileum and terminal ileum in CT enterography. However, further studies are needed to explore other suitable oral contrast agents for CT enterography in lactose- or cow's milk-intolerant patients.</p>

Synchronous unilateral infiltrating ductal and lobular breast carcinoma

The incidence of synchronous bilateral infiltrating breast cancer has been reported to be 2%. However, synchronous unilateral infiltrating ductal carcinoma and infiltrating lobular carcinoma (ILC) are very rarely reported. We present a woman with palpable ILC who was later found to have synchronous well-circumscribed ductal carcinoma on further imaging. We also discuss the use of diagnostic approaches such as ultrasonography, mammography and histopathology. This case highlights the importance of careful assessment of concurrent lesions in the breast in the presence of an existing carcinoma.

Cerebral venous sinus thrombosis: Comparison of multidetector computed tomography venogram (MDCTV) and magnetic resonance venography (MRV) of various fi eld strengths

Objectives: To investigate the accuracy of multidetector computed tomography venography (MDCTV) and magnetic resonance venography (MRV) of differing fi eld strength in diagnosing cerebral venous sinus thrombosis (CVST). To assess the visualization rate of the cerebral veins and dural sinuses between these imaging modalities. Methods: A retrospective review of 27 patients with clinical suspicion of CVST who underwent both MDCTV and corresponding MRI/MRV brain scans at 0.35T, 1.5T or 3.0T fi eld-strength were performed. Results were compared with the defi nitive fi nal diagnosis. In the nonthrombotic group of patients, a descriptive study of the anatomical visualization of cerebral veins and dural sinuses was also undertaken. Results: Ten of the 27 patients had a fi nal diagnosis of CVST. The Neuroradiologists’ consensus reading identifi ed these 10 cases of CVST in both the MDCTV and MRV scans with a sensitivity rate of 100%. There was one false positive in MDCTV and three false-positives in the MRV group (1.5T) with positive predictive values of 90.9% and 76.9%, respectively. MDCTV and MRV demonstrated a specifi city of 94.1% and 82.4%, respectively in diagnosing CVST. No false negative images were seen demonstrating a negative predictive value of 100% in both modalities. There was no statistical difference in the anatomical visualization rate of the cerebral veins or the dural sinuses among the various strengths of MRI. However, statistically signifi cant (p<0.05) fewer visualised vessels in MRV compared to MDCTV, for the SSS, ISS, straight sinus, ICV and VOG. Conclusion: MDCTV is equal to MRV of various strength in its sensitivity for CVST diagnosis. MDCTV also provides better anatomical visualization of the dural sinuses and cerebral veins.

Late-onset Nipah virus encephalitis 11 years after the initial outbreak: A case report

Nipah virus infection is known to cause late-onset and relapsed encephalitis, in addition to an acute encephalitic illness. This is a report of a 35 years old woman, who had exposure to the Nipah virus infection during the 1999 Malaysian outbreak, was positive for Nipah IgG by immunofl uorescence, and had multiple small hyperintense lesions in brain MRI typically seen in acute Nipah encephalitis patients, indicating asymptomatic Nipah virus infection. She subsequently developed acute encephalitis after 11 years, manifesting as diplopia, internuclear opthalmoplegia and epileptic seizures with pleocytosis in cerebrospinal fl uid examination. She had another episode of relapsed encephalitis a year later, with seizures, memory impairment, chorea and new lesions in MRI brain. This patient is unusual in the long incubation of 11 years before manifesting with late-onset Nipah encephalitis.

Central nervous system intravascular lymphoma in a Malaysia

Intravascular lymphoma (IVL) is a rare subtype of extranodal diffuse large cell lymphoma, characterized by intravascular proliferation of B or T lymphocytes within small blood vessels; which may lead to occlusive symptoms, its neurological involvement has been said to be uncommon among Asians.1 We describe a Malaysian with central nervous system IVL, to demonstrate that IVL is an important differential diagnosis in diffuse brain pathology also among Asians.

Progressive multifocal leukoencephalopathy limited to the posterior fossa

Progressive multifocal leukoencephalopathy (PML) is a rapidly progressive demyelinating disease caused by the reactivation of JC papova virus usually in immunocompromised hosts.1 The disease is a chronic viral infection resulting in mortality within a year.2 The condition characterized by white matter changes in multiple locations of the brain is caused by destruction of the oligodendrogliocytes.2 We report a case of AIDS associated PML presenting with progressive cerebellar symptoms, with the unusual feature of imaging abnormalities limited to the posterior fossa.