A rare case of central nervous system amyloidoma treated with fractionated radiotherapy

A 54-year-old female presented with multiple episodes of emesis, intractable headaches, worsening balance, and slowly progressive right facial weakness. Imaging demonstrated a 3-cm mass in the left internal capsule and corona radiata region with associated edema, mass effect, and midline shift concerning for high-grade glioma, lymphoma, or brain metastasis. Stereotactic biopsy of the mass was consistent with amyloid deposition. Systemic workup for amyloidosis was negative, and the mass was thought to represent a focal tumor-like deposit of amyloid, also referred to as “amyloidoma.” In the absence of systemic disease, therapy, which can include surgery or radiotherapy, can be directed at the local process. The location of the patient's lesion was not amenable to resection; therefore, she was treated with fractionated radiotherapy of 30.6 Gy at 1.8 Gy per fraction. Serial brain MRI demonstrated stability 18 months out from therapy. To the authors' knowledge, this is the first documented case of focal fractionated radiotherapy for CNS amyloidoma. The authors concluded that radiotherapy can prevent further progression of amyloidomas in anatomical locations that prohibit resection.

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Symptomatic Giant Virchow-Robin Spaces: A Rare Cause of Spastic Quadriparesis in 43-Year-old Ethiopian Patient: A Case Report

Ethiopian Journal of Health Sciences ◽

10.4314/ejhs.v30i5.24 ◽

2020 ◽

Vol 30 (5) ◽

Author(s):

Biniyam Ayele ◽

Guta Zenebe ◽

Abenet Mengesha ◽

Yegeta Teshale

Keyword(s):

Brain Mri ◽

Cranial Nerves ◽

Internal Capsule ◽

Mass Effect ◽

Neurological Complications ◽

Perivascular Spaces ◽

Anatomical Structures ◽

Visual Blurring ◽

Spastic Quadriparesis ◽

Progressive Weakness

BACKGROUND: Virchow-Robin Spaces (VRS) are perivascular spaces that surround small arteries and arterioles. These normal anatomical structures are thought to be involved in the drainage of interstitial fluid and also to play an immunomodulatory role by hosting macrophages. Rarely, it becomes giant and symptomatic resulting in mass effect on adjacent neuronal structures and ventricular system causing different neurological disorders.CASE PRESENTATION: We report a 43-year-old, Ethiopian woman who presented with progressive weakness of all her extremity over the period of seven years. She had associated speech difficulty, visual blurring and pseudo-bulbar affect. Neurologic examination revealed spastic quadriparesis with increased deep tendon reflexes and up going plantar bilaterally. She had horizontal nystagmus, dysarthria and reduced bilateral visual acuity, otherwise normal cognition and cranial nerves examination. Brain MRI showed T1 hypointense, T2 hyperintense and non-enhancing multiple cystic lesions of different size, mainly in bilateral basal ganglia area with mass effect on adjacent internal capsule and lateral ventricles. Considering her clinical presentation and typical radiological features, diagnosis of symptomatic dilated Virchow-Robin spaces was made, and the patient was treated symptomatically.CONCLUSION: Commonly, dilation of Virchow-Robin spaces are not symptomatic, but giant Virchow-Robin spaces, as in our patient may result in spastic quadriparesis, causing great disability on the patient. Thus, we recommend considering symptomatic Virchow- Robin spaces as a potential differential diagnosis of progressive quadriparesis, as early neurosurgical intervention may reduce the neurological complications, such as spastic quadriparesis.

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DYSPLASTIC WHITE MATTER LESIONS IN PATIENT WITH NEUROFIBROMATOSIS 1

Romanian Journal of Neurology ◽

10.37897/rjn.2015.2.5 ◽

2015 ◽

Vol 14 (2) ◽

pp. 90-92

Author(s):

P. Amaresh Reddy ◽

◽

Amit Agrawal ◽

V. Umamaheshwar Reddy ◽

P. Radharani ◽

...

Keyword(s):

White Matter ◽

Neuropsychological Functioning ◽

Brain Mri ◽

White Matter Lesions ◽

Internal Capsule ◽

Mass Effect ◽

Neurofibromatosis 1 ◽

Hyperintense Lesion ◽

Malignant Lesions

Dysplastic white matter lesions/unidentified bright objects /Foci of abnormal signal intensities (FASi’s) in brain MRI are the commonest intracranial abnormality with Neurofibromatosis 1 seen in approximately 70-75% of patients. They are usually multiple, small in size and are typically located in globus pallidus, brainstem, centrum semiovale, thalamus, internal capsule, corpus callosum, and cerebellum. Although clinically silent, patients can present with reduced attention span however neuropsychological functioning of these lesions depends upon the region involved. NF1 lesions should be kept as differential for any hyperintense lesion in basal ganglia and caution is advised not to confuse these lesions with malignant lesions like gliomas as biopsies from these lesions showed benign etiology. Parental counselling regarding the prognosis is very important to alleviate unnecessary apprehension. Interval follow-up is advised for large lesions causing mass effect, showing contrast enhancement or when lesions are located in optic pathway.

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LINC-06. OBSERVATION ONLY IN A PATIENT WITH SUSPECTED LOW GRADE GLIOMA. SHOULD NEUROSURGERY ALWAYS BE THE FIRST STEP IN LOW AND MIDDLE INCOME COUNTRIES?

Neuro-Oncology ◽

10.1093/neuonc/noaa222.441 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii379-iii379

Author(s):

Carlos Leal - Cavazos ◽

Jose Arenas-Ruiz ◽

Oscar Vidal-Gutierrez

Keyword(s):

Brain Mri ◽

Internal Capsule ◽

Pediatric Brain Tumor ◽

Clinical History ◽

Low Grade ◽

Large Tumor ◽

Middle Income ◽

Dismal Prognosis ◽

Close Observation ◽

The Right

Abstract BACKGROUND Low grade gliomas (LGGs) are the most frequent pediatric brain tumor and they comprise a variety of histologies. Complete surgery is curative but sometimes its location makes it difficult. Recent publications highlight the excellent long-term outcomes of patients with LGGs with complete and incomplete resected tumors. Current strategies are focused on reducing risks of treatment related sequelae. METHOD We describe a patient with a suspected LGG managed by close observation. We describe the case of a 6 year old female with 5 months history of focal onset seizures. During this time a brain MRI was requested and tumor was evidenced. After “tumor diagnosis” was made family visited a handful of private neurosurgeons with a uniformly dismal prognosis and high risk morbidity from procedures offered. When first seen at our Hospital, the clinical history seemed compatible with a LGG and seizures well controlled with antiepileptic drugs. Neurological examination was completely normal. MRI showed a large tumor (7x5x5 cm) hypointense on T1, hyperintense on T2, without contrast enhancement, involving the right temporal lobe white matter, insula, internal capsule, hipoccampus, thalamus and mesencephalus with middle cerebral artery encasement. Interval imaging was proposed and after 4.5 years since diagnosis the tumor has been stable and patient clinically excellent. CONCLUSION Overall survival in pediatric LGGs is excellent and risk of sequelae should always be part of multidisciplinary team considerations. In centers with significant neurosurgical morbidity, biopsy of large tumors that are compatible with LGG may not be required in selected cases.

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Low and high grade glioma segmentation in multispectral brain MRI data

Acta Universitatis Sapientiae Informatica ◽

10.2478/ausi-2018-0007 ◽

2018 ◽

Vol 10 (1) ◽

pp. 110-132 ◽

Cited By ~ 8

Author(s):

László Szilágyi ◽

David Iclănzan ◽

Zoltán Kapás ◽

Zsófia Szabó ◽

Ágnes Győrfi ◽

...

Keyword(s):

Early Stage ◽

Brain Mri ◽

High Grade Glioma ◽

Detection Algorithm ◽

Gabor Wavelet ◽

Low Grade ◽

Data Sets ◽

Morphological Operations ◽

High Grade ◽

Mass Screening System

Abstract Several hundreds of thousand humans are diagnosed with brain cancer every year, and the majority dies within the next two years. The chances of survival could be easiest improved by early diagnosis. This is why there is a strong need for reliable algorithms that can detect the presence of gliomas in their early stage. While an automatic tumor detection algorithm can support a mass screening system, the precise segmentation of the tumor can assist medical staff at therapy planning and patient monitoring. This paper presents a random forest based procedure trained to segment gliomas in multispectral volumetric MRI records. Beside the four observed features, the proposed solution uses 100 further features extracted via morphological operations and Gabor wavelet filtering. A neighborhood-based post-processing was designed to regularize and improve the output of the classifier. The proposed algorithm was trained and tested separately with the 54 low-grade and 220 high-grade tumor volumes of the MICCAI BRATS 2016 training database. For both data sets, the achieved accuracy is characterized by an overall mean Dice score > 83%, sensitivity > 85%, and specificity > 98%. The proposed method is likely to detect all gliomas larger than 10 mL.

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Posterior Fossa Cyst and Hydrocephalus

Pediatric Neurosurgery ◽

10.1093/med/9780190617073.003.0027 ◽

2019 ◽

pp. 239-245

Author(s):

Peter J. Morone ◽

John C. Wellons

Keyword(s):

Posterior Fossa ◽

Imaging Modality ◽

Brain Mri ◽

Mass Effect ◽

Dandy Walker Malformation ◽

Treatment Indications ◽

Posterior Fossa Cyst ◽

Walker Malformation ◽

4Th Ventricle ◽

Foramen Of Luschka

A Dandy-Walker malformation is an anatomical triad characterized by a large posterior fossa, agenesis of the cerebellar vermis, and dilation of the 4th ventricle. Up to 90% of patients will have hydrocephalus secondary to obstruction of fourth ventricular outflow through the foramen of Luschka and Magendie. The differential diagnosis of Dandy-Walker malformation includes Blake’s pouch cyst, a cystic structure originating within the roof of the 4th ventricle that herniates into the cisterna magna, and posterior fossa arachnoid cysts, which typically displace both the cerebellum and 4th ventricle anteriorly and demonstrate a propensity to enlarge. Brain MRI is the best diagnostic imaging modality to evaluate posterior fossa anatomy and cystic structures. Not all posterior fossa cysts require treatment. Indications for surgical intervention include enlargement, symptomatic mass effect and/or hydrocephalus.

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Short-lasting unilateral neuralgiform headache with autonomic symptoms associated with idiopathic hypertrophic pachymeningitis

Cephalalgia Reports ◽

10.1177/2515816318790546 ◽

2018 ◽

Vol 1 ◽

pp. 251581631879054

Author(s):

J Matthijs Biesbroek ◽

Dirk R Rutgers ◽

Sander van Gulik ◽

Catherina JM Frijns

Keyword(s):

Brain Mri ◽

Resonance Imaging ◽

Hypertrophic Pachymeningitis ◽

Trigeminal Autonomic Cephalalgia ◽

Autonomic Symptoms ◽

Meningeal Inflammation ◽

Magnetic Resonance Imaging Mri ◽

Multiple Episodes ◽

Rule Out

Short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) is a rare form of trigeminal autonomic cephalalgia. SUNA is frequently associated with a trigeminal neurovascular conflict and rarely occurs secondary to other intracranial pathology. We report a patient with SUNA that was associated with ipsilateral meningeal inflammation caused by idiopathic hypertrophic pachymeningitis (HP). During the 9-year follow-up, she experienced multiple episodes of SUNA, most of which occurred during exacerbations of the pachymeningitis. This is the third case of SUNA associated with HP reported in the literature. Based on this observation, we suggest that in patients presenting with SUNA, besides dedicated magnetic resonance imaging (MRI) of the trigeminal nerve, gadolinium-enhanced brain MRI should be performed to rule out HP.

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A Comparison Between the Sensitivities of 3-mm and 5-mm Thick Serial Brain MRI for Detecting Lesion Volume Changes in Patients with Multiple Sclerosis

Journal of Neuroimaging ◽

10.1111/jon199883144 ◽

1998 ◽

Vol 8 (3) ◽

pp. 144-147 ◽

Cited By ~ 5

Author(s):

Marco Rovaris ◽

Maria Assunta Rocca ◽

Ruggero Capra ◽

Francesca Prandini ◽

Vittorio Martinelli ◽

...

Keyword(s):

Multiple Sclerosis ◽

Brain Mri ◽

Lesion Volume ◽

Volume Changes ◽

Serial Brain

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PL2.2 Pembrolizumab (PEM) in recurrent high-grade glioma (HGG)patients with mismatch repair deficiency (dMMR): an observational study

Neuro-Oncology ◽

10.1093/neuonc/noz126.003 ◽

2019 ◽

Vol 21 (Supplement_3) ◽

pp. iii2-iii2

Author(s):

M Caccese ◽

M Simonelli ◽

M Fassan ◽

M Padovan ◽

P Persico ◽

...

Keyword(s):

Immune Checkpoint Inhibitor ◽

Brain Mri ◽

High Grade Glioma ◽

Median Number ◽

Anaplastic Gliomas ◽

Repair Deficiency ◽

Grade 3 ◽

Microsatellite Stability ◽

Ecog Ps

Abstract BACKGROUND Pem, an immune checkpoint inhibitor, demonstrated to be activein various neoplasms with MMRd. No data exists about its efficacy in MMRdglioma patients. MATERIAL AND METHODS MMRd HGG relapsed after receiving RT and CT weretreated with Pem. MMR status was analyzed by immunohistochemistry,including the MLH1, MSH2, MSH6, and PMS2 markers. MMR deficiency wasdefined as presence of a weak (wMMRd) or absent (aMMRd) signal atimmunohistochemistry for at least one MMR protein. Other inclusion criteriawere: ECOG PS 0–2, histologically confirmed gliomas, dexamethasone ≤4 mg.Pem was administrated at 200 mg every 3 weeks until progression disease orunacceptable toxicity. Tumor response was evaluated by brain MRI every 10 weeksaccording to the RANO criteria. OS and PFS were evaluated by Kaplan-Meiercurves. CTCAE v4.0 was used for toxicity. RESULTS among 167 glioma patients, we found 22 MMRd gliomas. 12 PTS were treated with Pem: 8 wMMRd and 4 aMMRd. According to Bethesda criteria, allPTS had microsatellite stability. Tumor histologies included 5 anaplasticastrocytoma, 1 anaplastic oligodendroglioma, 6 glioblastoma (GBM). MSH2deficiency was found in 6 cases, MSH6 deficiency in 9 cases, PMS2 and MLH1deficiency in 2 cases. Median number of prior line of chemotherapy was 1 (range 1–5). Stable disease (SD) was reported in 4 PTS (33%); 8 PTS showedprogressive disease (PD). PTS with anaplastic gliomas showed a statisticallysignificant association with SD (p=0.03, OR=3); all GBM PTS reported PD; status of MMRd (weak/absent), IDH (mutated/wild-type), MSH2 and MLH6(deficient/proficient) were not associated with SD. Median follow up was 14.7 ms. OS was 5.6 ms (95% CI 0.1–13.8), PFS 2.4 ms (95% CI 1.8–2.9). OS was 2.8 ms and 5.6 ms (p=0.9), PFS was 1.8 ms and 3.1 ms (p=0.5) in PTS with wMMRd and aMMRd. PTS reporting SD and PD had PFS of 7.4 ms (95% CI 4.6–10.2) and 1.8 ms (95% CI 0.2–3.4), p=0.002; OS was “not reached” and 2.8 ms in PTS having SD vs PD (p=0.04). Grade ≥3 adverse eventswere reported in 8% of PTS. CONCLUSION a subgroup of recurrent MMRd HGG might benefit from Pem,especially anaplastic gliomas. There was a trend for a longer PFS and OS in PTS with aMMRd. Analyses for identifying additional molecular predictive factors is ongoing.

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Delayed neurological deterioration due to progressive pneumocephalus

Romanian Neurosurgery ◽

10.1515/romneu-2015-0018 ◽

2015 ◽

Vol 22 (1) ◽

pp. 128-131

Author(s):

Amit Agrawal ◽

S. Satish Kumar ◽

Umamaheswara Reddy V.

Keyword(s):

Ct Scan ◽

Clinical Presentation ◽

Mass Effect ◽

Neurological Status ◽

Tension Pneumocephalus ◽

Delayed Neurological Deterioration ◽

Mass Lesions ◽

Multiple Episodes ◽

Significant Mass Effect ◽

Significant Mass

Abstract Pneumocephalus can develop immediately following head trauma or clinical presentation may be delayed for days. We report a case of 35 year male whose initial CT scan brain plain small specks of pneumocephalus in left para-sellar region. However the next day he was complaining of severe headache and had multiple episodes of vomiting. Repeat CT scan showed increase in the size of pneumocephalus including appearance of intraventricular air with mild cerebral edema. The patient recovered well with conservative management. The present case is a gentle reminder that in a subgroup of head injury patients, intracranial air can produce significant mass effect leading to tension pneumocephalus which can can behave like other intracranial mass lesions and causes worsening of the neurological status of these patients.

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