scholarly journals Spongiform leukoencephalopathy: A unique case of biopsy confirmed leukoencephalopathy secondary to toxic, non-inflammatory exposure

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110429
Author(s):  
Kristen Zemina ◽  
Yolanda Piña ◽  
Patrick Malafronte ◽  
Niraja Suresh ◽  
Rebeca Hurst
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Clinical Presentation ◽  
Brain Biopsy ◽  
Disease Diagnosis ◽  
High Dose ◽  
White Matter Disease ◽  
Resonance Imaging ◽  
Chemotherapy Drugs

Toxin-induced leukoencephalopathy is a rare neurological condition that has been previously associated with intracranial radiation, chemotherapy, drugs of abuse, and environmental exposures. Herein, we present a patient with brain-biopsy proven toxin-induced leukoencephalopathy, likely secondary to multiple environmental offenders including insecticides and non-Food and Drug Administration approved anabolic steroids, opioids, and benzodiazepines. A 60-year-old man presented to our service as a direct transfer from an outside facility for evaluation of a rapidly progressive neuropsychiatric decline. Extensive workup with blood work, cerebrospinal fluid analysis, paraneoplastic panel, serial magnetic resonance imaging brain with and without contrast, and electroencephalograms were unrevealing. Magnetic resonance imaging brain showed diffuse confluent white matter disease, which was non-specific. The patient was treated with high-dose methylprednisolone and trials of intravenous immunoglobulin without any significant improvement. Finally, a brain biopsy was performed, and pathology confirmed a spongiform leukoencephalopathy, favoring a toxin-related etiology. The diagnosis of toxin-induced leukoencephalopathy should be considered in patients with steep neuropsychiatric decline and associated diffuse white matter disease. Diagnosis relies heavily on history of exposure, clinical presentation, imaging findings, and ultimately, histopathology from brain biopsy. The recognition of the clinical presentation is important to pursue the appropriate diagnostic workup and treatment.

Seizures and Enhancing Brain Lesions

2021 ◽  
pp. 219-221
Author(s):  
Josephe Archie Honorat ◽  
Andrew McKeon
Keyword(s):  
Magnetic Resonance Imaging ◽  
Mycophenolate Mofetil ◽  
White Matter ◽  
Magnetic Resonance ◽  
Brain Magnetic Resonance Imaging ◽  
Brain Biopsy ◽  
Resonance Imaging ◽  
History Of ◽  
High Doses ◽  
T2 Hyperintensity

A 51-year-old man sought care for a 4-month history of generalized seizures. The description of his seizures was consistent with generalized tonic-clonic seizures with focal onset. The patient had no history of head trauma or central nervous system infection and no family history of seizures. The patient reported having visual disturbances for 2 years before the seizures. His medical and surgical history was unremarkable. Brain magnetic resonance imaging showed left temporo-occipital, white matter, T2-signal intensity with gadolinium-enhancing lesions. Brain magnetic resonance imaging showed patchy gadolinium enhancement with T2 hyperintensity in the left parietotemporal and occipital lobes. Brain biopsy of the left temporal lobe showed white matter lesions with necrosis and chronic infiltration with macrophages and CD3-positive T lymphocytes and a predominant perivascular distribution. Focal, secondary vasculitis was present. There was no evidence of lymphoma. A repeated brain biopsy of the parietal lobe after another inflammatory relapse showed pathologic findings identical to the first biopsy. The patient was diagnosed with inflammatory encephalitis without additional defining features on biopsy. The patient received levetiracetam for seizure control, but the seizures remained refractory. He then was treated with high doses of intravenous methylprednisolone and then oral prednisone. Simultaneously, mycophenolate mofetil was initiated. The patient was monitored every 3 months with complete blood cell counts and liver function tests. Three months later, the prednisone dose was slowly tapered. During that process, the patient had no new seizures, and brain magnetic resonance imaging showed no active inflammation. After discontinuation of corticosteroids, the patient had a relapse with a generalized seizure, and brain magnetic resonance imaging showed new gadolinium-enhancing lesions. Prednisone was resumed, with near-remission. He then reinitiated mycophenolate mofetil and continued levetiracetam. With this regimen he remained clinically and radiologically stable, with only occasional visual phenomena that were possibly epileptic, although follow-up electroencephalography when he was symptomatic was normal. Encephalitis of unknown origin represents approximately one-third of cases. This proportion is decreasing over time with the development of novel diagnostic technologies, such as sequencing techniques to identify causative infectious agents and advances in neural autoantibody diagnostics.

Tumefactive demyelinating lesions: conventional and advanced magnetic resonance imaging

2005 ◽  
Vol 11 (2) ◽  
pp. 135-139 ◽  
Author(s):  
Christian Enzinger ◽  
Siegrid Strasser-Fuchs ◽  
Stefan Ropele ◽  
Peter Kapeller ◽  
Reinhold Kleinert ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Magnetization Transfer ◽  
Correct Diagnosis ◽  
Brain Biopsy ◽  
High Dose ◽  
Resonance Imaging ◽  
Neuronal Marker ◽  
Imaging Characteristics ◽  
Demyelinating Lesions

In rare instances, demyelinating disorders present with radiological features that mimic a brain tumour. This often leads to biopsy, which-apart from carrying significant morbidity-frequently turns out as nondiagnostic or dispensable. We therefore set out to assess the contribution of repeated conventional magnetic resonance imaging (MRI), 1H-MR spectroscopy and magnetization transfer imaging in establishing a correct diagnosis of tumefactive demyelinating lesions (TDLs). We studied two females and one male, who presented with TDLs that led to brain biopsy in two cases, for up to three years. TDLs were characterized by the following features: (a) delayed or absent response to high-dose steroids together with progressive lesion growth over several weeks; (b) late or sparse enhancement, ill-defined borders, signal inhomogeneity and considerable concomitant oedema; and (c) normalization of initial increases in lipid and lactate peaks within three to four weeks, followed by persistent, marked reductions of the neuronal marker NAA and MTR values around or below 30%. These imaging characteristics reflected the histological correlate of marked demyelination in the absence of significant inflammation. MRI techniques thus appear to have the potential to establish a correct diagnosis of this subtype of TDLs. Awareness of these possibilities might obviate the need for biopsy at least in some cases in future.

scholarly journals Acute Disseminated Encephalomyelitis Secondary to Herpes Virus Infection in a Child

2018 ◽  
Vol 37 (2) ◽  
pp. 191-193
Author(s):  
Zakaria Ouassou ◽  
Youssef Motiaa ◽  
Aziza Bentalha ◽  
Alea El Koraichi ◽  
Selma Ech-Chrif El Kettani
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Acute Disseminated Encephalomyelitis ◽  
High Dose ◽  
Resonance Imaging ◽  
Rare Form ◽  
Neurological Signs ◽  
Magnetic Resonance Imaging Mri ◽  
Post Infectious

The acute disseminated encephalomyelitis ADEM is a rare form of encephalitis. We report a case after herpes infection. The diagnosis was based on the existence of post-infectious and neurological signs and demyelinating reaching the white matter in the magnetic resonance imaging MRI. The outcome was favourable after treatment with high-dose steroids.  

White Matter Lesions Detected by Magnetic Resonance Imaging After Radiotherapy and High-Dose Chemotherapy in Children With Medulloblastoma or Primitive Neuroectodermal Tumor

2004 ◽  
Vol 22 (22) ◽  
pp. 4551-4560 ◽  
Author(s):  
Maryam Fouladi ◽  
Murali Chintagumpala ◽  
Fred H. Laningham ◽  
David Ashley ◽  
Stewart J. Kellie ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Primitive Neuroectodermal Tumor ◽  
White Matter Lesions ◽  
High Dose Chemotherapy ◽  
Neuroectodermal Tumor ◽  
High Dose ◽  
Resonance Imaging ◽  
Neurologic Deficits

Purpose White matter lesions (WMLs) have been described as a delayed effect of cranial irradiation in children with brain tumors, or a transient subacute effect characterized by an intralesional or perilesional reaction. We report the occurrence of subacute WMLs detected by magnetic resonance imaging (MRI) in children treated for medulloblastoma or primitive neuroectodermal tumor (PNET) and document the associated clinical, radiologic, and neurocognitive findings. Patients and Methods Among 134 patients with medulloblastoma or supratentorial PNET treated prospectively with risk-adjusted craniospinal irradiation and conformal boost to the tumor bed, followed by four high-dose chemotherapy (HDC) cycles with stem-cell rescue, 22 developed WMLs on T1-weighted imaging with and without contrast and/or T2-weighted imaging on MRI. Patients had ≥ 12 months of follow-up. Neurocognitive assessments included intelligence quotient (IQ) tests and tests of academic achievement. Results Twenty-two patients developed WMLs at a median of 7.8 months after starting therapy (range, 1.9 to 13.0 months). Lesions were predominantly in the pons (n = 8) and cerebellum (n = 6). Sixteen patients (73%) had WML resolution at a median of 6.2 months (range, 1.68 to 23.5 months) after onset; two patients developed necrosis and atrophy. Three developed persistent neurologic deficits. Cumulative incidence of WMLs at 1 year was 15% ± 3%. Patients with WMLs had a significant decline in estimated IQ (−2.5 per year; P = .03) and math (−4.5 per year; P = .003) scores. Conclusion WMLs in medulloblastoma or PNET patients treated with conformal radiotherapy and HDC are typically transient and asymptomatic, and may mimic early tumor recurrence. A minority of patients with WMLs develop permanent neurologic deficits and imaging changes. Overall, the presence of WMLs is associated with greater neurocognitive decline.

Magnetic Resonance Imaging Techniques in White Matter Disease

2009 ◽  
Vol 20 (6) ◽  
pp. 301-312 ◽  
Author(s):  
Sven Haller ◽  
Vitor Mendes Pereira ◽  
Francois Lazeyras ◽  
Maria Isabel Vargas ◽  
Karl-Olof Lövblad
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Imaging Techniques ◽  
White Matter Disease ◽  
Resonance Imaging ◽  
Magnetic Resonance Imaging Techniques
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