A Woman With Progressive Gait Difficulty and White Matter Abnormalities

2021 ◽  
pp. 171-173
Author(s):  
Adrian Budhram ◽  
Ralitza H. Gavrilova
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
White Matter ◽  
Magnetic Resonance ◽  
Urinary Urgency ◽  
Resonance Imaging ◽  
Spinal Cord Involvement ◽  
Sequence Variations ◽  
T2 Hyperintensity ◽  
The Right

A 45-year-old woman was assessed for slowly progressive gait difficulty. Eight years earlier, she first noticed dragging of her right foot and stumbling on uneven surfaces. She also described difficulty bending the right knee and intermittent spasms of the right leg. She reported bilateral, mild hand weakness when she was doing repetitive tasks such as using scissors. She reported increasing urinary urgency and frequency over the past 3 years. Magnetic resonance imaging of the spinal cord showed continuous, relatively symmetric, T2 hyperintensity of the dorsal columns and, to a lesser extent, the lateral columns, as well as medullary pyramids. Brain magnetic resonance imaging showed T2 hyperintensity in the corticospinal tracts bilaterally extending from the precentral gyrus through the corona radiata and again seen in the pons. Review of the magnetic resonance imaging pattern of white matter abnormalities led to specific clinical suspicion for leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation, an autosomal recessive condition due to DARS2 gene sequence variation. Brain magnetic resonance spectroscopy did not show lactate peaks. Genetic testing findings were abnormal and showed compound heterozygous DARS2 sequence variations. The presence of DARS2 sequence variations confirmed the diagnosis of leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation. The patient was counseled that gradual progression of her gait difficulties could be expected. The following year she reported increasing urinary urgency and several episodes of urge incontinence. She was referred to the urology clinic. Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation is a leukodystrophy that most commonly presents clinically with spasticity, dorsal column dysfunction, cerebellar ataxia, and sometimes mild cognitive decline. Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation classically has a childhood onset and therefore may not be included in the differential diagnosis of chronic progressive white matter disease in adults.

scholarly journals Multimodal Magnetic Resonance Imaging reveals alterations of sensorimotor circuits in restless legs syndrome

SLEEP ◽  
2019 ◽  
Vol 42 (12) ◽  
Author(s):  
Ambra Stefani ◽  
Thomas Mitterling ◽  
Anna Heidbreder ◽  
Ruth Steiger ◽  
Christian Kremser ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
White Matter ◽  
Magnetic Resonance ◽  
Gray Matter ◽  
Disease Duration ◽  
Gray Matter Volume ◽  
Resonance Imaging ◽  
Iron Storage ◽  
Matter Volume ◽  
The Right

Abstract Study Objectives Integrated information on brain microstructural integrity and iron storage and its impact on the morphometric profile is not available in restless legs syndrome (RLS). We applied multimodal magnetic resonance imaging (MRI) including diffusion tensor imaging, the transverse relaxation rate (R2*), a marker for iron storage, as well as gray and white matter volume measures to characterize RLS-related MRI signal distribution patterns and to analyze their associations with clinical parameters. Methods Eighty-seven patients with RLS (mean age 51, range 20–72 years; disease duration, mean 13 years, range 1–46 years, of those untreated n = 30) and 87 healthy control subjects, individually matched for age and gender, were investigated with multimodal 3T MRI. Results Volume of the white matter compartment adjacent to the post- and precentral cortex and fractional anisotropy (FA) of the frontopontine tract were both significantly reduced in RLS compared to healthy controls, and these alterations were associated with disease duration (r = 0.25, p = 0.025 and r = 0.23, p = 0.037, respectively). Corresponding gray matter volume increases of the right primary motor cortex in RLS (p < 0.001) were negatively correlated with the right FA signal of the frontopontine tract (r = −0.22; p < 0.05). Iron content evaluated with R2* was reduced in the putamen as well as in temporal and occipital compartments of the RLS cohort compared to the control group (p < 0.01). Conclusions Multimodal MRI identified progressing white matter decline of key somatosensory circuits that may underlie the perception of sensory leg discomfort. Increases of gray matter volume of the premotor cortex are likely to be a consequence of functional neuronal reorganization.

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.

Breakthrough Disease While on Multiple Sclerosis Immunomodulatory Therapy

2021 ◽  
pp. 55-56
Author(s):  
Jonathan L. Carter
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Spinal Cord ◽  
White Matter ◽  
Magnetic Resonance ◽  
Disease Activity ◽  
Brain Magnetic Resonance Imaging ◽  
White Matter Lesions ◽  
Dimethyl Fumarate ◽  
Resonance Imaging

A 36-year-old woman with a history of relapsing-remitting multiple sclerosis was evaluated for new multiple sclerosis symptoms accompanied by new, enhancing, white matter lesions on brain magnetic resonance imaging. Her multiple sclerosis presented with L’hermitte sign when she was 24 years old. She had onset of bilateral lower extremity and left upper extremity tingling at age 26 years. Magnetic resonance imaging and cerebrospinal fluid examination at the time were supportive of the diagnosis of multiple sclerosis, and disease-modifying therapy was recommended by her neurologist. She initiated therapy with dimethyl fumarate at age 30 years after several further relapses. Surveillance magnetic resonance imaging showed new gadolinium-enhancing lesions on brain magnetic resonance imaging on each of 3 consecutive yearly scans. Urine culture and sensitivity tests were performed to rule out occult urinary tract infection; results of this testing were negative. magnetic resonance imaging of the brain concurrently showed new enhancing white matter lesions. The patient was diagnosed with clinical and radiographic breakthrough disease activity while receiving therapy for multiple sclerosis. The patient was treated with 5 days of intravenous methylprednisolone for her relapse. After discussion with the patient, it was decided to transition therapy from dimethyl fumarate to ocrelizumab infusions for her breakthrough disease activity. This decision was further supported by the patient’s concerns that she might be entering an early progressive phase of the disease. In patients with spinal-predominant multiple sclerosis, or with symptoms potentially indicating new spinal cord involvement, it may be necessary to include spinal cord imaging to assess for new disease activity.

scholarly journals Leucoencephalopathy with brainstem and spinal cord involvement and high lactate: quantitative magnetic resonance imaging

Brain ◽  
2011 ◽  
Vol 134 (11) ◽  
pp. 3333-3341 ◽  
Author(s):  
Marianne E. Steenweg ◽  
Petra J. W. Pouwels ◽  
Nicole I. Wolf ◽  
Wessel N. van Wieringen ◽  
Frederik Barkhof ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
Magnetic Resonance ◽  
Resonance Imaging ◽  
Quantitative Magnetic Resonance Imaging ◽  
Spinal Cord Involvement ◽  
Quantitative Magnetic Resonance ◽  
High Lactate

scholarly journals Magnetic resonance imaging of the cervical spinal cord in multiple sclerosis at 7T

2015 ◽  
Vol 22 (3) ◽  
pp. 320-328 ◽  
Author(s):  
Adrienne N Dula ◽  
Siddharama Pawate ◽  
Richard D Dortch ◽  
Robert L Barry ◽  
Kristen M George-Durrett ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Sclerosis ◽  
Spinal Cord ◽  
White Matter ◽  
Magnetic Resonance ◽  
High Resolution ◽  
Healthy Volunteers ◽  
Cervical Spinal Cord ◽  
Resonance Imaging ◽  
Lower Field

Background: The clinical course of multiple sclerosis (MS) is mainly attributable to cervical and upper thoracic spinal cord dysfunction. High-resolution, 7T anatomical imaging of the cervical spinal cord is presented. Image contrast between gray/white matter and lesions surpasses conventional, clinical T1- and T2-weighted sequences at lower field strengths. Objective: To study the spinal cord of healthy controls and patients with MS using magnetic resonance imaging at 7T. Methods: Axial (C2–C5) T1- and T2*-weighted and sagittal T2*-/spin-density-weighted images were acquired at 7T in 13 healthy volunteers (age 22–40 years), and 15 clinically diagnosed MS patients (age 19–53 years, Extended Disability Status Scale, (EDSS) 0–3) in addition to clinical 3T scans. In healthy volunteers, a high-resolution multi-echo gradient echo scan was obtained over the same geometry at 3T. Evaluation included signal and contrast to noise ratios and lesion counts for healthy and patient volunteers, respectively. Results/conclusion: High-resolution images at 7T exceeded resolutions reported at lower field strengths. Gray and white matter were sharply demarcated and MS lesions were more readily visualized at 7T compared to clinical acquisitions, with lesions apparent at both fields. Nerve roots were clearly visualized. White matter lesion counts averaged 4.7 vs 3.1 (52% increase) per patient at 7T vs 3T, respectively ( p=0.05).

Microvascular decompression of a C-2 segmental-type vertebral artery producing trigeminal hypesthesia

2014 ◽  
Vol 121 (4) ◽  
pp. 919-923 ◽  
Author(s):  
Jonathan N. Sellin ◽  
Baraa Al-Hafez ◽  
Edward A. M. Duckworth
Keyword(s):  
Magnetic Resonance Imaging ◽  
Spinal Cord ◽  
Cervical Spine ◽  
Magnetic Resonance ◽  
Vertebral Artery ◽  
Microvascular Decompression ◽  
Complete Resolution ◽  
Resonance Imaging ◽  
Facial Numbness ◽  
The Right

The authors report a case of trigeminal hypesthesia caused by compression of the spinal cord by a C-2 segmental-type vertebral artery (VA) that was successfully treated with microvascular decompression. Aberrant intradural VA loops have been reported as causes of cervical myelopathy, some of which improved with microvascular decompression. A 52-year-old man presented with progressive complaints of headache, dizziness, left facial numbness, and left upper-extremity paresthesia that worsened when turning his head to the right. Magnetic resonance imaging of the cervical spine showed the left VA passing intradurally between the axis and atlas, foregoing the C-1 foramen transversarium, and impinging on the spinal cord. The patient underwent left C-1 and C-2 hemilaminectomies followed by microvascular decompression of an aberrant VA loop compressing the spinal cord. The patient subsequently reported complete resolution of symptoms.

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