Computed Tomography and Magnetic Resonance Imaging in Adult-Onset Leukodystrophy

Schwankhaus, John D.; Patronas, Nicholas J.; Dorwart, Robert H.; Eldridge, Roswell; Schlesinger, Sandra; McFarland, Henry F.

doi:10.1001/archneur.1988.00520330094015

Cited by 38 publications

(20 citation statements)

References 7 publications

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“…9 In the initial kindred, CT findings comprise signs of leukodystrophy most prominent in the frontoparietal and cerebellar white matter. 12 MR imaging has revealed findings also in the occipital and temporal lobes in more advanced disease and white matter changes in the whole length of the corticospinal tract in the brain and the corpus callosum. 3,12 Other typical findings in this disease entity were sparing or less severe alteration of the immediate periventricular white matter and slight atrophy of the corpus callosum.…”

Section: Discussionmentioning

confidence: 99%

“…12 MR imaging has revealed findings also in the occipital and temporal lobes in more advanced disease and white matter changes in the whole length of the corticospinal tract in the brain and the corpus callosum. 3,12 Other typical findings in this disease entity were sparing or less severe alteration of the immediate periventricular white matter and slight atrophy of the corpus callosum. Relatively slight atrophy in the cerebrum and brain stem was also found.…”

Section: Discussionmentioning

confidence: 99%

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MR Imaging Characteristics and Neuropathology of the Spinal Cord in Adult-Onset Autosomal Dominant Leukodystrophy with Autonomic Symptoms

Sundblom¹,

Melberg²,

Kalimo³

et al. 2008

AJNR Am J Neuroradiol

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

MR Imaging Characteristics and Neuropathology of the Spinal Cord in Adult-Onset Autosomal Dominant Leukodystrophy with Autonomic Symptoms

Sundblom¹,

Melberg²,

Kalimo³

et al. 2008

AJNR Am J Neuroradiol

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“…MRI showed that the WM abnormality started in the frontal lobes and extended to the cerebellum. 64 The available limited pathology showed microscopic vacuolar degeneration in the WM, preservation of U fibers and cortex, lack of inflammation and reactive gliosis.…”

Section: Other Genetically Defined Leukodystrophiesmentioning

confidence: 97%

Familial Multiple Sclerosis and Other Inherited Disorders of the White Matter

Kálmán

Leist

2004

The Neurologist

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“…Pigmented orthochromatic leukodystrophy (POLD), another variant of adult onset leukodystrophy, was defined by the presence of pigmented macrophages, myelin and axonal loss. Patients, with adult onset leukodystrophies, have presented with variable behavioral, cognitive and motor changes (1,8,9,16,17,22,26,27). There is considerable overlap in the morphologic findings of the published cases of HDLS and POLD (17,18).…”

Section: Introductionmentioning

confidence: 99%

Adult Onset Leukodystrophy with Neuroaxonal Spheroids: Clinical, Neuroimaging and Neuropathologic Observations

Freeman

Hyman

Sims³

et al. 2008

Brain Pathology

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Pigmented orthochromatic leukodystrophy and hereditary diffuse leukoencephalopathy with spheroids are two adult onset leukodystrophies with neuroaxonal spheroids presenting with prominent neurobehavioral, cognitive and motor symptoms. These are familial or sporadic disorders characterized by cerebral white matter degeneration including myelin and axonal loss, gliosis, macrophages and axonal spheroids. We report clinical, neuroimaging and pathological correlations of four women ages 34-50 years with adult onset leukodystrophy. Their disease course ranged from 1.5-8 years. Three patients had progressive cognitive and behavioral changes; however, one had acute onset. Neuroimaging revealed white matter abnormalities characterized by symmetric, bilateral, T2 hyperintense and T1 hypointense Magnetic Resonance Imaging signal involving frontal lobe white matter in all patients. Extensive laboratory investigations were negative apart from abnormalities in some mitochondrial enzymes and immunologic parameters. Autopsies demonstrated severe leukodystrophy with myelin and axonal loss, axonal spheroids and macrophages with early and severe frontal white matter involvement. The extent and degree of changes outside the frontal lobe appeared to correlate with disease duration. The prominent neurobehavioral deficits and frontal white matter disease provide clinical-pathologic support for association pathways linking distributed neural circuits sub-serving cognition. These observations lend further support to the notion that white matter disease alone can account for dementia.

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Computed Tomography and Magnetic Resonance Imaging in Adult-Onset Leukodystrophy

Cited by 38 publications

References 7 publications

MR Imaging Characteristics and Neuropathology of the Spinal Cord in Adult-Onset Autosomal Dominant Leukodystrophy with Autonomic Symptoms

MR Imaging Characteristics and Neuropathology of the Spinal Cord in Adult-Onset Autosomal Dominant Leukodystrophy with Autonomic Symptoms

Familial Multiple Sclerosis and Other Inherited Disorders of the White Matter

Adult Onset Leukodystrophy with Neuroaxonal Spheroids: Clinical, Neuroimaging and Neuropathologic Observations

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