Pattern and Course of Neurodegeneration in Langerhans Cell Histiocytosis

How I treat Langerhans cell histiocytosis

“…Neurodegenerative CNS LCH is a syndrome of uncertain etiology characterized by relentless progression of central neurodegeneration that may occur even 10 years after presumed resolution of LCH lesions. 53 Patients may develop clinical symptoms of dysarthria, ataxia, dysmetria, and behavior changes. MRI demonstrates hyperintensity of dentate nucleus and white matter of cerebellum on FLAIR and T2-weighted images, or hyperintense lesions of basal ganglia on T1-weighted images.…”

Langerhans Cell Histiocytosis

“…• Patients who have had CNS involvement, or involvement of CNS-risk bones (sphenoid, orbital, ethmoid, or temporal), should undergo brain MRI every 1-2 years for 10 years from diagnosis to look for new CNS disease and evidence of neurodegeneration [47].…”

Acquired hepatocerebral degeneration

“…In addition, neuroferritinopathy sometimes results in either eye of the tiger sign or confluent cavitary lesions involving the putamen or globus pallius. [75,87] Basal ganglia calcification Common causes include parathyroid disease and Fahr syndrome T1 sequences more often show low or iso-, signal intensity lesions in the basal ganglia, but hyperintensities have been reported; lesions do not enhance T2 sequences usually show corresponding iso-or hypointense lesions CT universally shows high-attenuation lesions and is the preferred imaging modality [3,128] Hypoxic-ischemic injury Prominent T1-hyperintensities of the basal ganglia may be seen following hypoxic-ischemic injury, sometimes with gadolinium enhancement, but T2 lesions and restricted diffusion commonly co-occur Associated imaging abnormalities are commonly found outside the basal ganglia, e.g., cortical laminar necrosis In neonates with hypoxic ischemic injury, metabolic disorders such as perinatal hypoglycemia, bilirubin encephalopathy, nonketotic hyperglycinemia, and urea-cycle disorders also may cause basal ganglia hyperintensities on T1-weighted imaging CT may show low-attenuation basal ganglia lesions [16,44,149,162] Carbon monoxide poisoning Basal ganglia T1-hyperintensities are possible, but T1 sequences more often show low signal intensity lesions in the medial globus pallidus, which may enhance T2 sequences usually show corresponding hyperintense lesions and apparent diffusion coefficient maps show restricted diffusion CT may show low-attenuation basal ganglia lesions [81] Langerhans cell histiocytosis T1 pallidal hyperintensities are common and gadolinium enhancing lesions may be present T2 sequences may show low, iso-, or high signal intensity lesions co-morbid extra-axial (osseous and dura-based) lesions always accompany parenchymal changes and other brain sites such as the pineal, pituitary, white matter, dentate and brainstem are commonly involved CT may show lytic lesions in the skull or skull base [22,119,160] Neurofibromatosis type I Basal ganglia T1-iso-or hyperintensities are possible, but T2 hyperintensities are more common; some lesions may enhance Lesions may be bilateral but are less homogenous than AHD Other MRI lesions may co-occur including astrocytomas, optic gliomas, sphenoid dysplasia, and plexiform neurofibromas CT typically shows normal basal ganglia [92] Fucosidosis T1 sequences may show pallidal hyperintensities similar to AHD but T2 sequences usually show corresponding basal ganglia hypointensities T2 sequences may show prominent white matter abnormalities and thalamic lesions CT may show low-attenuation lesions within the globus pallidus [34, 104,120] Microhemorrhage of the basal ganglia Bilateral basal ganglia microhemorrhage may result from hypoxia, infections such as Japanese encephalitis, or microangiopathies such as hemolytic-uremic syndrome or AIDS-related microangiopathy T1 and T2 sequences show low, iso-, or high signal intensity lesions depending on the oxidation state o...…”