Linfoistiocitosi eritrofagocitica

2003 ◽  
Vol 16 (3) ◽  
pp. 365-372 ◽  
Author(s):  
F. Caranci ◽  
A. D'Amico ◽  
F. Briganti ◽  
R. Migliorati ◽  
C. De Fusco ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Bone Marrow ◽  
Nervous System ◽  
White Matter ◽  
White Matter Abnormalities ◽  
Diffuse White Matter ◽  
Multisystem Involvement ◽  
Identification And Characterization

Erithrophagocytic lymphohistiocytosis is a rare disease characterized by exaggerated histiocytic proliferation and activation12 and multisystem involvement including visceral organs, lymph nodes, bone marrow and central nervous system4. Magnetic Resonance (MR) examination was performed in 10 patients with previously diagnosed Erithrophagocytic lymphohistiocytosis. This study was aimed at assessing MR accuracy in the identification and characterization of central nervous system lesions. MR findings show a good correlation with areas of parenchymal and meningeal lymphohistiocytic infiltration5, demonstrating diffuse white matter abnormalities in the early stages and necrotic areas with parenchymal volume loss as terminal findings4. In addition, MR allows the follow-up after chemotherapy and bone marrow transplantation2,3. Although rare, EL should be differentiated from other pediatric patchy white matter abnormalities5.

Clinical and Genetic Spectrum of 104 Indian families with Central Nervous System White Matter Abnormalities

2021 ◽  
Author(s):  
Parneet Kaur ◽  
Michelle C. Rosario ◽  
Malavika Hebbar ◽  
Suvasini Sharma ◽  
Neethukrishna Kausthubham ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
White Matter ◽  
White Matter Abnormalities

Unilateral Neuroimaging Findings in Pediatric Craniofacial Scleroderma: Parry-Romberg Syndrome and En Coup de Sabre

2020 ◽  
Vol 35 (11) ◽  
pp. 753-762
Author(s):  
Ronald R. Seese ◽  
Daniel Glaser ◽  
Andre Furtado ◽  
Kavita Thakkar ◽  
Kathryn S. Torok
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
White Matter ◽  
Neurological Symptoms ◽  
Localized Scleroderma ◽  
Imaging Findings ◽  
History Of ◽  
Parry Romberg Syndrome ◽  
Clinical Associations

Objective: Parry-Romberg syndrome (PRS) and en coup de sabre (ECDS) are subtypes of craniofacial localized scleroderma. Systematic analyses of central nervous system imaging findings and their clinical associations in children are lacking. Here, we aim to characterize neuroimaging findings and associated neurological symptoms in these conditions. Methods: Neuroimaging and neurological symptoms of children evaluated at our institution with a diagnosis of PRS or ECDS were retrospectively reviewed. Laterality, location, stability, and number of lesion(s) were evaluated, as was the presence of susceptibility lesion(s) and contrast enhancement. History of seizures or headaches was noted. Results: From 2003 to 2019, 80 patients with PRS or ECDS were followed at our institution. Neuroimaging was completed in 73 and found to be abnormal in 25. In 12 (48%) of these 25 cases, headaches and/or seizures were present. In the vast majority of these cases (22/25, 88%), lesions were ipsilateral to skin findings. White matter was involved in 19 (76%) patients. MRI abnormalities preceded a rheumatological diagnosis in 7 (28%). Susceptibility lesions were noted in 11 (44%), and 8 (73%) of these patients endorsed a history of headaches. Most lesions were in the supratentorial compartment, did not enhance, and were stable at 1-year follow up imaging. Of those with progression, susceptibility findings were present at baseline. Conclusions: Neuroimaging findings in pediatric PRS and ECDS are often supratentorial, stable, unilateral, and ipsilateral to skin findings, and they can precede cutaneous findings.

Diffuse encephalitic toxoplasmosis in HIV

2021 ◽  
Vol 14 (3) ◽  
pp. e237456
Author(s):  
Jasmine Pearce ◽  
Ray Sheridan ◽  
Johnathan Shaw ◽  
Thomas Senior
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
White Matter ◽  
Positive Patient ◽  
Hiv Positive ◽  
Cerebral Toxoplasmosis ◽  
Mri Imaging ◽  
White Matter Changes ◽  
Diffuse White Matter ◽  
Laboratory Results

This case demonstrates an atypical radiological presentation of cerebral toxoplasmosis in a 62-year-old HIV-positive patient. The diagnosis is discussed alongside MRI imaging, laboratory results and treatment. Central nervous system toxoplasmosis is typically associated with ring enhancing lesions on neuroimaging with contrast, but the radiology for this patient shows diffuse white matter changes and ependymal enhancement, which would normally suggest an alternative diagnosis.

Extensive Neurocysticercosis with Diffuse Leukoencephalopathy

Neurographics ◽  
2021 ◽  
Vol 11 (1) ◽  
pp. 30-34
Author(s):  
F.B. Gala ◽  
S. Kulkarni ◽  
A. Bharati ◽  
M. Bodhanwala
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
White Matter ◽  
Inflammatory Response ◽  
Skeletal Muscles ◽  
Parasitic Infection ◽  
Cerebral Hemispheres ◽  
Diffuse White Matter

In Central nervous system (CNS), neurocysticercosis (NCC) is the most common parasitic infection that affects the CNS. Cysticidal drugs are used for its treatment, and they can precipitate an inflammatory response, which results in worsening of symptoms. We report 2 cases of extensive neurocysticercosis that show diffuse white matter edema that involves both cerebral hemispheres. Of the 2 patients, one showed disseminated cysticercosis that involved the tongue, scalp muscles, and skeletal muscles of the extremities.

scholarly journals A phosphatidylinositol-linked peanut agglutinin-binding glycoprotein in central nervous system myelin and on oligodendrocytes.

1988 ◽  
Vol 106 (4) ◽  
pp. 1273-1279 ◽  
Author(s):  
D D Mikol ◽  
K Stefansson
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
White Matter ◽  
Gray Matter ◽  
Biochemical Characterization ◽  
Developmental Expression ◽  
Peanut Agglutinin ◽  
Human Central Nervous System ◽  
Binding Glycoprotein

Here we report the isolation and initial biochemical characterization of a 120-kD peanut agglutinin-binding glycoprotein from the adult human central nervous system (CNS), which is anchored to membranes through a phosphatidylinositol linkage. Myelin incubated with phosphatidylinositol-specific phospholipase C released the protein as a soluble polypeptide of 105 kD, which was isolated with peanut agglutinin-agarose affinity chromatography. The protein was found to be highly glycosylated. The protein appears to be confined to the CNS, where its developmental expression is region specific and parallels myelination. It is in greater quantity in white matter than in gray matter and it is in isolated human CNS myelin. Furthermore, ovine oligodendrocytes in culture contain the protein on their surfaces and release it into the supernatant as a soluble 105-kD form. We call this protein the oligodendrocyte-myelin protein.

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