Abstract:Lymphomatoid granulomatosis (LG) is an angiocentric and angiodestructive lymphoproliferative disease that mainly involves the lung. Thirty percent of patients with LG have neurological symptoms. We report serial changes in MRI of a patient with LG of the brain. Postcontrast MRI demonstrated multiple punctate and linear areas that preceded hemorrhage, as indicated by hypointensity with surrounding hyperintensity on subsequent T2-weighted images. The diagnostic value of these lesions observed with contrast MR im… Show more
“…These results suggest that spinal LYG is rarer than brain LYG. Spinal LYG was found to be more prevalent in men than in women in this study (6.5:1.0) than in previous reports (systemic LYG; 2:1 13 and isolated CNS LYG; 2.3:1.0 2 ). Moreover, the lung lesion is the most common LYG lesion, although only 42.9% had lung lesions and 6 cases were isolated CNS LYG cases in our series.…”
Section: Discussioncontrasting
confidence: 76%
“…By further application of strict inclusion and exclusion criteria, 14 articles were included in our final analysis ( Figure 3 ). 1 , 2 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 In this study, 15 cases, including our 1 case, were investigated ( Table 1 ). Figure 3 Systematic literature search for spinal lymphomatoid granulomatosis according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.…”
Section: Systematic Review Of Resultsmentioning
confidence: 99%
“… 16 53/Male — NL NL + Multiple Punctate C — + — Ⅰ/NL NL IFNα-2b NL NL 12 Miura et al. 13 28/Male HP PS for HP Lung + Single NL C~T + — + Ⅰ/NL LB CY, ST 13 Alive 13 Collins et al. 7 50/Male — — Lung, S — Single Extraaxial C4-5 + — + NL/NL LB & BMB NL NL NL 14 Herderscheê et al.…”
Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus−associated systemic angiocentric and angiodestructive lymphoproliferative disorder. It commonly involves the lungs and can also affect the skin, liver, kidney, and central nervous system. It can rarely occur in the spine, however, the details are unclear. We performed a systematic review of published cases (including our 1 case) of spinal LYG.
We performed a systematic search of studies in English on spinal LYG, focusing on its clinical features, imaging, and treatments, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines on the PubMed database. We identified 14 patients from the literature. We also found 1 case of isolated cervical LYG (grade 3) who was treated with steroid and radiation therapy for the spinal lesion after pathologic diagnosis. We performed a pooled analysis of these 15 cases.
The mean age was 43.4 years, and 13 of the 15 patients were male. Brain lesions were present in 11 of 12 intramedullary spinal lesions, and only 1 was an isolated spinal LYG case. Regarding the diagnostic methods, 1 case was not described. Of the 14 cases described, 12 patients underwent biopsies (7 brain, 4 lung, and 1 spinal cord lesion) and 2 underwent surgical removal for an extramedullary lesion. In the overall prognosis from a mean follow-up period of 21.6 months, 4 patients died despite several treatments.
Spinal LYG, particularly isolated spinal LYG, is rare. Thus further accumulation of cases may be necessary to better understand its characteristics.
“…These results suggest that spinal LYG is rarer than brain LYG. Spinal LYG was found to be more prevalent in men than in women in this study (6.5:1.0) than in previous reports (systemic LYG; 2:1 13 and isolated CNS LYG; 2.3:1.0 2 ). Moreover, the lung lesion is the most common LYG lesion, although only 42.9% had lung lesions and 6 cases were isolated CNS LYG cases in our series.…”
Section: Discussioncontrasting
confidence: 76%
“…By further application of strict inclusion and exclusion criteria, 14 articles were included in our final analysis ( Figure 3 ). 1 , 2 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 In this study, 15 cases, including our 1 case, were investigated ( Table 1 ). Figure 3 Systematic literature search for spinal lymphomatoid granulomatosis according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.…”
Section: Systematic Review Of Resultsmentioning
confidence: 99%
“… 16 53/Male — NL NL + Multiple Punctate C — + — Ⅰ/NL NL IFNα-2b NL NL 12 Miura et al. 13 28/Male HP PS for HP Lung + Single NL C~T + — + Ⅰ/NL LB CY, ST 13 Alive 13 Collins et al. 7 50/Male — — Lung, S — Single Extraaxial C4-5 + — + NL/NL LB & BMB NL NL NL 14 Herderscheê et al.…”
Lymphomatoid granulomatosis (LYG) is a rare Epstein-Barr virus−associated systemic angiocentric and angiodestructive lymphoproliferative disorder. It commonly involves the lungs and can also affect the skin, liver, kidney, and central nervous system. It can rarely occur in the spine, however, the details are unclear. We performed a systematic review of published cases (including our 1 case) of spinal LYG.
We performed a systematic search of studies in English on spinal LYG, focusing on its clinical features, imaging, and treatments, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines on the PubMed database. We identified 14 patients from the literature. We also found 1 case of isolated cervical LYG (grade 3) who was treated with steroid and radiation therapy for the spinal lesion after pathologic diagnosis. We performed a pooled analysis of these 15 cases.
The mean age was 43.4 years, and 13 of the 15 patients were male. Brain lesions were present in 11 of 12 intramedullary spinal lesions, and only 1 was an isolated spinal LYG case. Regarding the diagnostic methods, 1 case was not described. Of the 14 cases described, 12 patients underwent biopsies (7 brain, 4 lung, and 1 spinal cord lesion) and 2 underwent surgical removal for an extramedullary lesion. In the overall prognosis from a mean follow-up period of 21.6 months, 4 patients died despite several treatments.
Spinal LYG, particularly isolated spinal LYG, is rare. Thus further accumulation of cases may be necessary to better understand its characteristics.
“…1,2 Neurological symptoms occur in 30% of the cases 3 and sometimes represent the only manifestation of the disease. 4 Several studies [5][6][7][8] reported progressive lesion volume on MRI in LG but only a few reports 9,10 described brain atrophy. There are no reported cases of NP test performance in patients with LG, save one report of borderline IQ.…”
The authors report neuropsychological (NP) and serial quantitative magnetic resonance imaging (MRI) findings of a 29-year-old woman with lymphomatoid granulomatosis (LG). Disease course was characterized by acute psychosis, tremor, fever, seizures, and progressive cognitive impairment. At the time of symptom onset, brain MRI revealed mild lesion volume and normal parenchymal volume. This was followed by dramatic progression of brain lesions and atrophy over 2 years, at which point the patient expired. Atrophy was most prominent in the mesial temporal lobes. NP testing revealed marked amnesia and mild impairments in other cognitive domains. To our knowledge, this is the first recorded case of LG in which bilateral temporal lobe atrophy is evident and accompanied by anterograde amnesia. We speculate that temporal lobe atrophy was influenced by the established susceptibility of this region in various neurological diseases.
“…70 Multifocal involvement on brain MR imaging is commonplace, with punctate or linear T2 hyperintensities within perivascular spaces with corresponding enhancement on T1 postcontrast images, particularly within frontal and parietal regions-a finding not exclusive to LG and seen in angiitis and neurosarcoidosis but distinguishable from CNS lymphoma, which most often presents as a solitary lesion. [70][71][72][73] …”
SUMMARY:Most dementias begin insidiously, developing slowly and generally occurring in the elderly age group. The so-called rapidly progressive dementias constitute a different, diverse collection of conditions, many of which are reversible or treatable. For this reason, accurate identification and assessment of acute and subacute forms of dementia are critical to effective treatment; neuroimaging aids greatly in narrowing the diagnosis of these conditions. This second installment of a 2-part review of rapidly progressive dementias examines the use of imaging in an assortment of other etiologies in the differential diagnosis, from prion disease and neoplastic-related conditions to rare metabolic and other conditions such as Wernicke encephalopathy. In these clinical conditions, MR imaging has the potential to narrow this broad differential diagnosis and, at times, can definitively aid in the diagnosis of certain conditions on the basis of typical imaging patterns.
ABBREVIATIONS:CJD ϭ Creutzfeldt-Jakob disease; LG ϭ lymphomatoid granulomatosis; SREAT ϭ steroid-responsive encephalopathy with autoimmune thyroid-
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