Acute haemorrhagic leukoencephalitis (AHLE) – our experience and a short review

Pujari, Shripad; Kulkarni, Rahul; Ojha, Pawan; Gursahani, Roop; Nadgir, Dattatraya; Patil, Sharad D.; Soni, Girish; Bangar, Sachin S; Harshe, Amol; Mandolkar, Mahesh; Joshi, Amit; Kadam, Sagar; Goyal, Aditi

doi:10.1016/j.jneuroim.2021.577751

Cited by 8 publications

(7 citation statements)

References 28 publications

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“…As various case reports and systematic reviews have demonstrated the effectiveness of daily IVIG therapy in AHLE, 13,14 our patient was also administered IVIG therapy following the apparent failure of IVMP. Although no therapeutic trials have compared IVIG F I G U R E 2 Cerebral MRI after 1 year showing multiple altered intensity lesions hyper-intense on T2-weighted imaging in the periventricular region of white matter and showing multiple small areas of signal "blooming" on SWI sequence, suggestive of multiple hemosiderin deposits, suggesting radiological improvement when compared with pre-treatment images.…”

Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 94%

“…This conservative modality of treatment mainly comprises several immunomodulation therapies to address the ongoing intense inflammation in the background. Several case reports/series and other literature reviews support the use of high‐dose IVMP, IVIG, and PLEX as the mainstay treatment for AHLE 3,14 . Tenembaum et al 15 stated that an initial pulse dose of IVMP should be started along with antibiotics and acyclovir and continued for 3–5 days followed by oral prednisolone for 4–6 weeks.…”

Section: Discussionmentioning

confidence: 99%

“…Several case reports/ series and other literature reviews support the use of high-dose IVMP, IVIG, and PLEX as the mainstay treatment for AHLE. 3,14 Tenembaum et al 15 stated that an initial pulse dose of IVMP should be started along with antibiotics and acyclovir and continued for 3-5 days followed by oral prednisolone for 4-6 weeks. However, in our case, high-dose IVMP alongside acyclovir and antibiotics had no demonstrable effect upon the disease course as described in the case series by Pujari et al 14 In this case, whether IVMP was truly ineffective or the response was delayed remains unknown.…”

Section: Discussionmentioning

confidence: 99%

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Weston Hurst hemorrhagic leukoencephalitis: A novel association with mixed connective tissue disease: uncloaking the “unholy” etiology underneath

Ghosh

Chatterjee

Roy

et al. 2022

Clinical & Exp Neuroim

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Background: Weston Hurst syndrome (WHS), also known as acute hemorrhagic leukoencephalitis (AHLE), has not been previously associated with mixed connective tissue disease (MCTD), a rare rheumatologic disorder.Case presentation: We report a case of a 35-year-old female who presented with deep coma with features of previously undiagnosed MCTD and responded favorably to intravenous immunoglobulin (IVIG) therapy. On detailed historical enquiry, clinical examination, and investigation, AHLE was diagnosed. Furthermore, our patient showed significant improvement with IVIG administration, even though initial steroid therapy was unsuccessful.Conclusion: AHLE, an associated novel neurological manifestation of MCTD, can be responsive to IVIG, which makes this case reportable from the diagnostic as well as the therapeutic point of view.

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Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Weston Hurst hemorrhagic leukoencephalitis: A novel association with mixed connective tissue disease: uncloaking the “unholy” etiology underneath

Ghosh

Chatterjee

Roy

et al. 2022

Clinical & Exp Neuroim

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“…Immunotherapies can curtail inflammatory responses but may not have any effect on the irreparably damaged tissues once it is too late. [20]…”

Section: Discussionmentioning

confidence: 99%

Two-year follow-up of a young male with possible acute hemorrhagic leukoencephalitis: A case report

et al. 2022

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Rationale: Acute hemorrhagic leukoencephalitis (AHLE) is a rare but fetal fulminant demyelinating disease of unknown etiology. It is commonly regarded as a severe variant of acute demyelinating encephalomyelitis (ADEM). Its rapid clinical deterioration and high mortality appeal to clinicians to attach importance to early diagnosis. Immunosuppressive therapy is the main management to attenuate the autoimmune process, but with varied response and prognosis. Patient concerns: A young male presented with moderate fever, headache and seizures after extraction of impacted teeth, and then deteriorated rapidly to deep coma. Initial magnetic resonance imaging (MRI) revealed multiple plaque-like lesions in bilateral cerebra, right thalamus and pontobulbar region with massive edematous swelling and multifocal small hemorrhagic foci. Inflammatory parameters in the peripheral blood were only mild higher with a pleocytosis in CSF. Diagnosis: His clinical presentation, laboratory evaluation and radiological features were consistent with a suspected diagnosis of AHLE. Interventions: He underwent pulse corticosteroids initially but failed to respond to it. However, his consciousness improved obviously when he was treated with multiple courses of intravenous injection of immunoglobulin (IVIG) combined with mycophenolate mofetil (MMF). Outcomes: The patient regained consciousness gradually on day 180 and was in minimally conscious state (MCS) during the two-year follow-up. Lessons: AHLE presents distinctly from classical ADEM, and the situation may pose a diagnostic challenge. Clinicians should be vigilant in recognizing AHLE because of its rapid clinical deterioration and high mortality. We highlight the critical role of multimodal MRI, particularly susceptibility-weighted imaging (SWI) in the diagnosis of AHLE if cerebral biopsies are unavailable. Multiple courses of IVIG with MMF may be effective when early single pulse of corticosteroids fails. Individual who survives the initial insult may carry relatively good prognosis.

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Antiepileptic-drugs/immune-globulin/methylprednisolone

2022

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Acute haemorrhagic leukoencephalitis (AHLE) – our experience and a short review

Cited by 8 publications

References 28 publications

Weston Hurst hemorrhagic leukoencephalitis: A novel association with mixed connective tissue disease: uncloaking the “unholy” etiology underneath

Weston Hurst hemorrhagic leukoencephalitis: A novel association with mixed connective tissue disease: uncloaking the “unholy” etiology underneath

Two-year follow-up of a young male with possible acute hemorrhagic leukoencephalitis: A case report

Antiepileptic-drugs/immune-globulin/methylprednisolone

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