“Limbic encephalitis with acute onset and Hu antibodies treated with rituximab: Paraneoplastic or non-paraneoplastic disorder?”

Abstract A 57-year-old female presented at the emergency room with acute onset aphasia but unremarkable cCT (incl. angiography and perfusion). Suspecting an ischemic stroke, she received thrombolysis with quick recovery of aphasia. Afterwards, myoclonic jerks of the face and right arm occurred leading to anticonvulsant therapy. Follow-up cMRI surprisingly demonstrated swollen T2w-hyperintense and Gd-enhancing left limbic, temporal and frontal lobes. Suspected herpes-simplex-encephalitis was treated with aciclovir despite unremarkable CSF results (no pleocytosis, no BBB disruption, negative HSV-PCR) on day 2 and follow-up (day 5). Due to persisting cognitive deficits, autoimmune limbic encephalitis was suspected, and intravenous immunoglobulin therapy was added. Three weeks later, she experienced new neurological symptoms (weakness, blurred vision, vomiting, headache). Follow-up brain MRI demonstrated a massive increase of multifocal Gd-enhancing lesions. Partial resection revealed the diagnosis of an IDH-wildtype glioblastoma (GB). Next generation oncogene panel testing demonstrated a GOPC-ROS1 fusion which is rarely found in GB. Due to the gliomatosis-like infiltration of both hemispheres, radiotherapy was deemed to be too toxic. Instead, she received two cycles of lomustine in absence of a MGMT-promotor methylation. Two months later cMRI showed a symptomatic second multilocular progression. 2nd-line therapy with a ROS-inhibitor was rejected, whereupon she died five weeks later. Our case is in several aspects peculiar: It demonstrates that rare GB-mimics (i.e. HSV- and autoimmune limbic encephalitis) can only be ruled out in a fast manner by brain biopsy. Watchful waiting may neglect fast progression of GB leading to the inability to provide optimal treatment (i.e. radiotherapy). Thrombolysis is strictly contraindicated in primary brain tumors, but was unharmful in our case most probably to the early tumor stage without relevant neoangiogenesis. Rare genetic abnormalities like ROS1-fusions which are reported mostly in childhood glioblastoma may be present and serve as a therapeutic target also in adult GB.

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Paraneoplastic Limbic Encephalitis Resembling Acute Herpetic Encephalitis

Case Reports in Neurological Medicine ◽

10.1155/2013/608643 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4

Author(s):

Ioannis Markakis ◽

Athanasios Papathanasiou ◽

Ermioni Papageorgiou ◽

Kostantinos Siarkos ◽

Georgios Gkekas

Keyword(s):

Limbic Encephalitis ◽

Memory Loss ◽

Acute Onset ◽

Medial Temporal Lobes ◽

Neurological Improvement ◽

Paraneoplastic Limbic Encephalitis ◽

Csf Analysis ◽

Complete Tumour ◽

Herpetic Encephalitis ◽

Paratracheal Lymph Node

Introduction. Paraneoplastic limbic encephalitis (PLE) is a rare disorder that typically follows a chronic or subacute course of personality changes, memory loss, seizures, and hallucinations. Early diagnosis is difficult and characteristic symptoms can be mimicked by a variety of conditions. We present a case of PLE, initially presenting as acute herpetic encephalitis.Case Presentation. A 56-year-old male was admitted for evaluation of acute onset headache, fever, and confusion. On neurological examination he was confused with MMSE score of 15/30. CSF analysis revealed marked lymphocytic pleocytosis. A possible diagnosis of acute herpetic encephalitis was rendered and patient was treated with acyclovir. CSF PCR was negative. Cranial MRI revealed bilateral hyperintense lesions in medial temporal lobes with contrast enhancement. Despite treatment with acyclovir patient was deteriorated; thus, a paraneoplastic syndrome was suspected. Chest CT showed a right paratracheal lymph node mass, while a biopsy revealed neuroendocrine lung cancer. Auto antibodies to Hu were also detected. The patient was treated with steroids and chemotherapy. Six months later, he had complete tumour remission and marked neurological improvement.Discussion. PLE can rarely invade acutely, being indistinguishable from herpetic encephalitis. Inclusion of PLE in the differential diagnosis of acute encephalitis is of great clinical significance.

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GAD65-Positive Limbic Encephalitis and Large Temporal Arachnoid Cyst: A Diagnostic Conundrum

Neuropediatrics ◽

10.1055/s-0037-1602980 ◽

2017 ◽

Vol 48 (S 01) ◽

pp. S1-S45

Author(s):

M. Karenfort ◽

Krause Molle ◽

K. Müller ◽

T. Beez

Keyword(s):

Arachnoid Cyst ◽

Limbic Encephalitis

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Limbic encephalitis with voltage gated potassium channel antibodies in a child

Neuropediatrics ◽

10.1055/s-2008-1079504 ◽

2008 ◽

Vol 39 (01) ◽

Author(s):

E Haberlandt ◽

CG Bien ◽

A Reiter ◽

B Simma ◽

R Crazzolara ◽

...

Keyword(s):

Potassium Channel ◽

Limbic Encephalitis ◽

Voltage Gated

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Renal Infarction and Pulmonary Embolism in Patient with Myelodysplastic

Health Sciences ◽

10.15342/hs.1.182 ◽

2020 ◽

Vol 1 (1) ◽

Author(s):

Mounia Bendari ◽

Nouama Bouanani ◽

Mohamed Amine Khalfaoui ◽

Maryam Ahnach ◽

Aziza Laaraj ◽

...

Keyword(s):

Pulmonary Embolism ◽

Myeloproliferative Neoplasms ◽

Bone Biopsy ◽

Bone Marrow Aspiration ◽

Myeloproliferative Disorders ◽

Acute Onset ◽

International Prognostic Scoring System ◽

Renal Infarction ◽

The Right ◽

Enhanced Computed Tomography

The myelodysplastic syndrome-myeloproliferative neoplasms (MDS/MPNs) are defined by a group of heterogeneous hematological malignancies resulting from stem cell−driven clonal growth of pathological hematopoietic progenitors and ineffective hematopoiesis, they are characterized concomitant myelodysplastic and myeloproliferative signs. Myelodysplastic/myeloproliferative disorders have been considered to have a higher risk of thrombus formation.We report a rare case about a 64 years old Moroccan woman, experienced renal infarction (RI) associated with pulmonary embolism as a complication of a myelodysplastic/myeloproliferative disorder.The patient complained of acute-onset severe left flank pain, a contrast-enhanced computed tomography (CT) of the chest and abdomen revealed RI by a large wedge-shaped defect in the right kidney with pulmonary embolism.Biological exam showed deep anemia, the bone marrow aspiration found myelodysplasia.the bone biopsy showed signs of myeloproliferatif disease. The karyotype was normal, BCR-ABL, JAK2, CALR mutations were absents, and MPL mutation was positive. The International Prognostic Scoring System (IPSS-R) was 0, and the patient was included to the low risk group.Anticoagulation therapy was initiated with heparin to treat RI and pulmonary embolism. Three months later, pulmonary embolism had resolved without the appearance of additional peripheral infarction.This case emphasizes the need to consider myelodysplastic/myeloproliferative disorders as a cause of infraction renal and pulmonary embolism.

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