Rhomboencephalitis

Paraneoplastic neurologic syndromes are a set of rare neurological conditions with a wide variety of presentations, ranging from headache to gait imbalance. These conditions are often underreported and underdiagnosed. Paraneoplastic rhombencephalitis is a subtype that involves inflammation of the hindbrain. This case involves a 67-year-old female with metastatic small-cell lung cancer who acutely developed neurological symptoms with magnetic resonance imaging findings consistent with rhombencephalitis. Our case discusses the updated diagnostic criteria for paraneoplastic neurologic syndrome released in July 2021 compared with the prior criteria in 2004...

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No abstract text is available yet for this article.

Rhomboencephalitis-inflammation of the brainstem and cerebellum-has myriad clinical presentations including encephalopathy, cranial neuropathies, long tract signs and cerebellar dysfunction and is associated with significant morbidity and mortality. There are a variety of potential underlying causes that respond variably to treatment, including infections, parainfective syndromes, inflammatory disorders including autoimmune encephalitis and paraneoplastic syndromes. Here, we review its clinical presentation and outline a practical approach to its investigation, aiming to facilitate prompt diagnosis and confirmation of the underlying cause, to start appropriate management early and optimise the clinical outcome...

Importance: Recognizing the presenting and immunopathological features of Kelch-like protein-11 immunoglobulin G seropositive (KLHL11 IgG+) patients may aid in early diagnosis and management. Objective: To describe expanding neurologic phenotype, cancer associations, outcomes, and immunopathologic features of KLHL11 encephalitis. Design, Setting, and Participants: This retrospective tertiary care center study, conducted from October 15, 1998, to November 1, 2019, prospectively identified 31 KLHL11 IgG+ cases in the neuroimmunology laboratory...

Listeria monocytogenes is a gram-positive bacillus known to cause various human infections including central nervous system infections. The most common central nervous system manifestations are meningitis and rhomboencephalitis, however rare complications including hydrocephalus and intracerebral hemorrhage can occur and are associated with increased mortality and incidence of lasting neurologic sequelae. The mortality ranges from 17 % to greater than 30 % in patients with neurological involvement [[1], [2], [3]]...

In this case report we compare two patients presenting with similar symptoms of a brainstem syndrome including ataxia, dysarthria, and diplopia. Their MRIs showed hyperintense FLAIR signal changes with patchy areas of contrast enhancement within the brainstem particularly the pons and cerebellum. The broad differential diagnosis of this brainstem pathology included rhomboencephalitis, neurosarcoidosis, lymphoma, vasculitis, infection, and paraneoplastic or autoimmune process. Patient 1 had an extensive work up including CSF cytology, MRI brain spectroscopy, full body CT, cerebral angiogram, and ultimately brainstem biopsy...

Listeria monocytogenes is a gram-positive bacterium with a predilection to infect the central nervous system, often affecting immunocompromised or elderly patients. The most common manifestations are meningitis and rhomboencephalitis. We report two cases of Listeria meningitis complicated by acute hydrocephalus several days after presentation and we further review the literature of similar cases. We conclude that acute hydrocephalus is a significant, not often recognized, complication of Listeria meningitis, usually occurring several days from onset when coverage did not include anti-Listeria antimicrobials...

Rhomboencephalitis, at least in its acute phase, is often a severely disabling syndrome, and can be life threatening. A range of underlying conditions can lead to this clinical syndrome. Rapid diagnosis to initiate treatment early is key to a beneficial outcome. We report the case of a 22 year old Afro-Caribbean woman, who presented with a two -week history of walking difficulties, upper limb incoordination and slurred speech. Her brainstem function deteriorated at pace, and she developed hypersomnia. A broad diagnostic approach led to prophylactic treatment for the most common infectious causes...

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Acute disseminated encephalomyelitis (ADEM) is a disease characterized by inflammation and destruction of myelin. Acute hemorrhagic leukoencephalitis (AHLE) is a severe form of ADEM known for its particularly poor outcome. We present a case of a young Caucasian female who presented with drowsiness and slurred speech followed by rapid brainstem involvement resembling rhomboencephalitis. Despite multiple diagnostic tests and empiric therapy with immunosuppressants, immunoglobulins, and antimicrobials, she lost most brainstem reflexes within a few weeks and ultimately passed away...

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Brainstem involvement is a characteristic feature and accounts for the high mortality associated with listeriosis especially in immunosuppressed patients. We report two cases of rhombencephalitis infection by Listeria monocytogenes in a 65 and 63-year-old men with diabetes. They were referred to a neurologist due to fever and drowsiness after 3 weeks. The 65-year-old man had vertigo, diplopia, ataxia, bidirectional nystagmus and the 63-year-old man complained of perioral numbness, dysphagia and dysartheria...

We describe a case of an 87-year-old woman with rheumatoid arthritis on etanercept who developed subacute encephalopathy. Magnetic resonance imaging and blood cultures led to the diagnosis of Listeria monocytogenes rhomboencephalitis, which proved to be fatal. Postmortem examination of the brain revealed abscesses with extensive necrosis. Gram stain showed small gram-positive rods in the necrotic tissue and the wall lining the abscesses. While rhomboencephalitis is a rare entity, and clinical recognition may be hampered by immunosuppression such as in this case, early blood cultures, lumbar puncture, and empiric treatment with ampicillin should be initiated in all patients with suspected rhomboencephalitis if suggested by imaging and by a clinical concern for infection...

Opsoclonus-myoclonus syndrome (OMS) is characterized by opsoclonus and arrhythmic-action myoclonus that predominantly involves the trunk, limbs, and head. Human herpes virus-6 (HHV-6) can rarely cause encephalitis in immunocompetent children and adults. Here we report on a case of OMS associated with HHV-6 rhomboencephalitis. HHV-6 infection should be considered in OMS adults and detection of cell-free viral DNA, indicative of active infection, is mandatory in such cases.

BACKGROUND: Listeria monocytogenes infections have been poorly characterized in Chile. AIM: To evaluate clinical manifestations and risk factors associated to a fatal outcome in a series of patients. METHODS: retrospective analysis of cases from 1991 to 2012. RESULTS: Twenty three cases were identified, including 2 diagnosed after prolonged hospitalization (8.7%) with an average age of 68.4 years (range 44-90). Known predisposing factors were age > 65 years (60...

Here we originally describe the clinical scenario of a young immune-competent patient affected by acute rhomboencephalitis with severe parenchymal edema and acute hydrocephalus who developed sudden life-threatening cardiac derangement. Hemodynamic and perfusion parameters revealed cardiogenic shock, so intensive circulatory support with epinephrine infusion and intra-aortic balloon pump was needed to restore organ perfusion. Transesophageal echocardiographic examination showed severe left ventricular dysfunction (ejection fraction as low as 20%) with wall motion abnormalities resembling a pattern of Takotsubo-inverted cardiomyopathy...

We report a case of rhombencephalitis infection by Listeria monocytogenes in a 66-year-old man with cirrhosis. The CSF analysis indicated L. monocytogenes as the most likely pathogen. Blood and CSF culture were positive to L. monocytogenes and MRI findings were suggestive of rhomboencephalitis. The treatment was started empirically and then modified when the culture results were available. The patient had a full clinical recovery without neurologic sequelae. Clinicians should remember that L. monocytogenes most often presents as acute bacterial meningitis, particularly in the elderly, the immunosuppressed, and those with malignancies...

Listerial rhomboencephalitis is a rare, devastating treatable disease. A previously well 72-year-old man presented with a one-week history of fever and headache. Four days before admission he developed right facial numbness and ataxia. Diagnosis and treatment are described, including the relevant clinical, laboratory and radiological findings.

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