Propionibacterium acnes-associated chronic hypertrophic pachymeningitis followed by refractory otitis media: a case report.

BACKGROUND: Hypertrophic pachymeningitis (HP) is a rare disorder that involves localized or diffuse thickening of the dura mater. HP is associated with various inflammatory, infectious, and malignant diseases, such as rheumatic arthritis, sarcoidosis, anti-neutrophil cytoplasmic antibody-associated vasculitis, IgG4-related disorders, syphilis, tuberculosis, bacterial and fungal infections, cancer, and idiopathic diseases, when evaluation fails to reveal a cause. Among them, chronic infection with Propionibacterium acnes is a rare etiology of HP, and its pathology remains unclear. CASE PRESENTATION: An 80-year-old man having refractory otitis media with effusion of the right ear presented with progressive right-sided headache and nausea. Post-contrast brain magnetic resonance imaging revealed right mastoiditis and remarkable thickening of the dura mater and enhancement of pia mater extending from the right middle cranial fossa to the temporal lobe. HP secondary to middle ear infection was suspected, and a biopsy of the right mastoid was performed. An anaerobic culture of the biopsied right mastoid showed the growth of P. acnes, and histopathological examination using P. acnes-specific monoclonal antibody (PAB antibody) revealed the infiltration of inflammatory cells with P. acnes. Moreover, using PAB antibody, P. acnes was detected in the biopsy specimen of the thickening dura mater. No granulomas were identified in either specimen. HP was resolved with long-term administration of antibiotics and steroids. CONCLUSION: This is the first documentation of pathologically demonstrated chronic HP associated with P. acnes infection followed by refractory otitis media. This report showed that chronic latent P. acnes infection induces chronic inflammation.

Left Atrial Spinning Ball Thrombus in a Patient with Cardioembolic Stroke: A Case Report.

A left atrial movable ball thrombus is unusual and may cause fatal systemic emboli or left ventricular inflow obstruction. Among movable ball thrombi, a spinning ball thrombus is a rare and devastating occurrence that results in cardioembolic stroke. Here, we report the case of an 88-year-old woman with a large spherical thrombus spinning in the left atrium when a catastrophic cardioembolic stroke recurred. She had a history of atrial fibrillation but was unable to continue anticoagulation therapy due to hemorrhagic complications and developed an initial cerebral embolism with a large thrombus attached to the left atrium. Twelve days after the initiation of anticoagulation therapy, an extensive cerebral embolism throughout the bilateral frontal lobe recurred with disturbance of consciousness. Transthoracic echocardiography revealed a large detached spherical thrombus spinning in the left atrium. She did not recover consciousness and was moved to another hospital for palliative care three months later. Movable type left atrial thrombi are regarded as a high risk for thromboembolic events, but those with spinning movements may have a worse prognosis.

Paradoxical Cerebral Embolism as Initial Manifestation of Chronic Thromboembolic Pulmonary Hypertension: A Case Report.

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by chronic thrombi in the pulmonary arteries, causing pulmonary hypertension and right heart failure. Early and accurate diagnosis are essential for successful treatment but are often difficult because clinical signs and symptoms can be nonspecific and risk factors, such as history of venous thromboembolism, may not always be present. Here, we report a case involving a 76-year-old woman who demonstrated paradoxical cerebral embolism as the initial manifestation of CTEPH. She developed right hemiplegia without dyspnea or edema. Brain magnetic resonance imaging revealed multiple fresh infarctions, while transesophageal echocardiography revealed a patent foramen ovale. Based on these findings, she was diagnosed as having paradoxical cerebral embolism. During the search for the embolic source, right heart catheterization showed significant pulmonary hypertension and pulmonary angiography revealed chronic thrombi in the peripheral pulmonary arteries, consistent with a diagnosis of CTEPH. To our knowledge, this is the first case of CTEPH to be diagnosed with the onset of paradoxical cerebral embolism. Because CTEPH is the only potentially curable form of pulmonary hypertension, clinicians should consider paradoxical cerebral embolism as a possible initial manifestation of CTEPH.

Late-onset paradoxical reactions 10 years after treatment for tuberculous meningitis in an HIV-negative patient: a case report.

BACKGROUND: Although paradoxical reactions (PRs) to anti-tuberculosis (anti-TB) therapy during treatment are well-established occurrences, PRs presenting as a new lesion after the completion of treatment are extremely rare, and little is known about the management of such cases, particularly of central nervous system (CNS) tuberculosis. CASE PRESENTATION: A 27-year-old female, with a past medical history of tuberculous meningitis 10 years ago and who completed the anti-TB treatment with asymptomatic remnant tuberculomas in the basal cistern, was admitted to our hospital because of a headache and the worsening of pre-existing visual disturbance. Contrast-enhanced T1-weighted brain magnetic resonance imaging (MRI) revealed new tuberculomas in the left sylvian fissure with a diffuse low signal around it. Because repeated polymerase chain reaction and Mycobacterium tuberculosis culture presented negative results and the patient had no laboratory data suggestive of a relapse of tuberculous meningitis, she was diagnosed with late-onset post-treatment PRs and treated with oral corticosteroids, tapered off over 1 year. Eventually, the symptoms were relieved, and the tuberculomas disappeared. CONCLUSIONS: Clinicians should consider the possibility of PRs long after the completion of tuberculous meningitis treatment. Hence, a precise MRI-based examination is imperative for the follow-up of CNS tuberculosis, and the unnecessary administration of anti-TB drugs should be avoided. The use of corticosteroids as a treatment option for post-treatment PRs is seemingly safe when the isolated M. tuberculosis is sensitive to the first-line anti-TB therapy.

Syringomyelia due to Lumbar Spinal Fluid Drainage in the Acute Phase of Subarachnoid Hemorrhage: A Case Report.

Lumbar spinal fluid drainage is a common procedure for treating hydrocephalus and alleviating vasospasm by egesting blood in the subarachnoid cavity after subarachnoid hemorrhage. Despite being an effective and safe procedure, cerebrospinal fluid overdrainage might result in serious complications. Here we report the case of a 49-year-old man who suffered from tonsillar herniation with subsequent cervicothoracic syringomyelia in the acute phase of subarachnoid hemorrhage due to vertebral artery dissection. About 2 weeks after lumbar drainage was switched from external ventricular drainage initiated on the day of subarachnoid hemorrhage, the recovery from the disturbance of consciousness revealed tetraplegia, and magnetic resonance imaging demonstrated tonsillar herniation and syringomyelia. Removal of the spinal drain and resumption of external ventricular drainage resulted in the restoration of the herniated tonsils to the normal position and the complete disappearance of syringomyelia 11 days later. We should consider that spinal syringomyelia could develop as a complication of lumbar spinal fluid drainage in the acute phase of thick subarachnoid hemorrhage, particularly in the posterior cranial fossa.

CD11chigh B Cell Expansion Is Associated With Severity and Brain Atrophy in Neuromyelitis Optica.

BACKGROUND AND OBJECTIVES: Neuromyelitis optica (NMO) is an autoimmune astrocytopathy mediated by anti-AQP4 antibody-producing B cells. Recently, a B-cell subset highly expressing CD11c and T-bet, originally identified as age-associated B cells, has been shown to be involved in the pathogenesis of various autoimmune diseases. The objective of this study was to determine the relationship between the frequency of CD11chigh B cells per CD19+ B cells in the peripheral blood of patients with NMO and the clinical profiles including the brain volume. METHODS: In this observational study, 45 patients with anti-AQP4 antibody-positive NMO in remission and 30 healthy control subjects (HCs) were enrolled. Freshly isolated peripheral blood mononuclear cells were analyzed for immune cell phenotypes. The frequency of CD11chigh B cells per CD19+ B cells was assessed by flow cytometry and was evaluated in association with the clinical profiles of patients. Brain MRI data from 26 patients were included in the study for the analysis on the correlation between CD11chigh B-cell frequency and brain atrophy. RESULTS: We found that the frequency of CD11chigh B cells in CD19+ B cells was significantly increased in patients with NMO compared with HCs. The expansion of CD11chigh B cells significantly correlated with EDSS, past relapse numbers, and disease duration. In addition, a higher frequency of CD11chigh B cells negatively correlated with total brain, white matter, and gray matter volumes and positively correlated with T2/FLAIR high lesion volumes. When the past clinical relapse episodes of patients with or without the expansion of CD11chigh B cells were compared, relapses in the brain occurred more frequently in patients with CD11chigh B-cell expansion. CD11chigh B cells had distinct features including expression of chemokine receptors associated with migration into peripheral inflammatory tissues and antigen presentation. CD11chigh B-cell frequency was positively correlated with T peripheral helper-1 (Tph-1) cell frequency. DISCUSSION: Even during the relapse-free period, CD11chigh B cells could expand in the long disease context, possibly through the interaction with Tph-1 cells. The increased frequency of CD11chigh B cells associated with brain atrophy and disease severity, indicating that this cell population could be involved in chronic neuroinflammation in NMO.

Pulse-gated noncontrast thoracic magnetic resonance angiography for acute aortic dissection with transient ischemic attack: A case report.

Aortic dissection is a rare cause of an acute ischemic stroke or transient ischemic attack (TIA). Aortic dissection is particularly challenging in stroke patients who are eligible for thrombolysis secondary to the diagnostic difficulty within a narrow time window (4.5 h) and have a risk of developing life-threatening hemorrhagic complications following thrombolysis. Computed tomographic angiography (CTA) has been the mainstay of imaging when evaluating acute aortic syndrome. However, it cannot be routinely performed for pregnant patients and those with renal failure or iodine-contrast media allergy. We report a case of a 72-year-old woman who developed transient right-hand paralysis without any chest symptoms. Brain magnetic resonance imaging (MRI) showed no recent infarction; however, the brachiocephalic trunk was not well visualized on carotid magnetic resonance angiography (MRA). Subsequent thoracic pulse-gated noncontrast three-dimensional balanced steady-state free precession MRA (bSSFP-MRA) detected a Stanford type A acute aortic dissection (TAAAD). This was confirmed by CTA, leading to the diagnosis of TIA due to Stanford TAAAD. Pulse-gated noncontrast thoracic bSSFP-MRA was acquired a few minutes after a series of brain MRI scans. This imaging modality is expected to be used as a screening platform to rule out Stanford TAAAD during the hyperacute phase of stroke.

Activation of a Cryptic Splice Site of GFAP in a Patient With Adult-Onset Alexander Disease.

BACKGROUND AND OBJECTIVE: Alexander disease (ALXDRD) is an autosomal dominant neurologic disorder caused by mutations in the glial fibrillary acidic protein (GFAP) gene and is pathologically defined by Rosenthal fiber accumulation. Most mutations are exonic missense mutations, and splice site mutations are rare. We report a very-late-onset autopsied case of adult-onset ALXDRD with a novel splice site mutation. METHODS: Genetic testing of GFAP was performed by Sanger sequencing. Using autopsied brain tissues, GFAP transcript analysis was performed. RESULTS: The patient presented mild upper motor neuron symptoms in contrast to the severe atrophy of spinal cord and medulla oblongata. The patient had c.619-1G>A mutation, which is located in the canonical splice acceptor site of intron 3. The brain RNA analysis identified the r.619_621del (p.Glu207del) mutation, which is explained by the activation of the cryptic splice acceptor site in the second and third nucleotides from the 5' end of the exon 4. DISCUSSION: GFAP gene expression analysis is necessary to clarify the effects of intronic mutations on splicing, even if they are in canonical splice sites. This case showed a much milder phenotype than those in previous cases with missense mutations at Glu207, thereby expanding the clinical spectrum of ALXDRD with Glu207 mutation.

Remarkable improvement in progressive multifocal leukoencephalopathy following acute pyelonephritis with bacteremia.

Progressive multifocal leukoencephalopathy (PML) is caused by John Cunningham (JC) virus in immunocompromized patients such as those with human immunodeficiency virus (HIV) infection, hematological malignancy, autoimmune disorder, and immunodeficiency disorder as well as those undergoing chemotherapy or immunosuppressive therapy. No effective treatments have been established for PML, which commonly causes severe neurological sequelae. We describe the first case of PML in a patient without HIV infection who exhibited remarkable improvement following acute pyelonephritis with Escherichia coli bacteremia.

Intrathecal IgG Synthesis and Persistent Inflammation Are Associated with White Matter Lesions in HIV-negative Patients with Cryptococcal Meningoencephalitis.

Objective Cryptococcal meningoencephalitis (CM) causes significant morbidity and mortality in human immunodeficiency virus (HIV)-negative and HIV-positive populations. White matter lesions (WMLs) have been reported in both populations of CM patients; however, the mechanisms underlying WML formation remain unknown. We herein report the relationship between the intrathecal immune response and the development of WMLs in HIV-negative patients with CM. Methods Eleven consecutive HIV-negative patients with CM who presented at one of three emergency hospitals in Japan from April 2001 to March 2018 were enrolled. For all patients, we retrospectively assessed the relationships between clinical and laboratory information and the presence of WMLs. Results At presentation, 6 patients had WMLs on magnetic resonance imaging (MRI). The cerebrospinal fluid immunoglobulin G (CSF IgG) index was significantly higher in the patients with WMLs than in those without WMLs (mean, 1.34 vs. 0.70, p=0.017). The time from the symptom onset to initial neuroimaging was also significantly longer in the patients with WMLs than in those without WMLs (median, 31.5 vs. 7.0 days; p=0.008). The clinical outcome was comparable among the patients with and without WMLs. Conclusion In HIV-negative patients with CM, a persistent, aberrant immune response to Cryptococcus, such as intrathecal IgG synthesis, may induce WML formation.

Pitfalls in the diagnosis of pupil-sparing oculomotor nerve palsy without limb ataxia: A case report of a variant of Claude's syndrome and neuroanatomical analysis using diffusion-tensor imaging.

Midbrain infarction causing oculomotor nerve palsy with contralateral ataxia is named Claude's syndrome. Herein we report the case of a variant of Claude's syndrome, which shows pupil-sparing oculomotor nerve palsy without the accompanying neurological deficits other than subtle truncal ataxia. MRI and Diffusion Tensor Imaging revealed that midbrain infarction was located rostrally above the decussation of the superior cerebellar peduncle (SCP) and might have partially destructed the tectospinal tract, which resulted in the absence of limb ataxia and presence of subtle truncal ataxia. In this variant of Claude's syndrome, we should carefully assess truncal ataxia to avoid misdiagnosing it as isolated pupil-sparing oculomotor nerve palsy because the patient showed apparently normal gait and truncal ataxia was only revealed by unstable tandem gait.

Dynamic spinal compression revealed by computed tomography myelography in overshunting-associated myelopathy: A case report.

RATIONALE: OSAM is a rare ventriculoperitoneal (VP) shunt complication where cervical spinal cord compression by epidural venous plexus engorgement is caused by cerebrospinal fluid (CSF) overdrainage. Symmetrically indented deformity of the upper cervical spinal cord and surrounding epidural venous engorgement are characteristic radiological findings. Both of them are typically detected on magnetic resonance imaging (MRI) and enhanced computed tomography (CT). PATIENT CONCERNS: The 77-year-old man who underwent the placement of a VP shunt without an antisiphon device to treat post-subarachnoid hemorrhage (SAH) hydrocephalus presented with progressive quadriplegia 10 years postoperatively. DIAGNOSIS: MRI revealed a symmetrically indented spinal cord from the craniocervical junction (CCJ) to the C2 level and enhanced CT showed the epidural venous engorgement, which were characteristic radiological findings of overshunting-associated myelopathy (OSAM). However, MRI atypically failed to detect the engorged epidural vein and showed no compressive lesion around the spinal cord. INTERVENTION: In order to reveal how the cervical spinal cord was deformed and compressed by engorged epidural vein, CT myelography was performed. OUTCOMES: CT myelography proved that the epidural vein dynamically engorged and compressed the cervical spinal cord immediately after rotation and extension of the neck. LESSONS: CT myelography combined with neck rotation and extension revealed the dynamic change of the epidural venous engorgement, and is useful for evaluation and diagnosis of OSAM especially when epidural venous engorgement was not detectable on MRI.