Recurrent Meningoencephalitis Research Articles

Primary Sjogren’s syndrome presents as recurrent meningoencephalitis

Primary Sjogren’s syndrome is an autoimmune disease mostly affecting middle-aged females. It usually involves the exocrine glands system resulting in dryness of the mouth and eyes. It rarely involves the Central nervous system. Here we present a case of Sjogren’s syndrome with a rare presentation at an unusual age. An 80 years old patient who presented with 3 episodes of recurrent aseptic meningitis in a year, was diagnosed with primary Sjogren’s syndrome after extensive workup. She responded well to immunomodulatory treatment. Clinicians should consider the rare presentation and age of autoimmune diseases when facing the challenging clinical situation.

Relapsing optic neuritis and meningoencephalitis in a child: case report of delayed diagnosis of MOG-IgG syndrome

BackgroundRecurrent optic neuritis (ON) was previously thought to be associated with multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD). Meningoencephalitis has recently been suggested to be a clinical finding typical of myelin oligodendrocyte glycoprotein (MOG) encephalomyelitis. We report a Chinese patient with recurrent ON at disease initiation, who had a delayed diagnosis of MOG-IgG syndrome, until recurrent meningoencephalitis appeared and serum MOG-IgG was detected.Case presentationFrom the age of 7 years, an AQP4-IgG negative female patient had 10 disease recurrences, including 4 episodes of recurrent ON, 4 episodes of fever and meningoencephalitis, and 2 episodes of ON as well as meningoencephalitis. She was initially diagnosed as recurrent ON and treated with glucocorticoids followed by gradual tapering when ON reoccurred. Later, she was diagnosed as central nervous system infection when fever and meningoencephalitis appeared, and antiviral drugs and glucocorticoids were used. However, when she returned to our department for follow-up on July 2017, the results of serum demyelinating autoimmune antibody revealed positive MOG-IgG (titer 1:320 by an in-house, cell-based assay using live cells transfected with full-length human MOG). A diagnosis of MOG-IgG syndrome was established.ConclusionsTesting for MOG-IgG in atypical MS and NMOSD patients, and patients with meningoencephalitis with a history of relapsing demyelinating symptoms is warranted.

Neuro-Behçet's psychiatric symptoms

Neuro-Behçet (NB) results from inflammatory peri-vasculitis affecting the central nervous system. Non-specific neurological and psychiatric symptoms are rarely the first presentation and may difficult or delay diagnosis and treatment.We review, based on a case study and published literature, the psychiatric symptoms of NB when no clear evidence of neurologic disease activity is present.We present the case of a female patient, who was diagnosed NB at age 23 for recurrent meningo-encephalitis, that showed progressive behavioral changes, with increased impulsivity, disinhibition, hostility and self-neglect.Raised in a dysfunctional family, she ran away from home at 16, abandoned her studies, started abusing cannabinoids and showed an erratic life course.In 2015, because of increasing psychiatric symptoms, she was admitted to hospital and a complete neuropsychological evaluation showed that she had a significant decline from an above average premorbid cognitive function, specifically related to memory deficits. CT and MRI didn’t show typical signs of active disease. However, a SPECT scan showed hypo-perfusion of the frontal cortex compatible with the patient's symptoms.It is difficult to assess NB's activity as brain inflammation is only observed after structural changes are present. In this case the SPECT correlated well with psychiatric symptoms. The differential diagnosis includes organic pathology with psychiatric symptoms, psychiatric disease, personality disorders and substance abuse.Disclosure of interestThe authors have not supplied their declaration of competing interest.

Recurrent meningoencephalitis and hyponatremia in childhood neurosarcoidosis

Purpose Sarcoidosis is a rare multi-system inflammatory disease of unknown etiology, the pathology of which is characterized by the presence of non-caseating granulomas. Neurosarcoidosis can affect any part of the peripheral or central nervous system and occurs in approximately 5-15% of all sarcoidosis patients. In this report, we present a case of isolated neurosarcoidosis in a child manifested as recurrent meningoencephalitis and hyponatremia. Results

Neurological Involvement in Paediatric Behçet's Disease

Behçet disease (BD) is a systemic vasculitis which can affect the neurological system. Neuro-Behçet's disease (NBD) is not well described in children. Our study aimed to analyse the clinical patterns of paediatric NBD and to describe their repercussions on children's schooling. We performed a retrospective chart review of 12 NBD children and a phone interview of patients and their physicians to investigate their last physical and schooling status. In 40 BD patients reviewed, 12 (sex ratio 8M/4F) had neurological involvement. The mean age of onset was 11 years. In 4 cases, neurological symptoms were the initial presentation. In other cases, NBD occurred within a mean time of 10 months after BD was diagnosed. Cerebral venous thromboses were frequent (5/12) as compared to recurrent meningoencephalitis (2/12), rhombencephalitis (2/12), transverse myelitis (1/12), peripheral neuropathy (1/12) or psychiatric disturbances (1/12). 9 patients had sequelae and 8 had difficulties in learning. 6 stopped at middle school level. For the other patients, an arrangement of the teaching environment was needed due to visual, auditory and concentration disorders. Neurological involvement is frequent in BD children and its consequences could be severe. Timely accommodations are required to facilitate their ability to follow the normal curriculum.

Association of post-traumatic communication of endocranium and nasal cavity with recurrent meningoencephalitis without rhinorrhea

A gunshot head injury, characterized by a huge intensity of mechanical force, in addition to the direct tissue damage at the location of direct impact, may cause a skull and skull base fracture, distant from the the point of direct impact, which could be further complicated by creating a communication between endocranium and nasal/paranasal cavities. Such cases pose a great diagnostic and therapeutic challenge for every clinician. The patient is presented with the history of a perforating gunshot head injury six years ago, with recurrent attacks of meningoencephalitis subsequently, without rhinorrhea. By using high resolution CT scans, previous traumatic skull injury was verified and a fissure in the frontoethmoidal region, far from the point of direct impact, was detected. The patient underwent transnasal endoscopic surgery, in order to seal the communication on skull basis. The patient did not suffer from meningoencephalitis during the next two years. In the cases with late occurrence of posttraumatic meningoencephalitis with no signs of rhinorrhea, a possibility of an existing communication between intracranial and nasal cavities should be considered, as well. By applying modern diagnostic and therapeutic procedures such communication should be precisely located and sealed.

ダプソンの併用により副腎皮質ステロイド薬の減量が可能になった神経Ｓｗｅｅｔ病の１例

A 35-year-old Japanese man was admitted to our hospital with recurrent meningoencephalitis of unknown etiology. He presented with fever, convulsions and loss of consciousness, which started at age 33. We diagnosed him with neuro-Sweet disease (NSD) based on human leukocyte antigen (HLA) B-54/Cwl positivity and neutrophilic infiltration into the dermis in a biopsied skin plaque. Intravenous methylprednisolone and oral prednisolone markedly improved his fever and CSF pleocytosis. Five years later he was again admitted to our hospital with high fever, oral aphthae and dull-red edematous plaques on the face and body. He was conscious, but he had neck stiffness, mild hyperreflexia in all limbs and an extensor plantar response. Laboratory tests revealed increased white blood cell, erythrocyte sedimentation rate (ESR) and C-reactive protein level. CSF analysis indicated mild pleocytosis. A skin biopsy from an edematous plaque revealed neurotrophils infiltrating the upper dermis. We treated him with intravenous methylprednisolone (1 g/day) for 3 days, followed by oral prednisolone (50 mg/day). His symptoms improved remarkably; however, he had recurrence of symptoms, such as fever, meningial irritation and oral aphtae, with attempted taper of prednisolone. We started treatment with dapsone (75 mg/day) in addition to prednisolone, and could taper oral prednisolone, without a relapse. However, because some mildly recurred with the tapering of dapson, we maintained dapsone treatment at 75 mg daily, added colchicine (1 mg/ day) and tapered only prednisolone. His symptoms were improved and no relapse has been observed. NSD is characterized by neurotrophic hyperactivation and infiltration of tissues. It is highly responsive to systemic corticosteroid therapy; however, some cases show frequent recurrences on tapering of corticosteroids. Dapsone is considered to prevent neurotrophic overactivity. In this case, dapsone was supposed to be effective to prevent reccurence of NSD upon tappering corticosteroids. Dapsone should be a therapeutic options for steroid-dependent NSD showing frequent recurrence.

Successful Treatment of Pituitary Abscess with Oral Administration of Sparfloxacin

Pituitary abscess is a rare infection and it is difficult to make the correct diagnosis. It is usually treated by a combination of surgical drainage and intravenous administration of antibiotics. We describe a 74-year-old woman with recurrent meningo-encephalitis due to pituitary abscess. The abscess increased in size in spite of the intravenous administration of panipenem/betamipron (PAPM/BP), clindamycin (CLDM) and chloramphenicol (CP). Finally she was successfully treated with oral administration of sparfloxacin (SPFX) without operation.

Neuro-Behçet: MR study of a group of patients

Behçet's disease is a chronic inflammatory, multisystemic disease of unknown aetiology. Central nervous system involvement occurs in a variable proportion of cases (4 to 49%) and is due to intracranial hypertension secondary to dural sinus thrombosis, inflammatory parenquimal lesions or recurrent meningoencephalitis. We reviewed 12 patients, 7 men and 5 women, aged between 27 to 40 years at the time of diagnosis. Neurological manifestations were secondary to parenquimal lesions in 7 patients, meningoencephalitis in 3 patients (including one with extensive medullary lesion) and dural sinus thrombosis in 2. Magnetic Resonance (MR) findings in Neuro-Behçet are non-specific. Nevertheless, MR has a role in characterizing brain lesions topography, helping in the diagnosis and in the follow-up of these patients.

The human immunodeficiency virus (HIV) protease inhibitor indinavir directly affects the opportunistic fungal pathogen Cryptococcus neoformans

Highly active antiretroviral therapy (HAART), that includes human immunodeficiency virus (HIV) protease inhibitors (PIs), has been remarkably efficacious including against some opportunistic infections. In this report we investigated the effect(s) of the PI indinavir on protease activity by Cryptococcus neoformans, an opportunistic fungal pathogen responsible for recurrent meningoencephalitis in AIDS patients. Indinavir was also tested for potential effects on other parameters, such as fungal viability, growth ability and susceptibility to immune effector cells. It was found that indinavir impaired cryptococcal protease activity in a time- and dose-dependent fashion. The phenomenon was similarly detectable in ATCC/laboratory strains and clinical isolates. C. neoformans growth rate was also significantly reduced upon exposure to indinavir, while fungal viability was not affected and mitochondrial toxicity not detected. Furthermore, as assessed by an in vitro infection model, indinavir significantly and consistently augmented C. neoformans susceptibility to microglial cell-mediated phagocytosis and killing. Overall, by providing the first evidence that indinavir directly affects C. neoformans, these data add new in vitro insights on the wide-spectrum efficacy of PIs, further arguing for the clinical relevance of HAART against opportunistic infections in AIDS.

Nramp1 gene affects selective early steps in macrophage-mediated anti-cryptococcal defense

Cryptococcus neoformans is an opportunistic fungus responsible for severe and often recurrent meningoencephalitis in immunodepressed patients. Initial evidence suggests that C. neoformans is a facultative intracellular pathogen; however, the strategies by which C. neoformans undergoes survival and eventually proliferation have not been elucidated. We investigated the role of Nrampl gene in macrophage-mediated anti-cryptococcal defense. Using cell lines expressing the functional, mutated or knockout gene, it was established that Nramp1 (1) is not involved in the phagocytic event, (2) influences anti-cryptococcal activity in the early steps but not at later times, and (3) is unrelated to the biomolecular pathways through which C. neoformans impairs macrophage secretory response. Although the functional role of Nramp1 is still far from being elucidated, the present data add insight into its involvement in macrophage-mediated antimicrobial defense, particularly in the initial steps allowing C. neoformans growth inhibition.