Balamuthia amebic meningoencephalitis and mycotic aneurysms in an infant

Balamuthia amebic encephalitis is rarely reported in infants. To the best of our knowledge, amebic encephalitis complicated by a mycotic aneurysm was only described once. We report on an 8-month-child with laboratory-confirmed Balamuthia mandrillaris meningoencephalitis, complicated by a mycotic aneurysm of the middle cerebral artery.     

An autopsy case of amebic meningoencephalitis. The first Japanese case caused by Balamuthia mandrillaris

We report here the first case of amebic meningoencephalitis caused by Balamuthia mandrillaris in a 78‐year‐old Japanese woman with Sjögren's syndrome. Fourteen days before her death, she presented with high fever and lost consciousness and later developed neck stiffness and abducens palsy. Computed tomography scans of the brain demonstrated multiple low‐density areas throughout the brain. Neuropathologically, hemorrhagic and necrotic lesions with many amebic trophozoites were scattered in the brain and spinal cord. Granulomatous lesions were only rarely found. The amebas were identified as Balamuthia mandrillaris based on immunofluorescence assay. Clinicopathologically, our case was thought to be an intermediate between primary amebic meningoencephalitis due to Negleria fowleri and granulomatous amebic encephalitis due to Acanthameba species. Essentially, the case was one of an elderly person with suspected immunodeficiency with fulminant necrotic meningoencephalitis and scanty granulomatous lesions of 14 days course.     

Pediatric granulomatous cerebral amebiasis: a delayed diagnosis

We present four cases of cerebral amebae infection treated at our neurosurgical department. Patient 1 was a 12-year-old male with skin lesions of 2 years' progression involving the midface. He received a corticosteroid course, and, after that, he presented a right body hemiparesis. Patient 2 was a 5-year-old male, with a past surgical history of fibula fracture and osteomyelitis of 1-year evolution, associated with lesions of the surrounding skin that presented with partial seizures. Patient 3 was a 3-year-old female who presented with a stroke-like episode and with partial seizures. Patient 4 was a 6-year-old male who had ulcerative lesions in the face of 1-year evolution. After a corticosteroid course, he presented with right-body hemiparesis. All patients were human immunodeficiency virus-negative and died 1 month or less after surgery because of progressive evolution of the disease. Histopathology revealed granulomatous amebic encephalitis. All patients revealed infection from Balamuthia mandrillaris (Leptomyxiidae). Treatment consisting of pentamidine, clarithromycin, fluconazole, and 5-fluorocytosine was ineffective. Although extremely uncommon, granulomatous amebic encephalitis should be considered in the differential diagnosis of cerebral lesions while nonspecific, associated granulomatous skin lesions support the diagnosis of amebiasis.     

Steroid-responsive diffuse cerebral white matter lesions in a case of intractable fungal meningoencephalitis]

We report a case of fungal meningoencephalitis with steroid-responsive diffuse cerebral white matter lesions. A 49-year-old male developed auditory hallucination, confusion and fever, on April, 1994. He was diagnosed as having cryptococcal meningoencephalitis based on the detection of cryptococcal antigens in the cerebrospinal fluid (CSF). Intravenous administration of fluconazole resulted in improvement of his neurologic symptoms and CSF findings. For the next seven months, he was treated with oral fluconazole and the neurological status was stable. However, soon after the dose of fluconazole was tapered, he became confused and febrile, which made him admitted to our hospital. Neurological examination on admission showed disturbance of consciousness, disorientation and meningeal irritation. The CSF examination revealed mild pleocytosis (mostly lymphocytes), elevated protein and normal glucose levels, although fungus was not detected. The T2-weighted image of brain MRI demonstrated diffuse hyperintense lesions in the bilateral cerebral white matters. GD-DTPA enhanced MRI showed spotty enhanced lesions in the periventricular white matters. The neurologic symptoms were once relieved after intravenous administration of fluconazole was started, but two months later, he became comatose and needed ventilatory support, despite amphotericine B therapy. Then, a needle brain biopsy targeting the white matter lesion was done. Histopathology of the specimen showed chronic inflammation with granuloma formation and T lymphycyte infiltrate around the small vessels, though fungus was not detected in the tissue. Combined therapy with corticosteroid and antifungal agents remarkably improved the neurological symptoms as well as the MRI findings. In the present case, fungal infection possibly induced an altered immune reactions which resulted in the steroid responsive diffuse cerebral white matter lesions.     

Asymptomatic aneurysm of the cavernous and supraclinoid internal carotid artery in a patient with Balamuthia mandrillaris encephalitis

This is the first report to our knowledge of the successful treatment of an asymptomatic mycotic aneurysm associated with Balamuthia mandrillaris encephalitis. A 27-year-old male with end-stage renal disease presented with generalized seizures following renal transplantation. MRI demonstrated multiple brain masses and an aneurysm of the cavernous and supraclinoid carotid artery. Autopsy of the donor’s brain revealed Balamuthia encephalitis. The patient was placed on an anti-amebic regimen, his condition improved, and 126 days after the kidney transplant, MRI brain showed resolution of the aneurysm and improvement of the enhancing lesions. Balamuthia mandrillaris has been shown to cause a granulomatous encephalitis, with prominent vasculitis. This is the first report to demonstrate the risk of aneurysm formation associated with this infection. Prolonged anti-amebic treatment resulted in resolution of the aneurysm without clinical evidence of subarachnoid hemorrhage.     

Disseminated histoplasmosis causing reversible gaze palsy and optic neuropathy.

Subacute disseminated histoplasmosis is an uncommon entity. Typical neuro-ophthalmologic manifestations are usually secondary to histoplasmomas or encephalitis. A 45-year-old man noted blurred vision while receiving empiric antituberculosis therapy for fever and diffuse granulomatous disease of unknown origin. Vertical-gaze palsy, right horizontal-gaze paresis, and mild right optic neuropathy were found on neuro-ophthalmologic examination. Further questioning revealed a history of frequent contact with fighting cocks from South America. Magnetic resonance images were consistent with multiple hemorrhagic infarcts, areas of inflammation, or both, and cerebral angiography showed changes consistent with vasculitis. A previously obtained biopsy specimen from the duodenum was restained and found to be positive for fungal elements. Serum antigen titers for Histoplasma capsulatum demonstrated evidence of active infection. This case is a rare example of a supranuclear ocular motility disturbance and optic neuropathy secondary to an occlusive vascular process in a patient with subacute disseminated histoplasmosis.     

A case of measles meningoencephalitis with marked increase of plasma cells in cerebrospinal fluid

Marked increase of plasma cells in cerebrospinal fluid (CSF) was observed in a 22-year-old female patient with measles meningoencephalitis. Typical measles exanthema was followed, 3 days later, by neurological abnormalities such as confusion, severe meningeal signs and bilateral pyramidal signs. Immunological examination showed slight elevation of IgG, normal T cell subsets and NK cell activity in peripheral blood. The antibody titer of measles was 32x in serum (4x after 2 months). CSF contained 167 cells/mm3, 66% of which was plasma cells. Total protein was 75 mg/dl, and IgG (24.5 mg/dl) showed high values. The antibody titer of measles was 4x (1x after 2 months). The antibody index was 7.15 (N = 2). The anti-myelin antibody was 8x. Oligoclonal band in CSF was negative. The appearance of large number of plasma cells in CSF in viral meningoencephalitis is extremely rare in the literature. However, B cell activation in peripheral blood in measles encephalitis has been reported. Therefore, immunologically abnormal findings in CSF of this case suggest marked B cell activation occurring in the central nervous system in measles meningoencephalitis.     

Acute onset of tuberculous meningoencephalitis presenting with symmetric linear lesions in the bilateral thalamus: a case report]

A 18-year-old woman was admitted to our hospital because of high fever and headache. Nuchal stiffness was present, and a CSF examination showed lymphocyte-domonant pleocytosis and a decreased level of glucose. Although antibiotics, aciclovir and an antimycotic drug were administered, disturbance of consciousness, involuntary movements, and pyramidal tract signs appeared. Soon after the medications were changed to antituberculous medicines, the meningoencephalitis started to subside, and was finally cured. Judging from the clinical findings, the CSF findings, the effectiveness of antituberculous medicines, an elevated ADA level in CSF, and positive conversion in tuberculin tests, the final diagnosis was made as tuberculous meningoencephalitis. At the severest stage of the disease, a brain MRI showed symmetric, linear lesions without the effect of Gd-enhancement in the bilateral thalamus, which thereafter disappeared along with the healing of the illness. From all these things, we conclude that thalamic and other parenchymal lesions should be kept in mind in case of acute tuberculous meningoencephalitis.     

Cerebrospinal fluid in acute necrotizing encephalitis. Hypochlorrhachia as a diagnostic aid

The chemical and microscopic findings in the cerebrospinal fluid (CSF) were compared in patients with acute necrotizing encephalitis (due to herpes simplex virus), nonherpetic aseptic meningoencephalitis and tuberculous meningitis. The prominent findings in the CSF of the patients with herpes simplex encephalitis were an increase in white and red blood cells, increased protein, normal glucose and low chloride levels. The latter finding differentiates this disease from other forms of viral encephalitis and may be helpful in the establishment of early diagnosis and treatment.     

Multifocal progressive leukoencephalitis in a patient given fludarabine for chronic lymphoid leukemia

A 74-year- man was hospitalized for subacute aphasia and right hemiparesis. He had had chronic lymphoid leukemia for 11 years and had been treated 5 months earlier with 3 courses of fludarabine. Magnetic resonance imaging showed lesions of the temporo-occipital white matter compatible with progressive multifocal leukoencephalitis (PML). The presence of JC virus was demonstrated first by in situ hybridation after a brain biopsy and second with polymerase chain reaction on CSF. The diagnosis of PML was confirmed. The causality of fludarabine treatment is discussed.     

Update on herpes simplex encephalitis

Herpes simplex encephalitis is the most common identified cause of sporadic viral encephalitis in the United States. Early diagnosis is critical because treatment with the antiviral drug acyclovir dramatically decreases morbidity and mortality. The use of polymerase chain reaction (PCR) techniques to amplify the genome of herpes simplex virus (HSV) from cerebrospinal fluid (CSF) has become the diagnostic procedure of choice. False-positive CSF HSV PCR results are rare when testing is performed in experienced laboratories. Negative CSF HSV PCR results should always be interpreted in the context of the timing of specimen collection and the likelihood of disease. Negative CSF HSV PCR tests can occur within the first 72 hours of illness, with subsequent tests becoming positive. Patients with HSV encephalitis will typically have a negative CSF HSV PCR after 14 days of acyclovir treatment, and a persisting positive PCR should prompt consideration of additional or revised antiviral therapy. Quantitative PCR testing provides information about HSV viral load in CSF, but the potential correlation of viral load with prognosis or other clinical features of disease remains uncertain. Although the neuroimaging abnormalities seen in HSV encephalitis are not unique, more than 90% of patients with proven HSV encephalitis will have magnetic resonance imaging (MRI) abnormalities involving the temporal lobes. Special MRI techniques, including fluid-attenuated inversion recovery and diffusion-weighted imaging, might reveal abnormalities not seen with conventional imaging sequences. Neuroimaging patterns in infants and children differ significantly from those seen in adults and include a higher frequency of extratemporal lesions.     

Neurosyphilis revealed by a multiple cranial neuropathy: magnetic resonance imaging findings

INTRODUCTION: We report the magnetic resonance imaging (MRI) findings in a case of neurosyphilis revealed by the involvement of two cranial nerves. CASE REPORT: A 41-year-old man developed a right cochleovestibular and left trigeminal neuropathy, associated with high serum titers of VDRL and TPHA, high titers of TPHA in the cerebrospinal fluid (CSF) and several CSF oligoclonal IgG bands. On MRI, hypertrophy and gadolinium contrast enhancement of these cranial nerves were associated with several supratentorial cortical nodules surrounded by marked cerebral edema, corresponding to syphilitic gummas. One of these cortical nodules was biopsied. Microscopic examination showed lesions of meningoencephalitis with necrosis and granulomatous vasculitis. After penicillin therapy, the serum VDRL titers and the MRI abnormalities disappeared, a partial clinical recovery was observed and a significant reduction of the serum TPHA titers was found. DISCUSSION: Such MRI abnormalities are not specific and can be observed in various tumoral, auto-immune and infectious diseases. They can also mimic neurofibromatosis type II. Cranial nerve involvements in neurosyphilis can result from nerve inflammation in basal meningitis, nerve ischemia in meningovasculitis or from compression by an adjacent gumma. In our case, the cranial neuropathy was related to a mixed meningovascular and parenchymatous form of neurosyphilis.     

Relapsing herpes simplex encephalitis following antiviral therapy

Although treatment for herpes simplex virus (HSV) encephalitis with antiviral agents has improved survival, occasional patients experience unexplained clinical exacerbations. This report presents evidence that some relapses may occur from recurrent viral encephalitis. An adult male developed the classic symptoms of HSV encephalitis. The cerebrospinal fluid (CSF), electroencephalogram, and isotope brain scan suggested a localized encephalitis involving the left temporal lobe. The pateint was treated for 10 days with high doses of cytosine arabinoside instead of the currently recommended adenine arabinoside. Progression of encephalitis stopped, and clinical recovery occurred. The HSV antibody titer increased eightfold. Fifty-four days after the initial encephalitis, the patient relapsed with a subacute progressive encephalitis involving the same brain area. The CSF demonstrated oligoclonal bands, elevated immunoglobulin G levels (100 mg/dl), and a high HSV antibody titer (1:8,192 by indirect hemagglutination test). From a left temporal lobe biopsy taken 74 days after onset of the initial encephalitis, herpes simplex virus type 1 was isolated. Without renewed antiviral drug therapy, the patient slowly recovered.     

Granulomatous encephalitis,intracranial arteritis,and mycotic aneurysm due to a free-living ameba

Summary Primary amebic meningoencephalitis and granulomatous amebic encephalitis are well recognized clinicopathological entities caused by free-living amebas. Associated arteritis and mycotic aneurysms with infiltration of intracranial arteries by lymphocytes, amebic trophozoites and cysts have not been previously reported.A 26-month-old girl had a 3-week history of encephalitis, characterized, initially, by vomiting and lowgrade fever. Subsequently, she developed ataxia, generalized weakness, lethargy, and esotropia. The first CSF showed 490 RBC/l, 705 WBC/l with 90% mononuclears. Her pupils reacted briskly to light. Moderate nuchal rigidity, nystagmus, fixed downward gaze, anisocoria, bilateral 6th nerve palsy, left arm monoparesis and left Babinski were present. CAT scan revealed slight symmetrical dilatation of anterior horns of lateral ventricles and an area of abnormal enhancement above the 3rd ventricle. She died 14 days after admission, 5 weeks after onset of symptoms.The brain showed focal necrotizing encephalopathy, involving thalami, cerebellum, brain stem, and cervical and upper thoracic spinal cord. Numerous free-living amebic trophozoites and cysts were present within a chronic granulomatous encephalitis. There were thrombosis of basilar, posterior cerebral, and vertebral arteries with profuse chronic panarteritis, fibrinoid necrosis, and mycotic aneurysms.     

The fourth case of Veillonella parvula meningoencephalitis diagnosed by stereotactic brain biopsy

IntroductionVeillonella parvula is a small, nonfermentative anaerobic gram-negative coccus. Meningitidis due to V. parvula seen extremely rare. Here we want to report the fourth case with meningoencephalitis due to V. parvula and emphasize the significance of brain biopsy in encephalitis cases with unknown ethiology.Case reportA 64-year-old male admitted to the emergency room with a frontal and bitemporal headache. On physical examination, the patient was concious and oriented, his reaction time was prolonged. There was no focal neurological deficit, fever and meningeal signs. The magnetic resonance imaging scan showed hyperintensity on the T2-FLAİR weighted sequences and diffusion restriction at the same place, concordant with encephalitis. On the third day of admission, oromandibular focal motor seizures were observed. We could not define any microbiological agent by analysis of cerebrospinal fluid. Stereotactic brain biopsy performed and the bacterial culture of the biopsy material grew V. parvula.DiscussionWe reported the fourth V. parvula encephalitis case in the literature. The aim of this case report is to emphasize that brain biopsy should be considered in patients with encephalitis of unknown etiology.     

Granulomatous Herpes Simplex Encephalitis in an Infant With Multicystic Encephalopathy: A Distinct Clinicopathologic Entity?

BackgroundHerpes simplex virus encephalitis can manifest as a range of clinical presentations including classic adult, neonatal, and biphasic chronic-granulomatous herpes encephalitis.MethodWe report an infant with granulomatous herpes simplex virus type 2 encephalitis with a subacute course and multicystic encephalopathy.CaseA 2-month-old girl presented with lethargy and hypothermia. Computed tomography scan of the head showed multicystic encephalopathy and calcifications. Cerebrospinal fluid analysis by polymerase chain reaction testing for herpes simplex virus 1 and 2, enterovirus, and cytomegalovirus was negative. Normal cerebrospinal fluid interferon-α levels argued against Aicardi-Goutières syndrome. The patient died 2 weeks after presentation. At autopsy, multicystic encephalopathy was confirmed with bilateral gliosis, granulomatous inflammation with multinucleated giant cells, and calcifications. Bilateral healing necrotizing retinitis suggested a viral etiology, but retina and brain were free of viral inclusions and immunohistochemically negative for herpes simplex virus-2 and cytomegalovirus. However, polymerase chain reaction analysis showed herpes simplex virus-2 DNA in four cerebral paraffin blocks. Subsequent repeat testing of the initial cerebrospinal fluid sample using a different polymerase chain reaction assay was weakly positive for herpes simplex virus-2 DNA.ConclusionGranulomatous herpes simplex virus encephalitis in infants can present with subacute course and result in multicystic encephalopathy with mineralization and minimal cerebrospinal fluid herpes simplex virus DNA load. Infectious etiologies should be carefully investigated in the differential diagnosis of multicystic encephalopathy with mineralization, in particular if multinucleated giant cells are present.     

An autopsy case of cryptococcal meningoencephalitis: correlation of MRI and pathologic findings

A comparative study of MRI and pathology was performed on a case of cryptococcal meningoencephalitis. An 11-year-old female presented with confusion and vomiting. On admission, CSF examination revealed spherical fungal cells with mild pleocytosis, decreased glucose and elevated protein level. MRI showed multiple punctate lesions in the basal ganglia with high intensity on T2-weighted image, while enhanced MRI revealed diffuse meningeal involvement. Post-mortem examination disclosed that the T2-weighted lesions found in the basal ganglia were aggregated small cystic lesions consisting of a cryptococcal invasion of Virchow-Robin spaces, termed "soap bubble lesions", characteristic findings of cryptococcal meningoencephalitis. Thus MRI findings of the basal ganglia and meninges may help to diagnose cryptococcal meningoencephalitis.     

微孢子虫脑炎一例临床和病理表现

目的研究微孢子虫脑炎的临床与病理形态学表现。方法对1例微孢子虫脑炎患者的临床、脑活体组织检查组织病理特点进行研究。结果此病呈亚急性或慢性起病，体温大多正常或37．5℃以下，升高见于病情加重出现昏迷时；患者不伴有人类免疫缺陷病毒感染但免疫力低下；全脑多发病灶，有脑膜刺激征；骨髓检查呈感染骨髓象；发病初期类风湿因子升高；中期以后出现间接胆红素升高现象；脑脊液蛋白、免疫球蛋白IgG、IgA升高，细胞学检查示淋巴细胞反应。患者伴有胸膜炎、腹膜炎及膀胱炎。脑MRI表现为双侧大、小脑半球脑白质区见斑片状等长T1、长T2信号影，压水扫描（FLAIR）呈高信号，无明显占位效应，强化扫描病灶呈斑点状及环形强化；神经系统损害症状呈永久性，治疗无效。脑组织病理显示神经元变性、核固缩，有血管周围炎现象，神经元内胞质内可见病原体。Wister大鼠腹腔内注射患者脑脊液2周后出现多脏器内炎症反应，腹腔灌洗液内发现大量病原体。结论患者伴有多脏器病变，脑内多发病灶，脑组织病理呈炎症反应，Kltiver-Barrer-PAS染色法可显示神经元内病原体。动物腹腔注射培养可增加病原体的检出率。