First report of Bickerstaff's brainstem encephalitis caused by Salmonella Dublin: a case report.

BACKGROUND: Diseases caused by nontyphoid Salmonella can range from mild, to self-limiting gastroenteritis and severe invasive infection. Relatively rarely, Salmonella may cause severe encephalopathy. CASE PRESENTATION: We report a suspected case of Bickerstaff's brainstem encephalitis caused by Salmonella Dublin. A young man presented with impaired consciousness, ataxia, dysarthria, limb weakness, and restricted eyeball abduction. His clinical symptoms were consistent with Bickerstaff's brainstem encephalitis. CONCLUSIONS: This is the first case report of Bickerstaff's brainstem encephalitis caused by Salmonella Dublin in the literature. After treatment, he recovered and was discharged. Early antibiotic treatment of sepsis may control the disease and avoid serious encephalopathy.

Burkholderia pseudomallei Rapidly Infects the Brain Stem and Spinal Cord via the Trigeminal Nerve after Intranasal Inoculation.

Infection with Burkholderia pseudomallei causes melioidosis, a disease with a high mortality rate (20% in Australia and 40% in Southeast Asia). Neurological melioidosis is particularly prevalent in northern Australian patients and involves brain stem infection, which can progress to the spinal cord; however, the route by which the bacteria invade the central nervous system (CNS) is unknown. We have previously demonstrated that B. pseudomallei can infect the olfactory and trigeminal nerves within the nasal cavity following intranasal inoculation. As the trigeminal nerve projects into the brain stem, we investigated whether the bacteria could continue along this nerve to penetrate the CNS. After intranasal inoculation of mice, B. pseudomallei caused low-level localized infection within the nasal cavity epithelium, prior to invasion of the trigeminal nerve in small numbers. B. pseudomallei rapidly invaded the trigeminal nerve and crossed the astrocytic barrier to enter the brain stem within 24 h and then rapidly progressed over 2,000 µm into the spinal cord. To rule out that the bacteria used a hematogenous route, we used a capsule-deficient mutant of B. pseudomallei that does not survive in the blood and found that it also entered the CNS via the trigeminal nerve. This suggests that the primary route of entry is via the nerves that innervate the nasal cavity. We found that actin-mediated motility could facilitate initial infection of the olfactory epithelium. Thus, we have demonstrated that B. pseudomallei can rapidly infect the brain and spinal cord via the trigeminal nerve branches that innervate the nasal cavity.

Multisystemic Listeriosis in a Common Brushtail Possum (Trichosurus vulpecula) and Two Common Ringtail Possums (Pseudocheirus peregrinus).

A single free-ranging common brushtail possum (Trichosurus vulpecula) and 2 captive sibling common ringtail possums (Pseudocheirus peregrinus)from a zoological facility in Sydney, Australia, were diagnosed with multisystemic listeriosis. The brushtail was found dead in an animal enclosure while the ringtails presented with signs of cardiovascular collapse and died shortly thereafter. All 3 animals were culture positive forListeria monocytogenesand demonstrated focal suppurative lesions within the brainstem in addition to fulminant disease in other areas of the thorax and/or abdomen. Listeriosis in phalangeriformes species has rarely been reported, and brainstem lesions have not previously been described. It is speculated that access to the brainstem by the organism may have occurred hematogenously or via retrograde migration along cranial nerves. Sources of infection and the possibility of transmission between animals are also discussed.

Misdiagnosed murine typhus in a patient with multiple cerebral infarctions and hemorrhage: a case report.

BACKGROUND: Rickettsias cause a wide spectrum of tick-, flea-, or mite-borne infections. Rickettsial infections have no classical manifestations and can often lead to encephalitis, which can be fatal if improperly diagnosed. CASE PRESENTATION: A 74-year-old male farmer was admitted to the hospital with fevers and a headache that had lasted for 10 days, followed by 4 days of unconsciousness, and his condition continued to deteriorate. Images showed multiple acute lesions in the brain stem, and bilateral cerebral and cerebellar hemispheres. He was finally diagnosed with endemic typhus and treated with antibiotics that resulted in improvement. CONCLUSION: The present report describes a patient with a rickettsial infection and subsequent deterioration to coma because of an initial misdiagnosis. Because of the similarity to other infectious diseases, physicians should be more vigilant towards the history and radiologic results to ensure early detection and avoid complications which may prove to be fatal.

Molecular characterization of encephalitic bovine listeriosis from southern Brazil.

Reports of bovine listeriosis in Brazil are uncommon, being restricted to citations within retrospective studies, resulting in scarce documented information of this important disease of cattle. This manuscript describes the molecular findings associated with spontaneous encephalitic listeriosis in two steers from distinct herds within the state of Paraná, southern Brazil. Both animals demonstrated altered consciousness suggestive of brain stem dysfunctions and died a few days after the initial onset of disease. Polymerase chain reaction (PCR) assays were designed to target specific genes of infectious neurological agents of cattle. These included bovine herpesvirus 1 and 5 (BoHV-1 and BoHV-5), ovine herpesvirus 2 (OvHV-2), Listeria monocytogenes, and Histophilus somni. Rabies virus was discarded in evaluations done at the official state diagnostic laboratory. Gross alterations were insignificant; histopathology demonstrated rhombencephalitis associated with macrophage-predominant, multifocal to coalescing microabscesses and extensive perivascular cuffings in both steers. The L. monocytogenes PCR assay amplified the 172-bp amplicon of the listeriolysin gene from the brain stem of both animals and from the telencephalon, thalamus, and cerebellum of one of them. Phylogenetic analyses demonstrated that the strains derived from this study clustered with known strains of L. monocytogenes lineage I. The BoHV-1 and BoHV-5, OvHV-2, and H. somni PCR assays were negative. These results confirm the participation of L. monocytogenes lineage I in the etiopathogenesis of the neurological disease herein described and represent the first complete description of encephalitic listeriosis in cattle from Brazil.

Successful treatment of brainstem blastomycosis with fluconazole.

The lipid formulation of amphotericin B is the initial drug of choice for central nervous system blastomycosis, but it is costly and associated with significant toxicity. This case report details a patient with primary pulmonary blastomycosis with dissemination to the skin, one joint, and the brainstem that was successfully treated solely with high-dose fluconazole.

Efficacy of alertness training in a case of brainstem encephalitis: clinical and theoretical implications.

Although attention functions are often impaired after stroke, traumatic brain injury or inflammatory diseases, little is known about the time course and the long-term efficacy of training-induced improvement. The present single case study evaluates the time course and longitudinal stability of attention improvement after alertness training by repeatedly testing the subject between individual training sessions as well as one and seven months after the end of the training. The outpatient (M.P.) trained developed severe alertness deficits following brainstem encephalitis in 2003 without signs of cortical damage, and since then had not achieved full recovery. In 2008, M.P. participated in 15 treatment sessions on 15 separate working days over a period of three weeks. In each session a 45-minute alertness training task was administered, using the CogniPlus ALERT computer training program. Attention performance was assessed by neuropsychological tests four years, one year, and immediately before the therapy after every third training session and three times after the termination of therapy. Furthermore, a self-report questionnaire measured subjective experience of attention in everyday life situations. In order to compare the performance between training sessions, a procedure specialised for psychometric single-case diagnosis was used to analyse the data. Surprisingly, even after three consecutive training sessions, M.P. showed immense improvement in alertness. Furthermore, after two weeks she felt more energetic and more able to concentrate. Six months after the end of the training the improvement remained stable. The unexpectedly fast time course of recovery induced by the training, as well as the stable long-term effects, probably depend on intact cortical structures. In M.P. it appeared that top-down control of the alertness network on impaired brainstem arousal structures had been re-activated by the training procedure and had remained stable across a long time period.

Latent herpes simplex virus in the central nervous system of rabbits and mice.

Herpes simplex virus (HSV) type 1 induces a long-standing latent infection in the central nervous system of mice and rabbits. The infection was extablished in the brain stems of rabbits after corneal inoculation of the virus, and in the spinal cords of mice after rear footpad infection. In these animals, infectious virus could not be recovered by direct isolation from tissues; it was detected only after the tissues were maintained as organ cultures in vitro.

Transneuronal transfer of herpes virus from peripheral nerves to cortex and brainstem.

The transneuronal transfer of neurotropic viruses may represent an effective tool for tracing chains of connected neurons because replication of virus in the recipient neurons after transfer amplifies the "tracer signal." Herpes simplex virus type 1 was transferred transneuronally from forelimb and hindlimb nerves of rats to the cortical and brainstem neurons that project to the spinal enlargements to which the nerves receiving injections are connected. This transneuronal transfer of herpes simplex virus type 1 from peripheral nerves has the potential to be used to identify neurons in the brain that are related transsynaptically to different nerves and muscles.

Nervous signs associated with otitis and cranial osteomyelitis and with Ornithobacterium rhinotracheale infection in red-legged partridges (Alectoris rufa).

A case of nervous signs in red-legged partridges (Alectoris rufa) associated with a severe otitis and osteomyelitis is reported. The outbreak was characterized by abnormal head position, torticollis and difficulty in standing, walking and flying. Pathological, microbiological and molecular genetic data supported an association with Ornithobacterium rhinotracheale (ORT) infection. Clinical signs persisted for several days and were accompanied by weight loss leading to death. Morbidity was approximately 20% and most birds died if untreated. Lesions were mainly characterized by a severe osteomyelitis of the cranial bones and purulent inflammation of the external, middle and inner ears. O. rhinotracheale was isolated from ear samples, skull and brain stem in pure culture. Genetic characterization by pulsed-field gel electrophoresis of the clinical isolates showed that the outbreak was caused by a single strain of ORT. This appears to be the first report of otitis associated with ORT in an avian species.

Fatal immune reconstitution inflammatory syndrome with human immunodeficiency virus infection and Candida meningitis: case report and review of the literature.

Immune reconstitution inflammatory syndrome (IRIS) is an increasingly recognized phenomenon of paradoxical worsening of patients with acquired immunodeficiency syndrome (AIDS) upon initiation of highly active antiretroviral therapy (HAART). To date, there have been limited reports of IRIS in the central nervous system (CNS). Here, the authors describe a 43-year-old man with AIDS who presented with subacute meningitis. No pathogenic organism was identified by routine diagnostic tests, and he was treated empirically with an antituberculous regimen and initiated on HAART therapy. Soon after, he had a precipitous neurologic decline leading to his death. Postmortem evaluation showed a basilar Candida meningitis as well as vasculitis characterized by CD8+ T-cell infiltration, consistent with IRIS. The authors discuss the challenges in diagnosing fungal meningitides and the risks of initiating HAART therapy in those with possible undiagnosed underlying opportunistic infections. Additionally, the authors review the literature regarding CNS IRIS.

A rare case of brainstem encephalitis by Listeria monocytogenes with isolated mesencephalic localization. Case report and review.

We describe a case of brainstem infection by Listeria monocytogenes with right oculomotor palsy and lip drop, facial hypoesthesia, left arm paresthesia, positive blood culture, and sterile liquor in a 63-year-old man. Magnetic resonance imaging revealed an isolated mesencephalic lesion. Localization of this kind accounted for 3% of 111 cases reviewed.

Encephalomalacia with Enterococcus durans infection in the brain stem and cerebral hemisphere in chicks in Japan.

Fourteen chicks, 7-12 days old, that died or had neurological signs (depression, paralysis, torticollis, incoordination, and rolling) were investigated pathologically and microbiologically. Variably sized foci of malacia were present within the parenchyma of the brain stem (cerebral peduncle, optic lobe, and medulla oblongata) and the cerebral hemisphere. Capillary thrombosis with congestion and hemorrhage was frequently observed within the malacia lesions. Gram-positive cocci were recognized in the blood capillary of malacia lesions. Bacteriologically, Enterococcus durans was isolated from the brain, liver, kidney, lung, and spleen. Detection of gram-positive cocci within the blood vessels in the malacia lesions observed in these cases may more strongly suggest the pathogenic role of E. durans on the malacia lesions in the brain stem and cerebral hemisphere.

Cerebral listeriosis in an adult Freiberger gelding.

Listeria monocytogenes infection, although rare in horses, can lead to septicaemia, gastroenteritis and abortion or stillborn foals. Equine cerebral listeriosis has been reported, but only in newborn animals. This report describes a Freiberger gelding with severe neuronal symptoms of sudden onset. The animal collapsed within 24 h and was humanely killed. Necropsy revealed multiple small brown to reddish foci within the brain stem and pons. Histopathology demonstrated multifocal suppurative meningoencephalitis with microabscesses and occasional intra-lesional, coccoid to rod-shaped, bacteria. These were identified immunohistochemically as Listeria spp. and further specified as L. monocytogenes by a commercial test system based on in-situ hybridization.

Brain-stem listeriosis: a comparison of SPECT and MRI findings.

Listeria monocytogenes, although uncommon as a cause of illness in the general population, can result in serious illness when it affects pregnant women, neonates, the elderly, and immunocompromised individuals. Typically, it is a food-borne organism. This report describes a case of brain-stem listeriosis in a previously healthy 51-year-old woman. The diagnosis was based on clinical findings, the results of cerebrospinal fluid (CSF) analysis, CSF culture, and magnetic resonance imaging (MRI) findings. MRI demonstrated upper brain stem and cerebellar peduncle involvement. In addition, Tc-99m exametazime (HMPAO)-labeled single photon emission computed tomography (SPECT) of the brain revealed bilateral cerebellar hypoperfusion. Antibiotic therapy resulted in partial clinical recovery after 3 weeks. At the end of 6 months, brain-stem findings had nearly resolved. However, although minimal residual findings were observed on MRI at 6 months, bilateral diffuse cerebellar hypoperfusion remained on Tc-99m HMPAO brain SPECT.

Manifestations of Neuro-Behcet's disease. Report of two cases and review of the literature.

Behcet's disease (BD) is a rare condition, more commonly seen in Turkey, the Middle East, the Mediterranean, and the Far Eastern countries. It is a multifaced disease involving the central nervous system (CNS), skin, joints, intestines and blood vessels. CNS involvement occurs in 10-25% of the patients with BD. We report two cases with Neuro-Behcet's disease. Both patients were females and they were in their second decade of life (early forties) when they suffered from relapsing retinitis and uveitis which led to blindness, despite treatment with steroids. They presented with clinical signs of brainstem syndrome, meningoencephalitis and the initial radiological investigations of our patients revealed encephalitic lesions in the brainstem. In the first case, an intracerebral abscess developed 4 years later, and it was evacuated under stereotactic guidance. The most common neuropathologic findings in Neuro-Behcet's disease are focal necrotic lesions in the brain. However, the association of Behcet's disease (BD) with cerebral abscess is very rare. There were only three reported cases of such. Ten years after the initial diagnosis of BD, our second patient suffered from subarachnoid hemorrhage due to a ruptured aneurysm of the superior cerebellar artery, which was then resected successfully. Intracranial aneurysm in patients with Behcet's disease has previously been documented in eight patients. To our knowledge, this is the only case of Neuro-Behcet's disease associated with an aneurysm of the superior cerebellar artery.

Listerial brainstem encephalitis--treatable, but easily missed.

Listerial brainstem encephalitis (LBE) is an uncommon form of listerial central nervous system infection that progresses rapidly and is invariably fatal unless detected and treated early. We report on six adult patients with LBE, of whom five were managed or co-managed by our unit during the period January - June 2012. All presented with a short prodromal illness followed by a combination of brainstem signs, including multiple cranial nerve palsies with emphasis on the lower cranial nerves, ataxia, motor and sensory long-tract signs, a depressed level of consciousness and apnoea. In two cases the diagnosis was delayed with adverse outcomes. LBE may be difficult to diagnose: clinicians may not be aware of this condition, the brainstem location may not be recognised readily, general markers of inflammation such as the erythrocyte sedimentation rate, C-reactive protein level or white cell count may be normal, and the cerebrospinal fluid is typically normal or there are only mild and nonspecific findings. Serological tests are unreliable, and diagnosis is achieved through blood cultures, magnetic resonance imaging and clinical recognition.

The growth of four human and animal enteroviruses in the central nervous systems of mice.

The pattern of CNS infection of our enteroviruses, Coxsackievirus A14, encephalomyocarditis (EMC) virus, GDVII virus, and Vilyuisk virus, was investigated. The regional sites of virus replication were correlated with pathological changes. These viruses were found to replicate and produce similar lesions in selected sites in the mouse CNS during acute infection. Virus antigen and histological lesions were essentially confined to the gray matter, with additional but less direct support for this being provided by virus assay of CNS regions. Virus antigen only could be confidently identified in neurons, and, histologically, neurons were observed in various stages of degeneration. The distribution of the involvement varied in the different infections. For example, EMC virus infection primarily affected the cerebral hemispheres and thalamus, while Coxsackievirus a14 infection predominantly involved the brainstem and spinal cord. GDVII and Vilyuisk virus infections more uniformly involved the entire neuraxis. There was no evidence of virus replication in the olfactory bulbs, leptomeninges, ependyma, choroid plexus, and vascular endothelium. The cerebellum was generally spared, with the cerebellar hemispheres affected only in Coxsackievirus A14 infection. It therefore appears that enteroviruses selectively infect different populations of nerve cells.

Herpes simplex virus (HSV) DNA in microglial nodular brainstem encephalitis.

Thirty-four brains with microglial nodular brain stem encephalitis were retrospectively investigated for herpes simplex virus (HSV) and cytomegalovirus (CMV) by in situ hybridization (ISH) with biotinylated cDNA probes, and by immunocytochemistry with polyclonal and monoclonal antibodies on formalin fixed paraffin embedded serial tissue sections. In 16 cases (47%), HSV DNA was found by ISH in the nuclei of neurons in microglial nodules or in the adjacent parenchyma of the brainstem, and more rarely at various cerebellar and telencephalic sites. None of the 34 cases was labeled for CMV DNA and none revealed HSV or CMV antigens. Ten control brains without microglial nodules were not labeled. This study suggests an HSV etiology for many cases with microglial nodular brainstem encephalitis.