Epstein-Barr virus hemorrhagic meningoencephalitis: case report and review of the literature

Neurologic complications related to Epstein-Barr virus (EBV) in immunocompetent adults are rare and most commonly self-limited. However, severe cases have been previously reported in the literature. We describe a case of meningoencephalitis with frontal bilateral hemorrhage in a non-immunocompromised adult following an EBV infection of the central nervous system confirmed by the presence of EBV-DNA in the cerebrospinal fluid. During the patient’s hospital stay, there was a favorable clinical and radiologic evolution and the patient was discharged asymptomatic. To our knowledge, this is the fourth case of hemorrhagic meningoencephalitis related to EBV and the first one in an immunocompetent patient with a favorable outcome.     

Unusual ocular motor findings in multiple sclerosis

In multiple sclerosis (MS), nystagmus or internuclear ophthalmoplegia (INO) are the usual ocular motor dysfunctions. However, in patients with focal brainstem lesions, other rare manifestations may be observed, such as an isolated ocular motor nerve palsy or complex ocular motor disturbances. We report five MS patients with unusual ocular motor disturbances (bilateral third nerve palsy [n = 2], opsoclonus, Horner's syndrome and one-and-a-half syndrome). We discuss possible correlations between clinical disturbances and MRI abnormalities. Patients were seen in two MS centres. They had a confirmed diagnosis of MS and underwent a brain MRI and a complete neuro-ophthalmological work-up. In one case (opsoclonus), ocular motor manifestations were the first manifestation of MS. In the other four cases they occurred 3 months (Horner syndrome), 6 years and 12 years (bilateral third nerve palsy) and 2 years (one-and-a-half syndrome) after the disease onset, respectively. Four out of five patients were still in a relapsing-remitting form of MS. In the opsoclonus case, there was no evidence of a brainstem lesion. A gadolinium-enhanced lesion (2 cases) or a new T2-weighted lesion located in the brainstem correlated with the clinical presentation. All patients completely or partially recovered after corticosteroid infusions. Our study shows some rare or previously undescribed complex ocular motor symptoms in MS.     

A rare cause of cerebral venous thrombosis: cryptococcal meningoencephalitis

Cryptococcal meningoencephalitis (CM) is a serious central nervous system infection caused by Cryptococcus neoformans, seen mostly in immunocompromised hosts and less in immunocompetent patients. The vast majority of cryptococcosis cases are seen as human immunodeficiency virus infections with advanced immunosuppression. Meningitis and meningoencephalitis are the most common clinical manifestations. Nevertheless, immunocompetent patients with CM are rarely reported. Cerebral venous sinus thrombosis is a rare complication of CM. Here, we report an immunocompetent patient with CM from a non-endemic area, who presented with an acute onset and atypical symptoms associated with cerebral venous thrombosis.     

Cerebral phaeohyphomycosis caused by a dematiaceous scopulariopsis species

This report describes a rapidly fatal case of cerebral phaeohyphomycosis in a 33-year-old immunocompetent male. The infection presented as a single large lesion in the deep white matter of one temporal lobe, which was then removed surgically. Histologic features observed in the lobectomy specimen were characterized by perivascular sleeves of mononuclear cells accompanied by hemorrhages. These were reminiscent of acute hemorrhagic leukoencephalitis except for the presence of rare fungal organisms and sparse multinucleated giant cells similar to those occurring in AIDS. During the four days following surgery, a large focus of cerebritis with massive invasion of fungi developed in each centrum semiovale around the ventriculostomy sites. Fungal culture of the brain obtained at autopsy grew an organism consistent with a Scopulariopsis species.     

Bilateral neuroretinitis associated with mumps

BACKGROUND: Involvement of the optic nerve is a rare complication of mumps infection. OBJECTIVES: To report a case of bilateral neuroretinitis complicating a mumps infection and to review 5 previously reported cases. DESIGN: Case report and literature review. SETTING: Tertiary hospital. PATIENT: A 7-year-old girl had sudden-onset blindness due to bilateral neuroretinitis. Approximately 3 weeks prior to the initial examination, she developed a self-limited febrile illness with parotid swelling and subsequent meningoencephalitis. RESULTS: Mumps was determined to be the underlying cause of the meningoencephalitis and bilateral optic neuritis because of the exposure history in this nonvaccinated child, the typical clinical signs and symptoms, and the positive serologic test results. Recovery of visual function was gradual but nearly complete. CONCLUSIONS: Physicians should be aware that optic nerve involvement may be a manifestation of mumps infection. The delayed onset of optic neuritis, the bilateral involvement, and the near complete recovery suggest an immune-mediated pathogenesis.     

Chikungunya infection presenting as mild encephalitis with a reversible lesion in the splenium: a case report

Chikungunya fever is an Aedes mosquito-transmitted infection caused by chikungunya virus, an RNA virus in the family Togaviridae. The disease is characteristically manifested as fever, arthralgia, and/or rash. Various neurological manifestations like meningoencephalitis, myelitis, and myeloneuropathy have been mentioned in various reports. We present a rare case of chikungunya fever presenting with mild encephalitis with a reversible lesion of the splenium (MERS), which showed complete clinical and radiological recovery.     

Subacute brachial diplegia associated with West Nile virus myelitis

Introduction: Brachial diplegia is a clinical term used to describe weakness restricted to the upper extremities. We report a case of brachial diplegia associated with West Nile virus infection. Methods: A 48‐year‐old man developed severe painless bilateral upper extremity weakness within a few weeks of a flu‐like illness. Results: Clinical examination revealed marked periscapular, shoulder girdle, and humeral muscle atrophy and bilateral scapular winging, with near symmetrical bilateral hypotonic upper extremity weakness. This was associated with clinical signs of an encephalomyelopathy without cognitive or sensory deficits. Electrophysiological studies demonstrated a subacute disorder of motor neurons, their axons or both, involving the cervical and thoracic myotomes, with ongoing denervation. Serological studies confirmed recent West Nile virus (WNV) infection. Gradual improvement occurred following conservative supportive therapies. Conclusions: Progressive brachial diplegia is a rare neuromuscular presentation of WNV neuroinvasive disease. This case report adds to the clinical spectrum of WNV‐induced neurologic sequelae. Muscle Nerve 45: 900‐904, 2012     

Clinical spectrum of muscle weakness in human West Nile virus infection

Poliomyelitis has recently been identified as a cause of muscle weakness in patients with West Nile virus (WNV) infection. However, the clinical spectrum of WNV-associated weakness has not been described. We reviewed data on 13 patients with WNV infection. Patients with muscle weakness were classified into one of three distinct groups based on clinical features. Group 1 comprised five patients who developed acute flaccid paralysis, four with meningoencephalitis and one without fever or other signs of infection. Paralysis was asymmetric, and involved from one to four limbs in individual patients. Electrodiagnostic studies confirmed involvement of anterior horn cells or motor axons. Group 2 involved two patients without meningoencephalitis who developed severe but reversible muscle weakness that recovered completely within weeks. Muscle weakness involved both lower limbs in one patient and one upper limb in the other. Group 3 consisted of two patients who experienced subjective weakness and disabling fatigue, but had no objective muscle weakness on examination. In addition to the three distinct groups, two other patients developed exaggerated weakness in the distribution of preexisting lower motor neuron dysfunction. We conclude that the clinical spectrum of WNV-associated muscle weakness ranges from acute flaccid paralysis, with or without fever or meningoencephalitis, to disabling fatigue. Also, preexisting dysfunction may predispose anterior horn cells to additional injury from WNV. Awareness of this spectrum will help to avoid erroneous diagnoses and inappropriate treatment.     

Coxsackie B meningoencephalitis in a patient with acquired immunodeficiency syndrome and a multiple sclerosis-like illness

Both Coxsackie infection and multiple sclerosis (MS) are rare in human immunodeficiency virus (HIV) infection. We report a 35-year-old woman with known HIV infection of 12 years’ duration and a clinical illness of 4 years’ duration consistent with MS. The latter was characterized by optic neuritis, bilateral abducens palsies, recurrent Bell’s palsy, hemiparesis, and ataxia coupled with white matter abnormalities on magnetic resonance imaging (MRI). Autopsy revealed Coxsackie B meningoencephalitis; no other infectious disease were detected and no histopathological features of MS were evident. We suggest that the relapsing-remitting neurological disease in this patient was the consequence of Coxsackie B meningoencephalitis. This is the first case report, to the best of our knowledge, of an enteroviral meningoencephalitis complicating human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS).     

Electrodiagnostic features of acute paralytic poliomyelitis associated with West Nile virus infection

West Nile virus (WNV) infection is a potentially fatal disease, with meningoencephalitis being its most common neurological manifestation. Guillain-Barré syndrome (GBS) has also been described, but acute paralytic poliomyelitis has only recently been recognized. We report the clinical and electrodiagnostic findings of five patients with WNV infection, who presented with acute paralytic poliomyelitis. Three patients manifested focal asymmetrical weakness, and two had rapid ascending quadriplegia mimicking GBS. Electrodiagnostic studies during the acute illness showed normal sensory nerve action potentials, compound motor action potentials of normal or reduced amplitude, and no slowing of nerve conduction velocities. Depending on the timing of the examination, fibrillation potentials were widespread, including in those with focal weakness. Cervical magnetic resonance imaging in one patient showed abnormal T2-weighted signals in the spinal cord gray matter. On follow-up, signs of clinical improvement were seen in one patient, whereas two remained quadriplegic and ventilator-dependent 5 months after the onset. This report highlights the value of the electrodiagnostic studies in the diagnosis and prognosis of focal or generalized weakness due to acute paralytic poliomyelitis associated with WNV infection.     

Neuronal and glial cerebrospinal fluid protein biomarkers are elevated after West Nile Virus infection

Neurotrophic West Nile virus (WNV) disease is a severe arbovirus infection in which neuronal loss is the likely anatomical substrate for the high morbidity and mortality. We investigated whether cerebrospinal fluid (CSF) protein biomarkers were elevated in vivo and related to disease severity in patients with WNV infection. This exploratory study included 114 patients (24 acute WNV, 77 noninflammatory controls, six peripheral neuropathies, seven aseptic meningoencephalitis). CSF levels of neuronal (neurofilaments, NfH‐SMI35) and glial (glial fibrillary acidic protein, GFAP, S100B) biomarkers were measured by enzyme‐linked immunosorbent assay (ELISA). Immunocytochemistry was performed in two fatal WNV cases. A significant proportion of patients with WNV had pathological CSF levels for NfH‐SMI35 (58%, median concentration 1.01 ng/mL), GFAP (58%, 10 pg/mL), and S100B (90%, 1.29 ng/mL). The results were consistent with postmortem evidence for neuronal death and astrogliosis. Surprisingly, CSF protein biomarker levels were also found to be pathological in a considerable proportion of patients who presented with WNV fever only (100% for GFAP and S100B and 43% for NfH‐SMI35). Elevated CSF protein biomarker levels are suggestive of neuronal death and glial pathology in human WNV infection. The results indicate the presence of neuroinvasive disease across the spectrum of WNV disease, including WNV fever. Muscle Nerve, 2010     

Terson syndrome with bilateral optic nerve sheath hemorrhage.

A 53-year-old man presented with an acute headache and mental status changes due to rupture of an anterior choroidal artery aneurysm. A preoperative CT scan demonstrated subarachnoid hemorrhage, bilateral optic nerve sheath hemorrhage, and bilateral intraocular hemorrhage. Ophthalmoscopy and B-scan ocular ultrasound disclosed vitreous hemorrhages, features consistent with Terson syndrome. This is the first CT report of Terson syndrome showing bilateral optic nerve sheath hemorrhage.     

Cerebellar venous infarction: case report with clinicopathologic correlation

A diabetic man developed a severe hyperosmolar state resulting in coma. CT of the head showed bilateral cerebellar hemorrhages. Despite medical treatment, he deteriorated and died. At autopsy, the straight sinus was thrombosed. There were bilateral, hemorrhagic, cerebellar venous infarctions. This condition is rare because of abundant collateral venous drainage.     

Meningoencephalitis or meningitis in relapsing polychondritis: four case reports and a literature review

Relapsing polychondritis (RP) is a rare autoimmune disease that affects cartilage throughout the body, causing episodic and progressive inflammation. Although rare, RP has diverse acute and subacute nervous system complications, which may sometimes precede systemic manifestations. Here, we report four patients with RP who presented with meningoencephalitis or meningitis without infectious aetiology. In addition, we review the literature for this disease with regard to clinical manifestations and treatment options.     

Vision loss,gaze palsy,and nystagmus in a patient with leukemia

A 22-year old male with a history of B-cell acute lymphoblastic leukemia with recent bone marrow transplantation and on immunosuppressive therapy presented with painless, subacute vision loss of two weeks duration. He exhibited a horizontal gaze palsy, nystagmus, and mildly swollen and hyperemic optic discs with peripapillary flame hemorrhage on retinal exam. He had bilateral cecocentral scotomas on visual field exam, and MRI of his brain/orbits demonstrated hyperintensities in the hypothalamus, periaqueductal gray, and dorsal rostral medullary regions. After continued progression of symptoms despite discontinuation of the patient’s tacrolimus, an empiric trial of IV thiamine treatment was started before the patient’s lab vitamin levels were available, given strong clinical suspicion for a nutritional etiology. The patient’s clinical presentation improved dramatically, and he achieved a final visual acuity of 20/20, full visual fields bilaterally, and resolution of nystagmus. A final diagnosis of Wernicke’s encephalopathy was supported by his clinical course, imaging findings, and further confirmation with blood thiamine levels. This case presents unique ocular manifestations of Wernicke’s encephalopathy and highlights the importance of early diagnosis in this potentially reversible condition.     

Ocular flutter following Zika virus infection

Zika virus (ZIKV) is an emerging flavivirus which has been linked to a number of neurologic manifestations such as Guillain-Barré syndrome (GBS), transverse myelitis, and meningo-encephalitis. Ophthalmologic manifestations are increasingly being reported; however, ocular dyskinesias have not been described in this context to date. Herein, we report a case of a 22-year-old female who presented with ocular flutter and associated Guillain-Barré syndrome following acute ZIKV infection. We speculate that although such symptoms may have originated from a direct viral insult, a post-infectious autoimmune mechanism may not be excluded. Physicians should include ZIKV as well as other flaviviruses in their diagnostic workup for all patients with ocular flutter/opsoclonus, after excluding other non-infectious causes of central nervous system pathology. To the best of our knowledge, this is the first report on the association of ocular flutter, GBS, and ZIKV infection.     

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.     

The Floccular Syndrome: Dynamic Changes in Eye Movements and Vestibulo-ocular Reflex in Isolated Infarction of the Cerebellar Flocculus

The cerebellar flocculus is a critical structure involved in the control of eye movements. Both static and dynamic abnormalities of the vestibulo-ocular reflex (VOR) have been described in animals with experimental lesions of the flocculus/paraflocculus complex. In humans, lesions restricted to the flocculus are rare so they can become an exceptional model to contrast with the clinical features in experimental animals or in patients with more generalized cerebellar diseases. Here, we examined a 67-year-old patient with an acute vestibular syndrome due to an isolated infarct of the right flocculus. We evaluated him multiple times over 6 months—to follow the changes in eye movements and vestibular function—with caloric testing, video-oculography and head-impulse testing, and the anatomical changes on imaging. Acutely, he had an ipsilateral-beating spontaneous nystagmus, bilateral gaze-evoked nystagmus, borderline impaired smooth pursuit, and a complete contraversive ocular tilt reaction. The VOR gain was reduced for head impulses directed contralateral to the lesion, and there was also an ipsilesional caloric weakness. All abnormalities progressively improved at follow-up visits but with a considerable reduction in volume of the affected flocculus on imaging. The vestibular and ocular motor findings, qualitatively similar to a previously reported patient, further clarify the “acute floccular syndrome” in humans. We also add new information about the pattern of recovery from such a lesion with corresponding changes in the size of the affected flocculus on imaging.     

West Nile virus meningoencephalitis

Since its first appearance in the US in 1999, West Nile virus (WNV) has emerged as the most common cause of epidemic meningoencephalitis in North America. In the 6 years following the 1999 outbreak, the geographic range and burden of the disease in birds, mosquitoes and humans has greatly expanded to include the 48 contiguous US and 7 Canadian provinces, as well as Mexico, the Caribbean islands and Colombia. WNV has shown an increasing propensity for neuroinvasive disease over the past decade, with varied presentations including meningitis, encephalitis and acute flaccid paralysis. Although neuroinvasive disease occurs in less than 1% of infected individuals, it is associated with high mortality. From 1999-2005, more than 8,000 cases of neuroinvasive WNV disease were reported in the US, resulting in over 780 deaths. In this review, we discuss epidemiology, risk factors, clinical features, diagnosis and prognosis of WNV meningoencephalitis, along with potential treatments.     

Magnetic resonance imaging of pachymeningeal enhancement in Vogt-Koyanagi-Harada disease

Vogt-Koyanagi-Harada (VKH) disease is a systemic disease consisting of bilateral granulomatous panuveitis combined with cutaneous and neurologic manifestations. However, there have been few reports of brain magnetic resonance imaging (MRI) in VKH disease. A 54-year-old Korean woman presented with severe periorbital pain, blurred vision and meningismus. Ophthalmologic examination disclosed bilateral optic disc edema with peripapillary nerve fiber hemorrhages. Lumbar puncture revealed monocytic pleocytosis. After a diagnosis of VKH disease was made, the patient was treated with high-dose corticosteroid. Brain MRI showed diffusely thickened posterior ocular walls with retinal detachment and perineural infiltrative changes along the optic nerves and adjacent pachymeningeal enhancement of the anterior temporal lobes bilaterally. We report a case of VKH disease with panuveitis and meningeal involvement of the anterior temporal lobe detected by brain MRI.