Chikungunya Neurological Manifestations: A Systematic Literature Review

Although the most common Chikungunya (do not capitalize the disease unless it is named after a proper noun such as Zika, Ebola or Carrion’s Disease) manifestations are osteoarticular, those which bring the most morbidity and mortality are neurological, where thorough mapping through studies with a methodological outline have not yet been well structured. Therefore, the objective was to review the literature to identify neurological manifestations of CHIKV. We used the Virtual Health Library (VHL) and PubMed with the following descriptors: #1 “Chikungunya” [MeSH]; #2 “neurological manifestations” [MeSH] and their equivalents in the Portuguese language, selecting literature published between July 2007 to January 2018. From the 180 studies that were found, 30 were selected. Findings were divided into two subcategories: “Chikungunya: Typical Neurological Manifestations” and “Chikungunya: Severe Neurological Manifestations”. The studies show that headaches were characterized as the most common symptom in adult patients affected by CHIKV, followed by meningeal involvement. Meningeal involvement is also a more serious clinical scenario associated with encephalitis, convulsions, polyneuropathies such as Guillain-Barré syndrome and death. CHIKV is a public health problem for many reasons including its chronic potential complications. Given the neurological symptoms, this disease is concerning in age extremes, for patients with comorbidities and for patients with more than one viral infection by arboviruses, in whom the most severe neurological manifestations are more common.

Sporadic supratentorial hemangioblastoma with meningeal affection: A case report and literature review

Background: Hemangioblastomas are vascular tumors, of benign behavior, that originate in the central nervous system. Supratentorial hemangioblastomas are extremely rare and are generally associated with Von Hippel-Lindau disease (VHL). The involvement of structures by contiguity, such as blood vessels or meninges, is something exceptional. There are few references in the literature of supratentorial hemangioblastomas with meningeal involvement and most of them are described in reports or small case series. Methods: We performed a systematic review of the literature to observe the characteristics of patients with supratentorial hemangioblastomas with meningeal involvement. In addition, we present the case of a 43-year-old male patient with a sporadic supratentorial hemangioblastoma with both, meningeal and vascular involvement that recurred years after treatment. Results: The patients presented supratentorial tumors with meningeal involvement, we had a 1.2:1 ratio male-female distribution. The mean age was 50 years. Most tumors were located in the cerebral hemispheres, the lobe affected most frequently was the frontal lobe. About 67% of the cases were sporadic and only 21% were related to VHL disease. There were three cases of recurrence after surgery. Conclusion: Supratentorial hemangioblastomas are extremely rare lesions. It is rare for supratentorial hemangioblastomas to invade adjacent structures such as blood vessels or meninges, however, when this happens, it is recommended a preoperative vascular imaging study, especially in parasagittal hemangioblastomas where superior longitudinal sinus may be involved. In these cases, en bloc surgical resection is difficult and the probability of recurrence is higher. Although clinical trials and studies with a greater casuistry are necessary to establish guidelines for the management of supratentorial hemangioblastomas, nowadays, contribution of new cases is useful for understanding this pathology.

Recurrent Nasal-Type Extranodal Natural Killer/T-Cell Lymphoma with Meningeal Involvement

Extranodal natural killer/T-cell lymphoma nasal type (NNKTL) is a type of non-Hodgkin’s lymphoma that has been associated with Epstein–Barr virus (EBV). It has an aggressive behavior, known for predilection to metastasize to different organs. Central nervous system (CNS) spread from a primary location has been reported. Different modalities of treatment such as chemotherapy and radiation therapy have been employed in the management of this disease. Severe toxicities of currently available treatment have made clinicians seek more targeted therapies using molecular profiling. We present a 44-year-old Hispanic patient who was diagnosed with an early-stage NNKTL and treated with the modified SMILE regimen for 6 cycles. His EBV DNA PCR turned undetectable and remained so throughout the treatment. He sustained complete right vision loss due to right optic nerve invasion by the tumor, leading to prophylactic intravitreal methotrexate to the contralateral eye. The patient achieved good response with minimal residual disease. He was supposed to start radiation as a sequential therapy. However, the acute development of severe headache and confusion lead to a complete workup showing leptomeningeal spread. He eventually succumbed to the disease.

Sign of the unilateral “Coca-Cola Bottle”: in addition to thyroid disease

Background: The “Coca-Cola Bottle Sign” is a classic sign of thyroid diseases, especially Graves’ disease, with the appearance of eye orbit muscles edema seen by Magnetic Resonance Imaging. The belly of the muscle increases in thickness, giving the characteristic appearance. Despite being classically associated with this etiology, the finding may be present in other diseases, especially infiltrative ones. Objectives: To demonstrate how this radiological signal can suggest other etiologies, when atypical. Methods: Case report of a patient with an image finding suggestive of “CocaCola Bottle Sign”. Results: Patient, 71 years old, with Breast Cancer and Hepatic Metastasis, using Anastrazole. Osmophobia started and after 3 months, reduced visual acuity in the right eye, evolving in 20 days to amaurosis in the right eye, dizzying, and loss of visual acuity in the left eye. Upon examination, he had a missing direct pupillary reflex in the right eye and only light perception, and counting fingers in the left eye; paresis of the Superior Rectus, Medial, and Lower Oblique muscles of the Left Eye, with paresis maintained in the forced duction test. On ophthalmoscopy, he had atrophy of the retinal pigment epithelium in the bilateral periphery, without Papilledema. Metabolic screening did not show any relevant changes. In the Magnetic Resonance of Orbits, an intraconal nodular image was seen in the right orbital cavity, with perineuritis and extension to the belly of the lateral rectus muscle on this side, as the “Coca-Cola Bottle Sign”. Due to unilateral muscle involvement and signs of meningeal involvement, lumbar puncture with cytopathological examination was requested, being positive for Carcinoma Metastasis. Conclusions: The “Coca-Cola Bottle sign” is a classic sign of Graves’ disease, however, some signs, such as, unilateral and single orbital musculature involvement, may be suggestive of involvement by other etiologies, suggesting the benefit of an early expanded investigation.

Embryonal Rhabdomyosarcoma in Middle Ear: A Rare Case Report

Rhabdomyosarcoma (RMS) in middle ear and mastoid are uncommon. Presentation of RMS in these sites include purulent or blood stained discharge, polypoidal mass and granulation tissue. Clinical diagnosis may be delayed due to similar presentation as Chronic Suppurative Otitis Media (CSOM). Authors herein report a case of six-year-old male patient, who presented with a blood stained discharge from right ear for 15 days. On otoscopic examination, a polypoidal mass in external auditory canal was identified. A history of ruptured right tympanic membrane with occasional discharge for two years had been given. Clinical diagnosis of CSOM with aural polyp had been made. Radiographically, it was a soft tissue lesion with poorly defined margins. There were no bony erosions with minimal collection in right middle ear cavity. On routine histopathological examination diagnosis of round cell tumour had been made. On Immunohistochemistry (IHC), tumour was immunoreactive for desmin and vimentin, while negative for Cytokeratin (CK), Leukocyte Common Antigen (LCA), chromogranin and synaptophysin. Tumour was diagnosed as RMS (Embryonal type). This case report shows importance of early diagnosis of RMS in middle ear, which is often delayed and misdiagnosed as CSOM. Delayed diagnosis may lead to, facial nerve involvement, local meningeal involvement and distant metastasis. Histopathological examination and IHC e.g., desmin positivity are the mainstay of diagnosis.

Early Onset Epilepsy Caused by Low-Grade Epilepsy-Associated Tumors and Focal Meningeal Involvement

Background: Low-grade epilepsy-associated neuroepithelial tumors (LEATs) are a frequent etiology in pediatric patients with epilepsy undergoing surgery. Objective: To identify differences in clinical and post-surgical follow-up between patients with focal meningeal involvement (MI) and those without MI within our cohort of pediatric patients with LEATs. Methods: We retrospectively reviewed all pediatric patients (<18 y) who underwent epilepsy surgery between 2011 and 2017 at our hospital. Cohort inclusion required histological diagnosis of LEATs and post-surgical follow-up of ≥2 y. We subsequently stratified patients according to presence of neuroradiological MI. Results: We identified 37 patients: five with MI and 32 without. Half of patients (19) were drug sensitive at surgery; similar between groups. The group with MI differed mainly for age of epilepsy-onset (0.6 vs. 7.0 y) but not for epilepsy duration (0.9 vs. 1.5 y). Post-surgery radiological follow-up (median 4.0 y; IQR 2.8–5.0 y) did not indicate disease progression. Seizure outcome was excellent in both groups, with 34 patients overall being both drug- and seizure-free. Conclusions: Our study identified a new subgroup of LEATs with focal MI and excellent post-surgical outcome. Moreover, this highlights the effectiveness of early surgery in pediatric LEATs.

Desmoplastic Infantile Astrocytoma and Desmoplastic Infantile Ganglioglioma – not so infantile anymore

DIA and DIG are rare, infantile, supratentorial neoplasms that usually occur in children before 2years of age and are exceedingly rare in older age groups. They appear as hypodense, cystic masseswith solid components showing dural attachment on neuroimaging. They are characterized byreticulin-rich spindle cell stroma containing connective tissue due to meningeal involvement,microscopically. These tumors have potential for misdiagnosis because they contain varyingproportions of neoplastic glial, neuronal and poorly differentiated cells, which causes them to have a“small round blue cell tumor” like appearance, though they have a good prognosis if correctlydiagnosed. The current study report two cases diagnosed at our institution that had very latepresentation with varying complaints which challenged the normally believed dictum of these tumorsbeing entirely infantile.

Familial Oculo-Leptomeningeal Transthyretin Amyloidosis Caused by Leu55Arg Mutation

Hereditary transthyretin amyloidosis (hATTR amyloidosis) is a multisystemic disease usually presenting in a mixed neurological and cardiological phenotype. We present a case of hATTR amyloidosis associated with Leu55Arg mutation causing a form of familial oculo-leptomeningeal amyloidosis. Two brothers and their mother presented with severe autonomic neuropathy, loss of visual acuity and lepto-meningeal involvement. One patient suffered subarachnoid hemorrhage as a possible complication of cerebral involvement. The patients suffered from treatment-refractory weight loss and recurring vitreous opacities. RNA interference-based treatment has led to stabilization of autonomic and peripheral neuropathy but has had no effect on ocular symptoms.

An Association of Varicella Zoster Virus, Facial Palsy, and Meningitis in a Young Immunocompetent Male

Varicella zoster is a secondary infection caused by the virus of chickenpox, after becoming latent in neurons of dorsal root ganglia or trigeminal ganglia. Varicella zoster virus (VZV) can be reactivated years later to produce shingles (zoster), generally in immunocompromised adults to produce neurological deficits and rash. Meningeal involvement of VZV is also reported and can be diagnosed. Herein, we report a 39-year-old immunocompetent male adult with a rare manifestation caused by VZV. The report is an association between facial nerve palsy and a polymerase chain reaction-confirmed VZV meningitis, even without the dermal typical rash. The patient eventually recovered after receiving acyclovir intravenously.