Seronegative panencephalitis complicated by viral encephalomyelitis in a case of Good's syndrome - a neuropathological report.

BACKGROUND: The role of thymus in the immune cascade of the body is still under active clinical scrutiny. Meanwhile, there is an unravelling of myriad presentations of thymoma with effects on various organ systems. These effects arise both due to autoimmunity or a paraneoplastic process secondary to thymoma. However, an occasional patient can be a conundrum and may give no clue regarding the pathogenesis and etiology of its clinical profile. MATERIAL AND METHODS: We present an enigmatic case of a 30-year-old male presenting with superficial fungal infections followed by multiaxial neurological involvement against a background of thymoma. Extensive workup for bacterial, viral, autoimmune and paraneoplastic etiologies were negative. Thymectomy confirmed thymoma (Type AB) but failed to ameliorate the progression of symptoms. He developed recurrent episodes of severe sepsis which remitted with sensitive antibiotics only to reappear again. Immunological profile showed complete absence of peripheral circulating B-cells with reversal of CD4/CD8 ratio, findings compatible with Good's syndrome. Radiological findings showed only progressive brain atrophy without any hyperintensity. He was also treated with intravenous immunoglobulins but failed to respond with the same. RESULTS: Autopsy revealed features of panencephalitis (neuronophagia, neuronal loss, perivascular lymphocytic cuffing and microglial nodules) with virus particles detected ultrastructurally. Although the distinction between seronegative autoimmune encephalitis versus a viral encephalomyelitis often blurs histologically, we speculated viral encephalomyelitis to have happened toward the latter part of his long illness. To our knowledge, this is a case of Good's syndrome presenting with seronegative autoimmune panencephalitis superimposed with a viral infection.

Utility of Imaging of Nigrosome-1 on 3T MRI and Its Comparison with 18F-DOPA PET in the Diagnosis of Idiopathic Parkinson Disease and Atypical Parkinsonism.

BACKGROUND: Loss of nigrosome-1 on 3T and 7T magnetic resonance imaging (MRI) is a recently explored imaging biomarker in the diagnosis of neurodegenerative parkinsonism. OBJECTIVES: This study was undertaken to evaluate the utility of imaging of nigrosome in the diagnosis of neurodegenerative parkinsonism on 3T MRI. METHODS: An institution-based prospective case-control study was conducted at a tertiary care center in North India. 3T venous blood oxygen level-dependent (VenoBOLD) and high-resolution susceptibility-weighted imaging (SWI) imaging sequences in MRI were performed in 100 patients with parkinsonism (56 with idiopathic Parkinson's disease [IPD], 30 with young onset Parkinson's disease [YOPD], 12 with progressive supranuclear palsy, and 2 patients with multiple system atrophy) and 15 controls. Grading of nigrosome was done in both the sequences. Each patient underwent 18F-DOPA positron emission tomography (PET), detailed neurological examination including Hoen and Yahr (H&Y) staging and Movement Disorder Society-Sponsored Revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS) scoring. RESULTS: The diagnostic sensitivity and specificity of the detection of loss of nigrosome-1 on VenoBOLD and SWI sequence at 3T MR imaging were 90% and 66.7% and 94% and 80%, respectively. A weak negative correlation was found between the grading of the nigrosome and clinical parameters (H&Y and UPDRS III). There was no correlation between the side of nigrosome loss and clinical asymmetry. However, nigrosome imaging was not able to differentiate between Parkinson's disease and atypical parkinsonism. CONCLUSIONS: The loss of nigrosome-1 on 3T MRI on SWI and VenoBOLD sequences may serve as a potential imaging marker in the diagnosis of degenerative parkinsonian syndromes. However, it cannot differentiate between idiopathic Parkinson's disease and atypical parkinsonian syndromes.

Subacute sclerosing panencephalitis and brain stem involvement: a rare combination.

Subacute sclerosing panencephalitis (SSPE) is a progressive lethal neurological inflammatory disease due to persistent, wild measles virus infection in the central nervous system that is seen most frequently in children and young adolescents. Atypical presentations are seen in up to 10% of cases. Most frequently and severely affected region in the brain is the parieto-occipital region of the brain. Less commonly involved organs are the cerebellum, basal ganglia and corpus callosum. Brainstem involvement is rare and usually occurs when other areas of brain are involved along with it. Here, we describe an unusual male patient of 15 years age, having SSPE with MRI of brain showing extensive involvement of brainstem with no significant involvement of other cortical structures of the brain. It is very rarely described in SSPE, but one should be vigilant about such involvement of brainstem and cerebellum, and SSPE should not be missed when brainstem hyperintensities are seen in MRI brain with or without other region of the brain to avoid misdiagnosis.

Atypical neurological manifestations of dengue fever: a case series and mini review.

BACKGROUND: In this mini review, we discuss some of the atypical neurological manifestations of dengue virus and attempt to bring them to attention to highlight the neurotropic property of the dengue virus. METHODS: Cases were chosen from retrospective hospital and outpatient records of all patients seropositive for dengue who attended the neurology referral. Seven patients have been chosen as illustrative examples of dengue-associated neurological involvement. We discuss the various central and peripheral nervous system involvement of patients and discuss the relevant findings in them. CONCLUSION: Through this case series, we wish to highlight that the dengue virus can affect the nervous system at various targets, using multiple mechanisms of pathogenesis to generate a plethora of presentations. Hence, it is vital to be aware of its presentations to be able to diagnose dengue and treat it accordingly.

Errors in the Diagnosis of Stroke-Tales of Common Stroke Mimics and Strokes in Hiding.

INTRODUCTION: Stroke mimics are some of the common causes of misdiagnosis of stroke, with an attendant surfeit of finances and resources. AIM: We attempt to discuss some of the common neurological conditions mimicking stroke in the emergency. METHODS: We reviewed the articles in English discussing stroke mimics in the last 5 years of publication. PURPOSE OF THE REVIEW: We discuss the most common causes of stroke mimics encountered in neurological practice. In this short communication, we shall focus on the discussion of the diagnostic pitfalls and clinical clues that will help distinguish mimics from a true stroke.