Rapidly progressive dementia: An eight year (2008-2016) retrospective study.

BACKGROUND AND PURPOSE: Rapidly progressive dementia (RPD) is an emergency in cognitive neurology, defined as cognitive impairment affecting the daily living activities developed over less than 1 year. This study investigated the profile of patients with rapidly progressive dementia at first presentation. METHODS: Retrospective case analysis was done in 187 patients with rapidly progressive dementia who presented to the Postgraduate Institute of Medical Education and Research, Chandigarh, India from January 2008 to August 2016. Patients were divided into three groups: (1) Reversible (treatable) secondary dementia group, (2) Prion dementia group (sporadic Creutzfeldt-Jakob disease), (3) Non-prion Neurodegenerative and vascular dementias (primary neurodegenerative and vascular dementia). Cases presenting with delirium secondary to metabolic, drug induced or septic causes and those with signs of meningitis were excluded. RESULTS: Secondary reversible causes formed the most common cause for RPD with immune mediated encephalitides, neoplastic and infectious disorders as the leading causes. The patients in this series had an younger onset of RPD. Infections presenting with RPD accounted for the most common cause in our series (39%) with SSPE (41%) as the leading cause followed by neurosyphilis (17.9%) and progressive multifocal leukoencephalopathy (15.3%). Immune mediated dementias formed the second most common (18.1%) etiologic cause for RPD. The neurodegenerative dementias were third common cause for RPD in our series. Neoplastic disorders and immune mediated presented early (< 6 months) while neurodegenerative disorders presented later (> 6 months). CONCLUSIONS: Rapidly progressive dementia is an emergency in cognitive neurology with potentially treatable or reversible causes that should be sought for diligently.

Reversible Cerebellar Ataxia Secondary to Carcinoid Tumor

No abstract available.

Tuberculomas Mimicking Posterior-Circulation Stroke

No abstract available.

The Diagnostic Dilemma of Neurolymphomatosis

Neurolymphomatosis (NL) defined as infiltration of the central nervous system or the peripheral nervous system (PNS) by malignant lymphoma cells is a rare clinical entity. However, the increasing use of fluorodeoxyglucose positron-emission tomography (FDG-PET) and magnetic resonance imaging in evaluating PNS disorders is resulting in; this condition being recognized more frequently. Here; we report five NL patients and review the current literature. We report five patients with non-Hodgkin's lymphoma (NHL) and NL, all of whom were men aged 47-69 years. The clinical presentation varied from symmetrical peripheral neuropathy to mononeuropathy. Peripheral neuropathy was the presenting manifestation of a systemic lymphoma in two patients (40%). Neuroimaging as well as whole-body FDG-PET helped in determining the correct diagnosis in all of the patients. NL is an unusual presentation of NHL resulting from infiltration of the PNS by malignant lymphomatous cells. While evaluating peripheral neuropathy, a high degree of suspicion of NL is required since the presenting symptoms vary, conventional radiology has only modest sensitivity, and a pathological diagnosis is often difficult. FDG-PET helps in the early diagnosis and treatment of this condition.

The Diagnostic Dilemma of Neurolymphomatosis

Neurolymphomatosis (NL) defined as infiltration of the central nervous system or the peripheral nervous system (PNS) by malignant lymphoma cells is a rare clinical entity. However, the increasing use of fluorodeoxyglucose positron-emission tomography (FDG-PET) and magnetic resonance imaging in evaluating PNS disorders is resulting in; this condition being recognized more frequently. Here; we report five NL patients and review the current literature. We report five patients with non-Hodgkin's lymphoma (NHL) and NL, all of whom were men aged 47-69 years. The clinical presentation varied from symmetrical peripheral neuropathy to mononeuropathy. Peripheral neuropathy was the presenting manifestation of a systemic lymphoma in two patients (40%). Neuroimaging as well as whole-body FDG-PET helped in determining the correct diagnosis in all of the patients. NL is an unusual presentation of NHL resulting from infiltration of the PNS by malignant lymphomatous cells. While evaluating peripheral neuropathy, a high degree of suspicion of NL is required since the presenting symptoms vary, conventional radiology has only modest sensitivity, and a pathological diagnosis is often difficult. FDG-PET helps in the early diagnosis and treatment of this condition.

Do cytokines correlate with disease activity in tuberculous meningitis?

Background: Tuberculous meningitis (TBM) remains to be one of the most fatal central nervous system infections. The exact pathogenesis of TBM at cellular level remains unclear. In this study, we assessed the cytokine levels in the serum and cerebrospinal fluid (CSF) of TBM patients and determined their correlation with the disease activity. Methods: The levels of tumor necrosis factor-α (TNF-α) and interleukin-1β (IL-1β) were measured by enzyme linked immunosorbant assay (ELISA) in both serum and CSF of 38 patients at baseline, and in 17 of these patients at 1 and 6 month of follow-up. Clinical examination and imaging was performed at baseline and on follow-ups. Results: There was a remarkable rise in the levels of serum and CSF TNF-α and IL-1β in TBM patients as compared to age and sex matched controls (p<0.05). A significant correlation was found between cytokine levels and stages of TBM (p< 0.05). TNF-α levels in both serum and CSF and IL-1β levels in serum were found to be significantly higher in those patients who died than those who survived and had better outcome. TNF-α was higher in patients who developed tuberculoma on follow-up than those who did not (p<0.05). The cytokine levels progressively declined over time but remained detectable till 6 months in most patients. Conclusions: The higher levels of TNF-α and IL-1β were associated with poor outcome in TBM. The higher cytokine levels in patients developing tuberculoma on antituberculous therapy and steroids suggests that these patients may benefit from immunomodulation agents like anti-TNF-α antibody.

Multiplex PCR for rapid diagnosis of tuberculous meningitis.

Rapid and specific diagnosis of tubercular meningitis is of paramount importance to decrease morbidity and mortality. The aim of the study was to evaluate multiplex PCR using protein b, MPB 64, and IS6110 primers directed against M. tuberculosis complex for the diagnosis of tuberculous meningitis (TBM). Multiplex PCR was performed on 18 TBM confirmed cases (culture was positive), 92 clinically suspected TBM cases and 100 non-TBM (control group) patients. Multiplex PCR had a sensitivity of 94.4% for confirmed cases and specificity of 100% for confirmed TBM cases. In 92 clinically diagnosed but unconfirmed TBM cases, multiplex PCR was positive in 84.78% cases. The overall sensitivity of microscopy, culture and multiplex cases were 1.81, 16.73, and 86.63% and specificity was 100, 100, and 100% respectively. Multiplex PCR using protein b, MPB 64, and IS6110 primers has a high sensitivity and specificity in diagnosis of tubercular meningitis.

Pantothenate kinase associated neurodegeneration (PKAN) presenting as a seizure disorder

Pantothenate kinase associated neurodegeneration (PKAN) is an uncommon degenerative disease of the basal ganglia caused by mutations in the PANK2 gene. We describe a 19-years-old man with clinically and radiologically diagnosed case of PKAN, who presented with generalized tonic clonic seizures 2 years preceding other classical extrapyramidal features of the disease. PKAN presenting with seizure has not, to our knowledge, been reported previously.