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.

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.
Tuberculosis, Meningeal ; Cytokines

No abstract available.
Stroke* ; Tuberculoma*

No abstract available.
Carcinoid Tumor* ; Cerebellar Ataxia*

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.
Animals ; Central Nervous System ; Diagnosis ; Early Diagnosis ; Humans ; Lymphoma ; Lymphoma, Non-Hodgkin ; Magnetic Resonance Imaging ; Male ; Marek Disease* ; Mononeuropathies ; Neuroimaging ; Peripheral Nervous System ; Peripheral Nervous System Diseases ; Positron-Emission Tomography

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.
Animals ; Central Nervous System ; Diagnosis ; Early Diagnosis ; Humans ; Lymphoma ; Lymphoma, Non-Hodgkin ; Magnetic Resonance Imaging ; Male ; Marek Disease* ; Mononeuropathies ; Neuroimaging ; Peripheral Nervous System ; Peripheral Nervous System Diseases ; Positron-Emission Tomography