Primary angiitis of central nervous system - A challenging diagnosis.

Primary angiitis of the central nervous system is a rare disease characterized by vasculitis of the central nervous system without any systemic involvement. This review aims to provide an insight into the existing stagnancies in the diagnostic approach and management of this disease. The clinical presentation is typically nonspecific, ranging from headaches, altered sensorium, and seizures to recurrent ischemic strokes. The definitive diagnosis can only be ascertained by histopathological studies of tissue obtained from a brain biopsy. While angiography can provide clues to diagnosis, it has often been normal, even in biopsy-proven cases. Primary angiitis of the central nervous system continues to be a diagnostic challenge as little progress has been made over the years in the diagnosis and management strategies. Considering the vast list of mimickers of primary angiitis of the central nervous system and the existence of a significant proportion of imaging-negative and biopsy-negative cases, it becomes imperative to devise universally accepted diagnostic criteria for this disease. Steroids in combination with cyclophosphamide are the agents used to achieve remission. Rituximab can be an alternative. The treatment-related toxicity of cyclophosphamide warrants larger trials for alternative drugs to be studied.

Role of antiparasitic therapy for seizures and resolution of lesions in neurocysticercosis patients: an 8 year randomised study.

Neurocysticercosis is a common cause of acquired seizure disorder in developing countries, including India. The role of antiparasitic (albendazole) therapy for seizure control and resolution of lesions is still controversial due to a lack of adequately controlled studies. The objective of the present study was to evaluate the role of albendazole therapy for neurocysticercosis patients with two or more lesions to achieve seizure-free status and resolution of lesions. This was a randomised controlled study in which patients suffering from neurocysticercosis were prospectively followed up for more than 5 years (from January 1997 to January 2005). Patients were divided into two groups: patients in group A (n=150) were treated with a combination of tapered doses of dexamethasone and albendazole, plus antiepileptic drugs; patients in group B (n=150) were treated with antiepileptic drugs plus a placebo control. Patients were followed up every month for the first 6 months and then at 3-month intervals thereafter up to 5 years. Variables of interest were (i) recurrence of seizures; (ii) encephalopathy (headache/vomiting/altered sensorium); (iii) need for subsequent hospital admission; (iv) death; (v) resolution of lesions on follow-up CT. During the first 6 months and at intervals thereafter, increased seizure frequency and hospital readmissions, and increased incidence of encephalopathy were observed in group A (p=0.01), and two patients in this group died with intractable seizures and encephalopathy. A greater proportion of lesions completely resolved in group B (p=0.05), whereas a greater proportion of lesions calcified in group A (p=0.05). Albendazole plus antiepileptic drugs did not have greater beneficial effects than antiepileptic drugs alone, but may have an adverse effect with respect to seizure control, encephalopathy, recurrent hospital admissions, calcification of lesions and cost of treatment.