ABSTRACT
Autoimmune limbic encephalitis is a rare autoimmune disease characterised by inflammation of the limbic system of the brain. The disease runs a sub-acute course with cognitive impairment, memory loss and seizures. These patients have been underdiagnosed in Pakistan. Here, we present a case of a middle-aged male, who presented to our Department after having multiple visits to different psychiatrists for his symptoms. The patient had been put on multiple psychiatric and antiepileptic medications, but his condition gradually declined. At our Department, he was thoroughly assessed and then diagnosed as limbic encephalitis on the basis of the typical history, positive anti-leucine-rich-glioma-inactivated 1 (LGI1) antibodies and MRI findings. The patient responded to plasmapheresis and immune modulating therapy and is being followed up. Emphasis is made on early diagnosis; and earlier treatment of such cases, as it holds a substantial importance in management and makes a difference in future outcome. Key Words: Autoimmune limbic encephalitis, Voltage-gated potassium channels, Cognitive dysfunction.
Subject(s)
Glioma , Limbic Encephalitis , Autoantibodies , Humans , Intracellular Signaling Peptides and Proteins , Leucine , Limbic Encephalitis/diagnosis , Limbic Encephalitis/drug therapy , Male , Middle AgedABSTRACT
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a form of autoimmune encephalitis. The characteristic clinical features include seizure, psychosis-like symptoms, abnormal movements, and autonomic disturbances. Patients with anti-NMDAR encephalitis can present with various types of movement disorders. Typically, the movement disorders start following intervals of psychiatric and prodromal manifestations in young adults; however, in children, these might be an early presentation of anti-NMDAR encephalitis. The disease is under-recognized and underdiagnosed in Pakistan. Early recognition of the disease is important to commence timely treatment leading to a better prognosis. Here we present a collection of anti-NMDAR encephalitis patients, specifically focussing on the different types of movement disorders and the differences in clinical manifestations between children and adults.
ABSTRACT
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune relapsing demyelinating disorder which often leads to severe disability typically targeting spinal cord, optic nerves, and brainstem. Around 75% of NMOSD patients have serum immunoglobulin-G (IgG) autoantibodies to the aquaporin-4 channel (AQP4-IgG). AQP4-IgG antibodies have a central role in new diagnostic criteria of NMOSD. These antibodies have a critical role in long-term management after the first attack. The prevalence of this disorder is lower than multiple sclerosis in European countries. However, NMO makes a substantial proportion of the demyelinating diseases of the central nervous system in countries like Pakistan, where it can be mistaken for multiple sclerosis. Accurate diagnosis is essential as some of the drugs for multiple sclerosis can potentially worsen NMOSD. We present a case of sequential optic neuritis with positive aquaporin 4 antibodies. We have discussed the history, examination findings, diagnostic workups, and treatment of the patient.