Limbic enchephalitis ppt - Copy

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Case of Acute short term memory loss : 

Dr Yogesh Sharma Dr Vasant Dangra Dr S V Khadilkar Case of Acute short term memory loss

Case history : 

Case history 45 M non DM non HT brought to hospital by relative with history of gradual onset of confusion , memory loss and behavior disturbance since 15 days On detail history wife recalled that he tend to ask same question multiple time ,despite answer given. He do thing repeatedly .He foget where he had left his wallet ,mobile and so many thing in day to day activity. But he can go from home to market alone and come back .During asking him about what he had done in morning He say irrelevantly and produce story that does not match reality. Identification of relative was not problem ,but he forget with whom he had met half and hour before.

Contd ..History : 

Contd ..History Also , wife told that his diet is increased since last 15-20 days and he ask for food many time a day. Another observation made was that he is more talkative than before .Previously he was kind of person ,who did not like to interact or talk unnecessary with people Hesitantly ,wife told that they did not have much physical relation since long , but since few days she noticed that he tried to ask about that and like to talk about sexual activity.He also noticed that he liked to touch her sister and stair toward girls like loffer and certain time make abnormal gestures

MINI MENTAL STATUS EXAMINATION( 15/09/09) : 

MINI MENTAL STATUS EXAMINATION( 15/09/09) 1.Orientation : Date 5 pts Place 5 pts 2.Calculation : Subtracting serial 7s from 100 5 pts 3.Registration : 3 objects 3 pts 4.Recall : 3 objects 0 pts 5.Naming : point to 2 objects and ask their names 2 pts 6.Repeating : say “no ifs,ands,or buts.” 1 pts 7.Verbal commands : Give the pt a plain piece of paper and say “Take this paper in your right hand, fold It in half, and put it on the floor” 2 pts 8.Written commands : Show the pt a piece of paper with “CLOSE YOUR EYES” printed on it. Give one point if the patient closes the eyes 1 pt 9.Writing : Ask the pt to write a sentence 1 pt 10.Drawing : Copy a pair of intersecting pentagons 1 pt

Memory ( 15/09/09) : 

Memory ( 15/09/09) Anterograde verbal memory Name & address : presnt Immediate recall (digit span backward) :not posible after 5 min New learning ability : :no - 4 unrelated words, eg: fun, carrot, ankle, loyalty After 5 mins, ask patient to repeat. - Story reproduction ( 26 items ) Anterograde nonverbal memory : impaired -visual memory : route learning (how to come to hospital), Hiding objects like pen or watch   Retrograde memory : normal famous events ( extrapersonel ) : eg ; Independence day autobiographical ( personal ) : eg ; when got married :normal

Evaluation : 

Evaluation Routine investigation normal Csf study normal EEG bil frontotemporal slowing and spikes Mri brain :

Neoplastic work up : 

Neoplastic work up Tumour marker profile normal HRCT thorax normal USG ABDO-KUB normal Antineuronal antibody negative FDG PET incresed uptake in mesial temporal area s/o limbic enchephalits

. Final diagnosis Limbic enchephalits ,etiology unknown : 

. Final diagnosis Limbic enchephalits ,etiology unknown Treated with prednisolone 1 mg/kg for 14 days f/b tapering dose Reviewed after 15 days

MINI MENTAL STATUS EXAMINATION 15/09/09 01/10/09 : 

MINI MENTAL STATUS EXAMINATION 15/09/09 01/10/09 1.Orientation : Date 5 pts 5pts Place 5 pts 5pts 2.Calculation : Subtracting serial 7s from 100 5 pts 5pts 3.Registration : 3 objects 3 pts 3pts 4.Recall : 3 objects 0 pts 3pts 5.Naming : point to 2 objects and ask their names 2 pts 2pts 6.Repeating : say “no ifs,ands,or buts.” 1 pts 1pts 7.Verbal commands : Give the pt a plain piece of paper and say “Take this paper in your right hand, fold It in half, and put it on the floor” 2 pts 3pts 8.Written commands : Show the pt a piece of paper with “CLOSE YOUR EYES” printed on it. Give one point if the patient closes the eyes 1 pt 1pts 9.Writing : Ask the pt to write a sentence 1 pt 1pts 10.Drawing : Copy a pair of intersecting pentagons 1 pt 1pts

Memory 15/09/09 01/10/09) : 

Memory 15/09/09 01/10/09) Anterograde verbal memory Name & address : presnt Immediate recall :not posible after 5 min recall 3 digit New learning ability : :no recall 2 word - 4 unrelated word - Story reproduction Anterograde nonverbal memor : impaired normal -visual memory   Retrograde memory : normal normal famous events ( extrapersonel ) : autobiographical ( personal ) : :normal normal

Limbic System : 

Limbic System • A functional anatomic system of interconnected cortical and subcortical structures • Area of intimate processing between the hypothalamus and cortical information processing • Named by Paul Broca: limbic means border and it names structures forming a border between hypothalamus and cerebral cortex • Olfactory and limbic system are called the rhinocephalon (smell brain)

Limbic System: Components : 

Limbic System: Components • Hippocampal formation • Entorhinal cortex • Amygdala • Cingulate gyrus and cingulum • Fornix • Mamillary bodies and hypothalamus • Thalamus

The broad functionaldivisions of the limbic system : 

The broad functionaldivisions of the limbic system Rostral limbic system: amygdala, septum, orbitofrontal cortex, anterior insula, and anterior cingulate – Important for emotion Caudal system: Hippocampus, posterior parahippocampal cortex and posterior cingulate – Important for memory and visual-spatial function

Slide 19: 

What do lesions of the amygdalacause? What do lesions of the septalregion cause? • Decreased emotion, social reactivity, affective responses to stimuli, facial learning, discrimination of facial emotions, affective vocalizations • Indiscriminate hypersexuality • Kluver-Bucy syndrome if bilateral • Hypersexuality • Rage • Hyperemotionality • Decreased memory • Stimulation causes: sexual arousal, orgasm, pleasure, euphoria

Limbic encephalitis is a neurological paraneoplastic syndrome often associated with small cell lung cancer andassociated with anti-neuronal antibodies (for example,anti-Hu). Limbic encephalitis is characterised clinically bysubacute cognitive dysfunction with severe memory impairment, seizures, and psychiatric features : 

Limbic encephalitis is a neurological paraneoplastic syndrome often associated with small cell lung cancer andassociated with anti-neuronal antibodies (for example,anti-Hu). Limbic encephalitis is characterised clinically bysubacute cognitive dysfunction with severe memory impairment, seizures, and psychiatric features Gultekin SH, Rosenfeld MR, Voltz R, et al. Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain 2000;123:1481–94.

Paraneoplastic limbic enchephalitis : 

Paraneoplastic limbic enchephalitis

Clinical feature : 

Clinical feature Patients with limbic encephalitis usually present with rapidly progressive short-term memory deficits, psychiatric symptoms, and seizures. long-term course of behavioral alterations and MRI findings in a patient with a limbic enchephalitis show severe anterograde memory loss, frontal executive dysfunction and behavioral alterations Most cases of limbic enchephalitis are paraneoplastic and many time it preced diagnosis of primary malignancy Neurologist. 2007 Sep;13(5):261-71.Limbic encephalitis and variants: classification, diagnosis and treatment. Tüzün E, Dalmau J.

Diagnosis: : 

Diagnosis: After excluding viral and systemic autoimmune disorders, many patients with limbic encephalitis (paraneoplastic or not) have cerebrospinal fluid inflammatory findings, EEG or MRI abnormalities in the temporal lobes, and antineuronal antibodies. These antibodies are directed against 2 broad categories of antigens: (1) intracellular or classic paraneoplastic antigens, including Hu, Ma2, CV2/CRMP5, and amphiphysin among others, and (2) cell membrane antigens, including voltage-gated potassium channels, N-methyl-D-aspartate receptor, and others expressed in the neuropil of hippocampus and cerebellum (pending characterization). Neurologist. 2007 Sep;13(5):261-71 Limbic encephalitis and variants: classification, diagnosis and treatment. Tüzün E, Dalmau J.

Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients.Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. : 

Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients.Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J. The diagnosis of PLE required neuropathological examination or the presence of the four following criteria: (i) a compatible clinical picture; (ii) an interval of <4 years between the development of neurological symptoms and tumour diagnosis; (iii) exclusion of other neuro-oncological complications; and (iv) at least one of the following: CSF with inflammatory changes but negative cytology; MRI demonstrating temporal lobe abnormalities; EEG showing epileptic activity in the temporal lobes

Treatment: : 

Treatment: Treatment of the tumour appeared to have more effect on the neurological outcome than the use of immune modulation Whereas the disorders related to the first category of antibodies associate with cancer (lung, testis and other), prominent brain infiltrates of cytotoxic T-cells, and limited response to treatment, The disorders related to the second category of antibodies associate less frequently with cancer (thymoma, teratoma), seem to be antibody-mediated, and respond significantly better to immunotherapy Neurologist. 2007 Sep;13(5):261-71.Limbic encephalitis and variants: classification, diagnosis and treatment. Tüzün E, Dalmau J

Prognosticating factor : 

Prognosticating factor Patients with anti-Hu antibodies usually had small-cell lung cancer , multifocal neurological symptoms and a poor neurological outcome. Patients with anti-Ta (also called anti-Ma2) antibodies usually are young men with testicular tumours , frequent hypothalamic involvement and a poor neurological outcome. In the group of patients without anti-Hu or anti-Ta antibodies, the tumour distribution was diverse, with cancer of the lung the most common. Relatively showed good neurological improvement. . Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients.Gultekin SH, Rosenfeld MR, Voltz R, Eichen J, Posner JB, Dalmau J.

[Non-paraneoplastic limbic encephalitis revealed by anterograde amnesia]Lang PO, Sellal F. : 

[Non-paraneoplastic limbic encephalitis revealed by anterograde amnesia]Lang PO, Sellal F. INTRODUCTION: Limbic encephalitis is a syndrome, most commonly paraneoplastic, related to an often undiagnosed cancer of unpredictable prognosis. Neurological symptoms are progressive for a few weeks before stabilizing. CASE: We report a case of limbic encephalitis in a 56-year-old man, revealed by anterograde amnesia. The diagnosis was suggested after magnetic resonance imaging (MRI) showed bilateral hippocampal lesions, with signals that were hypointense in IR sequences and hyperintense in FLAIR. The non-neoplastic causation was suggested by an array of clinical, laboratory, imaging, and therapeutic arguments. After five years of follow-up, no neoplasia has been found. DISCUSSION: This case provides the opportunity for a comparison of the radiologic, imaging, and neurologic findings related to bilateral lesions of the hippocampus

Atypical presentation : 

Atypical presentation J Neurol Neurosurg Psychiatry. 2003 Jan;74(1):110-2. Limbic encephalitis presenting with topographical disorientation and amnesia.Hirayama K, Taguchi Y, Sato M, Tsukamoto T. J Neurooncol. 2005 Apr;72(2):195-8. LinksFocal paraneoplastic limbic encephalitis presenting as orgasmic epilepsy.Fadul CE, Stommel EW, Dragnev KH, Eskey CJ, Dalmau JO. Paraneoplastic limbic encephalitis and possible narcolepsy in a patient with testicular cancer: Case study Joseph C. Landolfi1 and Mangala Nadkarni : Brain. 2005 Aug;128(Pt 8):1745-6. Treatment-responsive limbic encephalitis identified by neuropil antibodies: MRI and PET correlates.Ances BM, Vitaliani R, Taylor RA, Liebeskind DS Neurology. 2007 Jul 10;69(2):156-65. Post-transplant acute limbic encephalitis: clinical features and relationship to HHV6. Seeley WW, Marty FM, Holmes TM, Upchurch K, Soiffer RJ, Antin JH, Baden LR, Bromfield EB.

Slide 33: 

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