Seminar At Andheri, Mumbai on 26th June 2011
Speech problems. If the speech areas are affected, patients may be unable to translate their thoughts into words (expressive dysphasia) and have difficulty understanding what others say to them
(receptive dysphasia).
Cognitive deficits Patients can develop memory problems, intellectual impairment, and other neuropsychological deficits.
Causes :
There is convincing evidence that in most patients
RE is an autoimmune disorder. Many patients have antibodies in their blood that bind to nerve cells and which are capable of damaging the brain. Of particular interest is an antibody that binds to an important nerve protein called the type-3 glutamate receptor (GluR3). In addition, activated immune cells called T cells that are toxic to nerve cells are found in inflammatory brain tissue in biopsies from RE patients.
In most patients, it is not clear what triggers the abnormal immune response, although sometimes RE has followed an otherwise minor bacterial or viral infection, or head injury.
Diagnosis :
A serious disease needs intensive investigation. The tests are designed to confirm RE and to exclude other conditions. Diseases that can mimic RE include viral and toxoplasma encephalitis, autoimmune disorders such as vasculitis, and tumours.
The most useful investigations are
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Brain scans. MR, SPECT, and if available PET scans are useful.
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Blood test:. These include assays for a range of antibodies and tests to exclude infection.
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Lumbar puncture. Spinal fluid is examined for evidence of inflammation and infection.
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Brain biopsy. This is needed to confirm the diagnosis.
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Electroencephalogram (EEG). This records the electrical activity of the brain and is useful in characterising the type of seizures the patient has.
Treatment : Because in RE the seizures often do not improve with anti-epilepsy drugs and the disease only ends with destruction of the affected cerebral hemisphere, surgical removal of large areas, sometimes all of the hemisphere, became a standard treatment. However, the research evidence of autoimmune abnormalities in many patients, and the clear need for effective medical therapy early in the disease to prevent progression, helped prompt trials of immune therapy.
Case-Taking :
Investigations :
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07.12.2004 : Fundal Examination :
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Bilateral Congenital Cataract.
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06.01.2005 : CT Scan of Brain :
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Mild prominence of the ventricular system is noted.
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Confluent, ill defined, hypodensities are noted in the bilateral para-ventricular deep white matter.
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Bilateral maxillary, ethmoid and sphenoid sinusitis is noted.
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Sclerotic bilateral mastoids are noted.
09.11.2006 : MRI Scan of Brain :
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Diffuse T2 – Hyperintense signal in almost the entire (Lt) cerebral hemisphere. Predominantly affecting the grey matter with resultant swollen gyri. There is extension into occipito-temporo white matter & splenium of corpus callosum on (Lt) side.
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Findings are S/o Rasmussen’s Encephalitis.
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Generalised tonic-clonic seizures since age of 1½ year.
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Increased frequency since 8-9 months.
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Now multiple seizures daily – remaining for ½ min to 1 min.
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H/o Unconsciousness with seizures, previously.
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During attack : falls on front side mostly on forehead.
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Jerky movements of (Lt) hand and (Lt) leg, body turning to (Lt) side.
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Sometimes fast rolling of eyeballs.
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Since last 8-9 months, unable to speak due to these recurrent seizures.
09.11.2006 : Liver Function Test :