Encephalitis lethargica (von Economo disease)
Updates to Study Attributes
-BilateralBilateral and symmetric high signal lesions in mesencephal (including substantia nigra), hypothalamus, striatum and internal internal temporal lobe.
RespectSparing of the insular and basi frontal regions.
-Enhancement Enhancement of the basal ganglia, anterior commissure, striatum, and right medial temporal region.
-NormalNormal DWI.
-LipidsLipids peak, low ratio NAA/ creatine.
Updates to Quizquestion Attributes
Updates to Case Attributes
Association of clinic pharyngitis followed by sleep disorder, basalbasal ganglia signs (particularly parkinsonismParkinsonism), neuropsychiatric sequelae and MRI swelling in the basal ganglia and the substantia nigra is very evocativesuggestive of Encephalitis lethargica. It is a rare and mysterious disease, first decribeddescribed by von Economo in 1916 during an epidemic, affecting 500,000 people worldwide in 1916-1927. There has been no further epidemic of EL reported since the 1920s although sporadic cases have been described.
Alternative diagnoses were excluded with the help of multiple immunohistochemistry test in LCR.
Infectious Infectious/inflammatory laboratory and serological workup were normal. Furthermore a scan of the chest, abdomen, and pelvis was normal.
Intrathecal Intrathecal oligoclonal bands (OCB) were found.
RespectSparing of the insular and basifrontal regions rule out confounding diagnosis.
Steroid and immunomodulating therapy were administered with a beneficial effect. There were only mild neuropsychiatric symptoms and Parkinsonism sequelae.
Differential diagnosis:
-<p>Association of clinic pharyngitis followed by sleep disorder, basal ganglia signs (particularly parkinsonism), neuropsychiatric sequelae and MRI swelling in the basal ganglia and the substantia nigra is very evocative of <strong>Encephalitis lethargica</strong>. It is a rare and mysterious disease, first decribed by <strong>von Economo</strong> in 1916 during an epidemic, affecting 500,000 people worldwide in 1916-1927. There has been no further epidemic of EL reported since the 1920s although sporadic cases have been described.</p><p>Alternative diagnoses were excluded with the help of multiple immunohistochemistry test in LCR. </p><p>Infectious/inflammatory laboratory and serological workup were normal. Furthermore a scan of the chest, abdomen, and pelvis was normal.</p><p>Intrathecal oligoclonal bands (OCB) were found.</p><p>Respect of the insular and basifrontal regions rule out confounding diagnosis.</p><p>Steroid and immunomodulating therapy were administered with a beneficial effect. There were only mild neuropsychiatric symptoms and Parkinsonism sequelae.</p><p>Differential diagnosis:</p><ul>- +<p>Association of clinic pharyngitis followed by sleep disorder, basal ganglia signs (particularly Parkinsonism), neuropsychiatric sequelae and MRI swelling in the basal ganglia and the substantia nigra is very suggestive of <strong>Encephalitis lethargica</strong>. It is a rare disease, first described by <strong>von Economo</strong> in 1916 during an epidemic, affecting 500,000 people worldwide in 1916-1927. There has been no further epidemic of EL reported since the 1920s although sporadic cases have been described.</p><p>Alternative diagnoses were excluded with the help of multiple immunohistochemistry test in LCR. Infectious/inflammatory laboratory and serological workup were normal. Furthermore a scan of the chest, abdomen, and pelvis was normal. Intrathecal oligoclonal bands (OCB) were found.</p><p>Sparing of the insular and basifrontal regions rule out confounding diagnosis.</p><p>Steroid and immunomodulating therapy were administered with a beneficial effect. There were only mild neuropsychiatric symptoms and Parkinsonism sequelae.</p><p>Differential diagnosis:</p><ul>
-<li><a href="/articles/herpes-simplex-encephalitis">Herpes simplex encephalitis</a></li>-<li><a href="/articles/leptospirosis">Leptospirosis</a></li>- +<li><a href="/articles/herpes-simplex-encephalitis">herpes simplex encephalitis</a></li>
- +<li><a href="/articles/leptospirosis">leptospirosis</a></li>
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