Encefalitis de Von Economo / Von Economo Encephalitis

Introducción: Constantin von Economo reportó en 1917 múltiples casos de manifestaciones neurológicas secundarias a la pandemia de la gripe española, clasificándolos en tres grandes grupos: forma somnolienta-oftalmopléjica, mutismo y la hipercinética, con secuelas similares a la enfermedad de Parkinson. Objetivo: presentar un caso de reciente aparición de patología rara en Cali, Colombia con manejo adecuado en unidad de cuidados intensivos (UCI). Presentación del caso: paciente de 9 años con disminución de la fuerza en extremidades, disartria y somnolencia, que inició deterioro neurológico progresivo requiriendo manejo en UCI. El equipo multidisciplinario diagnosticó encefalitis letárgica e iniciaron manejo con plasmaféresis e inmunosupresión con mejoría significativa. Discusión y conclusiones: como la prevalencia es escasa, el diagnóstico exige un alto índice de sospecha como la ocurrencia de un cuadro infeccioso previo al inicio de los síntomas, ya que se considera una reacción autoinmune cruzada contra antígenos de la sustancia nigra. En algunos casos hay alteraciones en los estudios imagenológicos o en citoquímico de líquido cefalorraquídeo. El manejo con pulsos de metilprednisolona y filtración de plasma con plasmaféresis brinda mejoría significativa con disminución de las secuelas a futuro.

Introduction: In 1917, Constantin von Economo reported multiple cases of neurological manifestations secondary to the Spanish flu pandemic. He classified them into three main clinical forms: somnolent-ophthalmoplegic, mutism and hyperkinetic, causing sequelae resembling Parkinson ́s disease. Objective: to present a case of a recent appearance rare disease entity, in Cali Colombia, receiving appropriate management in the Intensive Care Unit (ICU). Case presentation: 9-year-old patient presenting with limb muscle weakness, dysarthria and somnolence, evidencing progressive neurological deterioration requiring admission to the ICU for management. A diagnosis of encephalitis lethargica (EL) was made by the attending multidisciplinary team and management with plasmapheresis and immunosuppression was started, obtaining significant improvement. Discussion and conclusions: as the prevalence is low, the diagnosis requires a high level of suspicion in cases presenting with infectious conditions prior to the development of symptoms, since it is considered an autoimmune cross-reaction against substantia nigra antigens. Alterations in brain imaging or in cerebrospinal fluid cytometry may be found in some cases. Management with methylprednisolone pulse therapy and filtration plasmapheresis provides significant improvement with a decrease in future sequelae.

Enfermedad de Von Economo: Caso clínico / Lethargic Encephalitis: Report of one case

Lethargic encephalitis (LE) is a Central Nervous System disorder following an upper respiratory tract infection, characterized by sleep disturbances, clinical symptoms corresponding to basal ganglia involvement and in some cases, neuropsychiatric sequelae. We report a 18-year-old mole with a history of sinusitis treated with azithromycin, two weeks before, presenting with fever, headache, confusion and myoclonus. Urine analysis was positive for cannabis. Cerebro spinal fluid analysis showed mononuclear pleiocytosis (109xmm³) and an increase in protein concentration ofl.6 g/dl. Forty eight hours after admission, the patient required mechanical ventilation and subsequently a status epilepticus appeared. Ten days later, fever, rigidity and resting tremor appeared. A magnetic resonance imaging showed hyperintensities in FLALR sequence in the right insular cortex. The patient continued with extreme rigidity, catatonia and mutism. Considering the possibility ofa LE, methyl prednisolone 1 g/day was administered for five consecutive days followed by prednisone 40 mgl day, observing a dramatic improvement of rigidity and tremors.

El triste final prematuro de la pianista chilena Rosa Renard Artigas 1894-1949 / The sad and premature ending of the chilean pianist Rosa Renard Artigas 1894-1949

Se analiza en forma restrospectiva la enfermedad neurológica que terminó prematuramente con la vida de Rosa Renard Artigas a los 55 años de edad. Ella fue conocida y admirada internacionalmente como una notable pianista y su muerte ocurrió 4 meses después de su triunfo presentación en el Carnegie Hall de Nueva York. El diagnóstico de encefalitis letárgica hecho por su médico personal aparece muy cuestionable, dados el comienzo subagudo y las manifestaciones neurológicas que son muy discordantes con lo descrito como signos característicos de la encefalitis letárgica por Von Economo. En cambio, la probabilidad de una posible enfermedad de Creutzfeldt-Jakob es altamente sugerente en este caso

Akathisia in Parkinson's Disease

BACKGROUND: Akathisia is defined as inner feeling or restlessness and can be associated with restless movement. It is a frequent and disabling complication of neuroleptics and was reportedly common in postencephalitic parkinsonism. Akathisia has been reported and appears to be common in Parkinson's disease(PD). OBJECTIVE: To determine the frequency and clinical features of akathisia and dexamine the relationship between presence of akathisia and various clinical parameters of PD, Method: we evaluated 137 PD patients from movement disorder clinics in Samsung Medical Center and Seoul National University Hospital, using a modified akathisia questionnare. We compared two groups between those with akathisia and those without akathisia. We analysed the pattern, location and chronology of akathisia, and the correlation between akathisia and clinical parameters of PD. RESULTS: 1. Of the 137 patients (76 women, 61 men), 43(22 women, 21 men) (31.4%) had akathisia. 2. Patients with akathisia had more advanced disease than those without akathisia as determined by Hoehn and Yahr stage (p<0.05). 3. Patients with akathisia were more often akinetic-rigid than tremor predominent (p<0.05). 4. Patients with akathisia were more frequently treated with levodopa than those without akathisia (p<0.05). 5. There was no difference between two groups for age, sex or disease duration(p<0.05). 6. Of the 43 patients with akathisia, 35 patients had motor restlessness and 27 patients had associated sensory complaints. 7. The whole body or legs were most frequently affected. 8. Akathisic symptoms occurred irregularly and mainly in the afternoon. 9. Nineteen patients had difficulty in their jobs due to akathisia. Conclusion: Akathisia is a common problem in PD especially in severe akinetic-rigid form. It can be the main cause of disability in PD. Recognition and proper management are needed.

Prevalence of parkinsonism in Assiut clinical and epidermiological study

Parkinson's disease probably occurs throughout the world. Epidemiological study of parkinsonism in Egypt is deficient. The present work aimed to study epidemiological and clinical data about parkinsonism in Assiut Upper Egypt. A representative sample of 42.000 subjects [7,000 families] which included different communities [urban, suburban and rural] were studied. Prevalence of parkinsonism in Assiut was 226/100,000. The atherosclerotic type was the most prevalent one [155/100,000] followed by post-encephalitic type [33/100,000] then drug-induced parkinsonism [24/100,000] and lastly paralysis agitans [14/100,000]. Paralysis agitans was observed at an age range from 50 to < 60 years, post-encephalitic from 30 to 40 years and atherosclerotic type from age period 50 t o < 70 years with maximum age range from 60 < 70 years and drug induced type in age period from 30 to < 40 years. All types of parkinsonism were more prevalent in rural areas than suburban and urban areas except paralysis agitans which was more prevalent in urban areas. Tremors, rigidity and hypokinesia represented the main presentation of different types of prkinsonism. Hypertension and diabetes were significantly higher in atherosclerotic parkinsonism than in controls [P < 0.05]. Heavy smoking was significantly [P < 0.05] incriminated among paients with the atherosclerotic type

Disturbances in water metabolism in parkinsonism

Sixteen patients suffering from parkinsonism due to paralysis agitans and post-encephalitis were studied. Eleven patients showed a defective elimination of water after a water load. In the two patients who showed the most marked diminution in water elimination, hypertonic saline failed to inhibit the urinary output after a water load, and produced an osmotic diuresis. The osmoreceptor hypothalamic pituitary mechanism is sluggish, both to the stimulus of hyper as well as hypoosmolarity, and the fault is most probably in the hypothalamic pituitary apparatus. 1] 16 patients suffering from parkin-sonism due to paralysis agitans and post-encephalitis were studied, 2] Eleven patients showed a defective elimination of water after a water load. 3] In the two patients who showed the most marked diminution in water elimination, hypertonic saline failed to inhibit the urinary output after a water load, and produced an osmotic diuresis. 4] The osmoreceptor hypothalamic pituitary mechanism is sluggish, both to the stimulus of hyper-, as well as hypo-osmolarity, and the fault is most probably in the hypothalamic pituitary apparatus