[Encephalitis Lethargica: Awakenings].

Postencephalitic parkinsonism is a characteristic chronic sequelae of encephalitis lethargica, a pandemic disorder which swept the whole world in 1917-1928. Based on his clinical experiences at the Mount Carmel hospital, Oliver Sacks published Awakenings in 1973, and an American drama film Awakenings was premiered in 1990. Success and setbacks of the levodopa trial in postencephalitic parkinsonism patients were chronologically described, and the mother's delight on her son's "return" was impressive; this scene can be shared by us neurologists, even if it was transient. Above all, the exemplary performances of the two distinguished actors, Robin Williams and Robert De Niro (especially the latter), enlivened the most excellent moments of the movie.

Post encephalitic parkinsonism following dengue viral infection.

BACKGROUND: Incidence of dengue fever as well as dengue hemorrhagic fever is increasing in Sri Lanka especially among elderly population. As the number of cases is rising, rare complications of dengue illness also can be seen in clinical practice when compared to the past few years. Prompt identification and treatment of such complications is challenging due to lack of awareness and unavailability of standard treatment. CASE PRESENTATION: 69 years old man presented with acute onset fever and was diagnosed as having dengue illness. On the 4th day of illness, the disease was progressed into dengue haemorrhagic fever and recovered uneventfully. Although he recovered from primary illness, his general condition continued to deteriorate due to new onset of features of parkinsonism. Cerebrospinal fluid analysis and electro encephalogram showed evidence of encephalitis. Cerebrospinal fluid analysis also revealed positive IgM antibodies against dengue virus. Then the diagnosis of post encephalitic parkinsonism following dengue viral infection was made and started on. He was started on SINEMET (carbidopa 10 mg and levodopa 100 mg) half tablet 6 hourly started. After 1 week of treatment he showed marked improvement and then patient was discharged with further follow up plan. CONCLUSION: Although the management of dengue illness and dengue haemorrhagic fever is straightforward, recognition and treatment of rare complications like post encephalitic parkinsonism following dengue viral infection is difficult without great clinical suspicion.

Postencephalitic parkinsonism: interesting clinico-imaging correlation.

IMPORTANCE: Post-encephalitic parkinsonism (PEP) is believed to be caused by a viral illness which causes degeneration of the nerve cells in the substantia nigra, resulting in clinical parkinsonism. Although common during the period of the First World War, the diagnosis of PEP is rarely entertained today. OBSERVATION: We report an adolescent boy of PEP, who was bedridden due to severe parkinsonism following the encephalitis attack. After relevant imaging and investigation, he was treated with levodopa which made him independent from a year long total dependent condition. RELEVANCE AND CONCLUSION: Encephalitis lethargica or PEP is one of the rare parkinsonian syndromes which may be amenable to treatment. Proper diagnosis and treatment may help the patient become ambulatory from the dreaded lethargic condition.

[Lethargic encephalitis. Report of one case]. / Enfermedad de Von Economo: Caso clínico.

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 of l.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 mg l day, observing a dramatic improvement of rigidity and tremors.

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.

[Isolated pulmonary hypertension and pergolide]. / Hypertension artérielle pulmonaire isolée et pergolide.

Pergolide, an ergot-derived dopamine agonist prescribed since the late 1980's mainly in Parkinson's disease and restless leg syndrome has recognized fibrosis side effects, affecting the pleural, pericardial and retroperitoneal systems. Pergolide-induced valvulopathies were first reported in 2002. We present here the history of a patient developing an isolated pulmonary hypertension related to the intake of pergolide. The dyspnea related to the pulmonary hypertension as well as the echocardiographic abnormalities improved after treatment replacement. Valvulopathies and pulmonary hypertension were previously described under appetite-suppressant drugs after years of clinical use, in a similar way.

Steroid-responsive encephalitis lethargica syndrome with malignant catatonia.

We report a 47-year-old man who is considered to have sporadic encephalitis lethargica (EL). He presented with hyperpyrexia, lethargy, akinetic mutism, and posture of decorticate rigidity following coma and respiratory failure. Intravenous methylprednisolone pulse therapy improved his condition rapidly and remarkably. Electroencephalography (EEG) showed severe diffuse slow waves of bilateral frontal dominancy, and paralleled the clinical course. Our patient fulfilled the diagnostic criteria for malignant catatonia, so we diagnosed secondary malignant catatonia due to EL syndrome. The effect of corticosteroid treatment remains controversial in encephalitis; however, some EL syndrome patients exhibit an excellent response to corticosteroid treatment. Therefore, EL syndrome may be secondary to autoimmunity against deep grey matter. It is important to distinguish secondary catatonia due to general medical conditions from psychiatric catatonia and to choose a treatment suitable for the medical condition.

The two faces of L-DOPA: benefits and adverse side effects in the treatment of Encephalitis lethargica, Parkinson's disease, multiple sclerosis and amyotrophic lateral sclerosis.

Parkinson's disease, encephalitis lethargica, multiple sclerosis and amyotrophic lateral sclerosis patients all display two distinct types of symptoms. Some of these are due directly to a deficiency of dopamine and are quickly reduced by laevodihydroxyphenylalanine (L-DOPA). The second set, however, are the result of neurological damage caused by metabolites of dopamine, which include dopachrome and other chrome indoles that are both hallucinogenic and neurotoxic. If this hypothesis is correct, three corollaries follow. Patients of all four disorders should display excessive oxidative stress, natural methyl acceptors should delay development and elevated antioxidant supplementation, given with L-DOPA, ought to prolong the "honeymoon" period in which the benefits of the drug out weigh its subsequent disadvantages. A literature review suggests that all three corollaries are probably correct.

Dopaminergic therapy in acute encephalitis lethargica.

We describe a patient with an acute illness, with clinical features characteristic of encephalitis lethargica, who responded repeatedly to trials of an apomorphine infusion and subsequently to oral levodopa therapy. This observation implies a direct acute neurotropic effect of the encephalitis on nigral dopaminergic cells. Dopaminergic medication may therefore be helpful in the acute stage of encephalitis lethargica as well as in the delayed post-encephalitic parkinsonian phase of the syndrome.

[Parkinsonian syndrome and post-encephalitic stereotyped involuntary movements responsive to L-dopa]. / Syndrome Parkinsonien et mouvements involontaires stéréotypés post-encéphalitiques, sensibles à la L-dopa.

In 1954, when he was five years old, a patient suffered from encephalitis with a prolonged lethargic state. Following this episode, he presented a severe parkinsonian syndrome which was associated, after a few years, with an axial dystonia and stereotyped involuntary movements of the upper limbs. These abnormal movements were particular by their coordinated appearance, their rhythmicity and their relative slowness. Treatment with L-dopa suppressed all akinetic, dystonic and dyskinetic symptoms. At age of 40 years, all the akinetic, dystonic and dyskinetic symptoms reappeared after drug withdrawal. Cerebral computed tomography, magnetic resonance imaging and fluorodeoxyglucose positron emission tomography were normal. Fluorodopa positron emission tomography revealed a significant bilateral reduction of tracer accumulation in the posterior part of both putamen, similar to that observed in patients with idiopathic Parkinson's disease. In this patient, pharmacological tests revealed that effectiveness of L-dopa was abolished by administration of a D2 antagonist, and was fully reproduced by a D2 agonist. Clinical signs, pharmacological data and past-medical history strongly suggested a limited lesion of the zona compacta of substantia nigra induced by viral agression. This complex and progressive extrapyramidal syndrome had strong similarities with the lethargic encephalitis of Von Economo and its late symptoms. Other diseases associating akinesia and dyskinesia or dystonic phenomena, like dopa-sensitive dystonia and juvenile Parkinson's disease, are very unlikely. Thus, the persistance of sporadic forms of Von Economo's encephalitis could be discussed.

Postvaccinal parkinsonism.

A 5-year-old boy, with a history of fever beginning 15 days after a vaccination for measles, developed a rigid-akinetic syndrome 3 days after the fever began. A spinal tap obtained 1 week after the onset of fever showed pleocytosis with a monocellular pattern. A CT scan of the head and EEG did not disclose any abnormality. An MRI performed 3 months after the event, however, showed clear-cut evidence of bilateral substantia nigra lesions, suggesting secondary gliosis. The response to levodopa was good, but adverse reactions appeared early. The child is now 7 years old. Bromocriptine, deprenyl, and levodopa have produced a remarkable improvement of the parkinsonian features.

[Correlation of clinico-physiologic indices and the serotonin content of the blood of patients with parkinsonism]. / Korreliatsiia kliniko-fiziologicheskikh pokazatelei i soderzhaniia serotonina v krovi u bol'nykh parkinsonizmom.

On withdrawal of L-DOPA-containing drugs the blood serotonin content decreased in parkinsonism patients irrespective of form and etiology of the disease. L-DOPA-containing drugs caused the blood serotonin levels to rise. Substantial correlations of the blood serotonin levels and clinico-physiologic indices were found mostly in postencephalitic parkinsonism.

[Experience with the use of parlodel in the treatment of Parkinson patients]. / Opyt primeneniia parlodela pri lechenii bol'nykh parkinsonizmom.

The dopamine agonist parlodel was given to 42 patients with parkinsonism for several months. The drug proved effective in more than one-third of the patients. Different side-effects were observed in almost half of the patients. Parlodel may be used alone in mild cases but generally it is used as an adjunct to L-DOPA therapy. The optimum dose of parlodel is individual and varies within a wide range.