Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023380 (lethargy)
5,697 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with agammaglobulinemia are particularly susceptible to chronic enteroviral infections of the central nervous system. Data on 42 patients were obtained by literature review, communications with other physicians, and personal experiences. Thirty-eight patients had congenital immunodeficiencies, most frequently X-linked agammaglobulinemia. Most patients who could be assessed were receiving maintenance therapy with intramuscular gamma-globulin before their enteroviral infection. Seven patients had not been recognized as hypogammaglobulinemic before the onset of infection. The commonest pathogens were echoviruses (37 of 41 cases), especially type 11 (11 cases). Thus far, four patients have had sequential or simultaneous infections with a second enteroviral serotype. Other features of the disease have included weakness, lethargy or coma, headaches, hearing loss, seizures, ataxia, and paresthesias. Some patients have also had nonneurologic manifestations of chronic enteroviral infection, including fever, the dermatomyositis-like syndrome, edema, rashes, and hepatitis. Treatment has consisted primarily of antibody administration, either in intravenous immunoglobulin preparations or in immune plasma. Twelve patients have received intraventricular immunoglobulin through reservoir devices; six of these 12 have improved substantially, as judged by clinical criteria.
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PMID:Chronic enteroviral meningoencephalitis in agammaglobulinemic patients. 329

We report a 14-year-old girl with juvenile dermatomyositis (JDM) complicated by severe inflammatory calcinosis successfully treated with thalidomide. She was diagnosed as JDM when she was 4 years old after a few months of increasing lethargy, muscle pain, muscle weakness, and rash. During three months, clinical manifestations and abnormal laboratory findings were effectively treated with oral prednisolone. However, calcinosis was recognized 18 months after disease onset. Generalized calcinosis rapidly progressed with high fever, multiple skin/subcutaneous inflammatory lesions, and increased level of CRP. Fifty mg/day (1.3 mg/kg day) of oral thalidomide was given for the first four weeks, and then the dose was increased to 75 mg/day. Clinical manifestations subsided, and inflammatory markers had clearly improved. Frequent high fever and local severe pain with calcinosis were suppressed. The levels of FDP-E, IgG, and tryglyceride, which were all elevated before the thalidomide treatment, were gradually returned to the normal range. Over the 18 months of observation up to the present, she has had no inflammatory calcinosis, or needed any hospitalization, although established calcium deposits still remain. Her condition became painless, less extensive and less inflammatory with the CRP level below 3.08 mg/dL. Recent examination by whole-body 18F-FDG-PET-CT over the 15 months of thalidomide treatment demonstrated fewer hot spots around the subcutaneous calcified lesions.
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PMID:Efficacy of thalidomide in a girl with inflammatory calcinosis, a severe complication of juvenile dermatomyositis. 2018 Oct 85

A 62-year-old woman presented to the emergency department with malaise, lethargy and proximal muscle weakness. She had a similar presentation in childhood. A muscle biopsy was inconclusive, however, she was treated for a presumptive diagnosis of inflammatory myositis. In a second flare in childhood, she developed proximal muscle weakness and calcinosis cutis. A muscle biopsy was consistent with juvenile dermatomyositis. She was treated with corticosteroids, however, she relapsed again 1 year later, requiring a prolonged course of corticosteroids, which led to remission and treatment was subsequently stopped. She remained well for 30 years without any relapses, however, at the age of 40 she was diagnosed with malignant melanoma, treated with local excision and radiotherapy. She had a further period of 22 years in good health, until her current presentation, with a relapse of dermatomyositis, 52 years after the last flare. She is currently in remission following successful immunosuppressive therapy.
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PMID:Childhood dermatomyositis recurring in adulthood half a century later. 2400 Feb 11

Intraventricular recombinant tissue plasminogen activator (IVT rt-PA) has improved outcomes for intraventricular hemorrhage (IVH). Patients with suspected or untreated arteriovenous malformations (AVMs) have been excluded from clinical trials. We present a patient with IVH secondary to a ruptured AVM safely treated with IVT rt-PA. A 48-year-old Hispanic male with a history of dermatomyositis presented to the emergency department with sudden left-sided weakness. En route to computed tomography (CT), he became lethargic. Computed tomography revealed extensive IVH with acute hydrocephalus, which was treated with the placement of external ventricular drain with clinical improvement. Computed tomography angiogram performed did not reveal AVM. Cerebral digital subtraction angiogram (DSA) was planned due to suspicion of AVM. Prior to DSA, patient became acutely lethargic. Computed tomography imaging revealed worsening hydrocephalus. External ventricular drain was noted to be draining. Repeat CT revealed improved hydrocephalus but with left lateral ventricle dilatation. Risks and benefits of IVT rt-PA were discussed with the family and a decision was made to treat. Three doses of 1 mg IVT rt-PA were administered with resolution of midline blood and lateral ventricular dilatation with clinical improvement. Digital subtraction angiogram revealed early draining vein on right internal carotid artery injection draining into the inferior sagittal sinus representing ruptured AVM without clear nidus. Repeat DSA with possible embolization was planned after discharge. In spite of additional in-hospital complications, the patient gradually improved and was ultimately discharged home. Our case supports the idea that the use of IVT rt-PA following an IVH caused by an underlying AVM could be further explored in carefully designed clinical trials.
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PMID:Safety of Intraventricular rt-PA for Pan-Ventricular IVH Caused by a Ruptured AVM: A Case Report. 2897 5