Recurrence of acute disseminated encephalomyelitis after a 12-year symptom-free interval

We report a patient with recurrent acute meningoencephalitis who had three episodes of headache, fever and unconsciousness; the first episode was at age 6 and the second, at age 7. After a 12-year symptom-free interval, she had a relapse, exhibiting the same symptoms as those in the previous two episodes. Head magnetic resonance imaging also revealed the recurrence of lesions in the basal ganglia and medial portion of the temporal lobe. The occurrences of stereotyped symptoms with meningoencephalitis and the same lesions in the basal ganglia observed in each episode favor the diagnosis of recurrent acute disseminated encephalomyelitis (ADEM) rather than multiple sclerosis or multiphasic disseminated encephalomyelitis. The occurrence of this rare case suggests that ADEM can relapse after a very long symptom-free interval.     

Acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is a demyelinating syndrome of the central nervous system. It is considered to be an autoimmune response to an antecedent antigenic stimulus, most frequently a prior infectious illness (postinfectious encephalitis) or immunization (postvaccination encephalitis) occurring in the days or weeks before neurologic illness. Clinically, ADEM is characterized by encephalopathy, or focal/multifocal neurologic signs; brain MRI is characterized by diffuse multifocal or patchy areas of demyelination. The differentiation between ADEM and a first episode of multiple sclerosis, a chronic central nervous system demyelinating disease, may be difficult but has important prognostic and treatment implications. Although no clinical trials have assessed the efficacy of treatment modalities in patients with ADEM, immunomodulators, including corticosteroids and intravenous immunoglobulin, are frequently used empirically. ADEM outcome is generally favorable, with resolution over weeks to months.     

Acute demyelinating encephalomyelitis (ADEM) in a patient with HIV infection

A case of acute demyelinating encephalomyelitis (ADEM) in a patient with HIV infection is reported. Although the diagnosis of ADEM is based on clinical and radiological findings, the potential for full recovery, with appropriate treatment, is highlighted by this case. A concise review of the subject is given in the discussion.     

Acute demyelinizating encephalomyelitis (ADEM) as initial presentation of primary HIV infection

The authors report a patient with sexual exposure, clinical symptoms, MRI, virological and CSF findings suggestive of acute demyelinizating encephalomyelitis (ADEM) as initial presentation of primary HIV infection. The aetiology, pathophysiology, diagnosis, and treatment of ADEM is reviewed, and the sparse existing literature on ADEM and HIV infection is discussed.     

A neuroimaging follow up study of a patient with juvenile central nervous system systemic lupus erythematosus

BACKGROUND: The course of central nervous system systemic lupus erythematosus (CNS-SLE) is largely unknown. New imaging techniques are available to assist in monitoring the disease course. OBJECTIVE: To report a case of juvenile CNS-SLE, in which magnetic resonance imaging (MRI) was used to assess disease activity. CASE REPORT: A 10-year-old female patient with SLE presented with convulsions; MRI and computed tomography (CT) of the cerebrum disclosed abnormalities. Despite adequate treatment, two years later she had a generalised convulsion, and MRI showed new lesions. MR spectroscopy (MRS) indicated neuronal loss, inflammation, and metabolically compromised tissue; magnetisation transfer imaging (MTI) showed an increase in whole brain lesion load. After exclusion of a malignancy, CNS-SLE was the most likely diagnosis, and cyclophosphamide pulses were administered. Initially, multiple sclerosis (MS)-like lesions regressed, but despite maximal immunosuppressive drugs, new lesions formed and disappeared. When immunosuppressive drugs had been stopped for six months MRI showed improved lesions and MTI histograms. DISCUSSION: In this case report, the anatomical substrate, metabolic aspect, neuroimaging, and clinical course of MS-like lesions in a child with CNS-SLE are described. The way in which radiological techniques can support clinical decision making in this young patient with progressive CNS-SLE is illustrated.     

Acute disseminated encephalomyelitis (ADEM) after allogeneic bone marrow transplantation for acute myeloid leukemia

Acute disseminated encephalomyelitis (ADEM) is a rare inflammatory demyelinating disease of the central nervous system. We describe here a patient who developed ADEM after allogeneic bone marrow transplantation (BMT). A 48-year-old woman with acute myeloid leukemia (M2) underwent allogeneic BMT from her HLA-identical sister. Cyclosporin for prophylaxis of acute graft-versus-host disease (GVHD) was discontinued from day 15 because of its toxicity. She was relatively well after the resolution of cytomegalovirus reactivation and chronic GVHD. Nine months after BMT, she suddenly developed diplopia, dysarthria, and gait disturbance. Computed tomography of the brain at that time revealed no abnormal findings. Leukemia recurrence was not revealed. The neurological symptoms were very mild without further deterioration. Her clinical course was carefully watched without therapy. Two weeks after onset, fluid attenuated inversion recovery magnetic resonance imaging (MRI) revealed multifocal abnormal high-signal intensity mainly in the white matter of the cerebrum as well as in the cerebellum and brainstem. Cerebrospinal fluid examination showed no abnormal findings. No laboratory findings suggested the presence of infectious agents. The typical MRI findings and an acute monophasic clinical course of this patient led to a diagnosis of ADEM. Twelve weeks after onset, the symptoms had almost resolved. Follow-up MRI showed a substantial improvement of the previous lesions without any new lesions. The symptoms had completely resolved 5 months after onset. This is a rare case of ADEM developing after allogeneic BMT.     

Acute disseminated encephalomyelitis (ADEM) after autologous peripheral blood stem cell transplant for non-Hodgkin's lymphoma

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disorder of the central nervous system with an acute clinical onset and a wide variability in severity and outcome. It usually follows a viral infection or an immunization and is thought to be immuno- mediated. We report a case of ADEM with a dramatic clinical onset in an autologous peripheral blood stem cell transplant (PBSCT) recipient for non-Hodgkin's lymphoma who developed the neurologic syndrome 12 days after PBSC reinfusion. This is the first report of ADEM in the setting of autologous PBSCT, a therapeutic procedure performed with increasing frequency in a wide variety of hematologic and solid malignancies.     

A case report of plasmapheresis in the treatment of acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system associated with significant morbidity and mortality. High-dose corticosteroid administration has been considered the mainstay of treatment for ADEM; however, some patients with ADEM are refractory to steroid therapy. We report a case of a 17-year-old man suffering from ADEM who did not respond to corticosteroid therapy, but who exhibited a dramatic recovery with plasmapheresis. He became comatose, requiring ventilatory support, and exhibited abnormalities of some brainstem reflexes prior to treatment. He underwent sequential courses of plasma exchange therapy for three days. Plasma exchanges were carried out with concomitant continuous hemodiafiltration (CHDF) to control intracranial pressure by stabilizing pH, plasma Na+ concentration, and colloid osmotic pressure. After plasma exchanges, his reflexes and level of consciousness gradually improved. Eleven months after this treatment, he had only minimal neurological deficit that did not interfere with any of his activities of daily living. The efficacy of plasmapheresis for ADEM has not yet been established. Plasmapheresis may be indicated for ADEM, not only for patients with severe disease in whom high-dose corticosteroid treatment has failed, but also as first-line treatment for ADEM. Early initiation of plasmapheresis appears to be associated with moderate to marked improvement. Early recognition and early treatment of ADEM are thus of paramount importance.     

Lethal acute demyelinization with encephalo-myelitis as a complication of cured Cushing's disease

Cushing's disease is usually associated with higher mortality rate, especially from cardiovascular causes. Development or exacerbation of autoimmune or inflammatory diseases is known to occur in patients with hypercortisolism after cure. We report for the first time a 34-year old woman with a psychiatric background, who developed four months after the surgical cure of Cushing's disease an acute disseminated encephalomyelitis (ADEM) presenting initially as a psychiatric illness. We hypothesize that the recent correction of hypercortisolism triggered ADEM and that the atypical presentation, responsible for diagnosis delay, led to the death of this patient.     

Haemorrhagic acute disseminated encephalomyelitis: an unusual case of blindness in an HIV-infected patient

Acute disseminated encephalomyelitis (ADEM) is a monophasic, polysymptomatic, immune-mediated demyelinating disorder involving central nervous system (CNS) white matter. It is usually seen in association with exanthematous viral illnesses, systemic infections or vaccinations. The haemorrhagic form of ADEM is rarer and follows a more fulminant course. We describe a case of haemorrhagic ADEM in an HIV-infected patient presenting solely with acute onset bilateral blindness and normal retina. ADEM was diagnosed from clinical, laboratory and radiological findings. Although ADEM has been seen with HIV seroconversion illness and in chronic infection, the haemorrhagic form has never been reported in association with HIV. Acute onset blindness is extremely rare in HIV-positive patients. A Medline search revealed very few reports. The importance of considering haemorrhagic ADEM as aetiology of blindness in such a scenario is discussed.     

Pancreas transplantation in Mauriac Syndrome: clinical and biochemical parameters after one year follow up

In select cases of type 1 diabetes mellitus (DM1) the pancreas transplantation has been shown to ameliorate the disease, to reduce the need for exogenous insulin and normalize glycosylated hemoglobin (A1c) levels. The efficacy of this therapy in Mauriac Syndrome (SM) is not yet well established. We report a patient with MS treated with intensive insulin therapy, physical activity program, nutritional and psychological assistance, with persistently elevated fast glycemia and A1c levels, inadequate lipid profile and decreased IGF-1 (insulin like growth factor) levels. Due to a poorly metabolic control, pancreas transplantation was indicated. After one year follow up, the patient had no symptoms and showed persistent insulin independence with fast glucose <110 mg/dl, normal lipid profile and IGF-1 levels and significant decrease in A1c (4.6%). The pancreas transplantation improved diabetes control and promoted better quality of life for this patient. Pancreas transplantation proved to be an effective treatment strategy in patients with MS, improving their clinical and biochemical derangements. In this report we present the first case of MS controlled by pancreas transplantation registered in the indexed medical literature, as an alternative therapy in this group of patients.     

Acute disseminated encephalomyelitis during treatment for idiopathic thrombocytopenic purpura

A 54-year-old woman had an episode of sudden oral bleeding and generalized petechiae 1 week after a sore throat and diarrhea. On admission, the platelet count was 0.1 x 10(4)/microl, and the platelet-associated IgG level was elevated. Hyperplasia of megakaryocytes in a bone marrow specimen and aberrant Epstein-Barr virus (EBV) antibody patterns led to a diagnosis of EBV-associated idiopathic thrombocytopenic purpura (ITP). Prednisolone (PSL) promptly restored her platelet count; however, she developed disorientation and affective lability soon after PSL was tapered. Subsequently, she ran a high fever and developed convulsive seizures. T2-weighted MRI demonstrated a high signal area in the subcortical white matter, and no abnormal findings were found on examination of the cerebrospinal fluid. The diagnosis of acute disseminated encephalomyelitis (ADEM) was made and steroid pulse therapy was started, which resulted in remission of the symptoms without recurrence in the following months. This is the first reported case of ADEM following EBV infection during treatment for ITP. Administration of PSL for ITP might mask the presenting clinical picture of ADEM. The possibility of ADEM should be investigated in patients of ITP following viral infection who develop acute encephalopathy.     

Left anterior descending coronary artery stenosis: in a patient with takotsubo cardiomyopathy

Takotsubo cardiomyopathy is characterized by chest pain, electrocardiographic abnormalities such as ST-segment elevation or depression, and elevated cardiac enzyme levels. Left ventriculography reveals transient akinesis of the involved segment of the myocardial wall (usually the left ventricular apex) and compensatory hyperkinesis of the noninvolved myocardium, which appears as apical ballooning during systole. Existing criteria for the diagnosis of takotsubo cardiomyopathy include the absence of obstructive coronary artery disease. Indeed, previous investigators have found incidental stenosis in only a minority of patients.Herein, we present the unusual case of an 84-year-old woman who sustained 4 episodes of takotsubo cardiomyopathy in 18 years. At the time of the initial episode, coronary angiography revealed no substantial stenosis. Concomitant with the 2nd episode, stenosis in the 1st obtuse marginal branch was treated with stenting. No new lesions were apparent after the patient's 3rd presentation, and the previously placed stent was patent. During the 4th (current) presentation, we detected and percutaneously treated severe stenoses in the patient's left anterior descending coronary artery and 2nd obtuse marginal branch.Although this report is of a single patient only, it definitively illustrates that severe coronary artery disease can occur in patients who have takotsubo cardiomyopathy. We recommend the thorough evaluation of possible coronary artery disease in high-risk patients, even upon the strong clinical suspicion of takotsubo cardiomyopathy.     

Pneumonia in a patient with familial Mediterranean fever successfully treated with anakinra—case report and review

We report the case of a 35-years-old renal transplant patient known to have familial Mediterranean fever with serum amyloid A (SAA)-amyloidosis, who presented with his second episode of bilateral pneumonia. As antimicrobials failed to control the first episode of pneumonia and all studies done were non-contributory, we attributed the condition to the highly active Mediterranean fever presumably resistant to colchicine and treated the patient with the interleukin-1 receptor antagonist anakinra: the patient substantially improved by clinical symptoms, chemistry and radiological evidence within no more than 2 days and was discharged in good health after 4 days.     

Levothyroxine-induced liver dysfunction in a primary hypothyroid patient

Here we report a case of levothyroxine-induced liver dysfunction. T4 (levothyroxine) has been more commonly used for the treatment of hypothyroidism than T3 active hormone (triiodothyronine), because with the former drug a stabler plasma concentration is obtained after oral administration. Although there are few reports on levothyroxine-induced liver dysfunction, we treated a primary hypothyroid patient with high serum aminotransferase after administration of levothyroxine. Liver dysfunction was improved after cessation of the drug administration. Antibody to T4 was found in the serum of the patient after this event. From clinical course and laboratory data of the patient, the episode of liver damage was considered to be induced by levothyroxine. We then administrated triiodothyronine, and it did not induce liver dysfunction. Changing levothyroxine to triiodothyronine resulted in a successful clinical course in this case, as re-administration of the doubtful drug is strictly limited.     

Case Report: Madariaga Virus Infection Associated with a Case of Acute Disseminated Encephalomyelitis

We present the first report of a pediatric case of acute disseminated encephalomyelitis (ADEM) associated with Madariaga virus infection (MADV, Alphavirus, Togaviridae; formerly known as South American variants of eastern equine encephalitis virus [EEEV]) in a patient of the 2010 alphaviral epidemic reported in Panama. The patient was admitted to the Hospital del Niño in Panama City with suspected meningitis, exhibited with decreased alertness and disorientation in space and time, hemiparesis, and left Babinski sign. The patient was transferred to the intensive care unit and treated with aciclovir and methylprednisolone. The magnetic resonance imaging (MRI) of the brain revealed multiple hyperintense lesions at T₂-weighted images (T2) and fluid-attenuated inversion recovery (FLAIR) on the cortical–subcortical level. Sera samples obtained on days 6 and 12 were immunoglobulin M (IgM) positive for MADV. The findings on the clinical and cerebrospinal analyses, rapid symptom progression as well as neuroimaging, and serologic studies support our diagnosis. Our results suggest that MADV should be included in the etiologic differential diagnosis of ADEM in endemic countries.     

Severe depression as an initial symptom in an elderly patient with acute disseminated encephalomyelitis.

We report a 63-year-old man with acute disseminated encephalomyelitis (ADEM), initially showing depression for one and a half months but subsequently meningoencephalitis followed by acute-onset myelopathy. Neuroradiological examinations of the brain demonstrated no focal lesion causative for his depression, while cerebrospinal fluid revealed elevated levels of inflammatory cytokines in parallel with disease activity. Because depression is usually a rare initial symptom for patients with ADEM, an increased production of inflammatory cytokines in the central nervous system as well as age-related alterations of immune response might have played an important role in the development of depression in this elderly patient.     

Necrotizing acute pancreatitis following therapeutic plasmapheresis in HCV-related cryoglobulinemia

Mixed cryoglobulinemia and hepatitis C virus infection are strongly connected and the therapeutic approach is standardized according to the severity of the symptoms. We report the difficult management of 59 year old female HCV patient presenting cutaneous lesions and arthralgia due to mixed cryoglobulinemia. No therapy was able to achieve a complete remission and during the six years of active disease we observed several clinical recurrences. The intensive plasmapheresis regimen led to a complete remission of the symptoms but it was associated with severe complications. In this case report we describe an episode of acute necrotizing pancreatitis due to intravascular haemolysis following therapeutic plasmapheresis. To the best of our knowledge the association between plasmapheresis and acute pancreatitis has not been previously described.     

Delayed onset adenosine deaminase deficiency associated with acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is a monophasic, immune-mediated demyelinating disorder that can appear after either immunizations or, more often, infections. Magnetic resonance imaging of patients shows inflammatory lesions in the brain and spinal cord. An immune-mediated mechanism may play a role in this disease, although its precise pathogenesis remains unclear. In this study, a 2-year-old boy presented with ADEM, and he showed improvement on treatment with high-dose intravenous corticosteroids. At the age of 3 years, the presence of recurrent bronchitis, bronchiectasia, and lymphopenia suggested that the patient was suffering from combined immunodeficiency. The patient was finally diagnosed with delayed onset adenosine deaminase deficiency. Delayed onset adenosine deaminase deficiency is frequently associated with autoimmune diseases, including thyroiditis and cytopenia, both of which were observed in the patient. The ADEM in this patient may be a presentation of delayed onset adenosine deaminase deficiency.     

Primary Sjögren's syndrome associated with pneumatosis cystoides coli

We report the case of a 43-year-old woman with primary Sjögren's syndrome, according to Americano–European criteria. Eighteen months after the diagnosis, the patient presented pneumatosis cystoides coli (PCC), which resolved with medical treatment consisting of diet and cisapride. Four years after this episode, the patient has not developed clinical features of another systemic inflammatory rheumatic disease and PCC has not relapsed. To the best of our knowledge, the association between primary Sjögren' syndrome and PCC has never been reported. Physiopathology and treatment of PCC are discussed.