Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0014070 (encephalomyelitis)
 13,017 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 27 year old woman developed a cerebellar syndrome with serological evidence of recent Mycoplasma pneumoniae infection. The cranial computed tomographic scan showed effacement of the fourth ventricle, enhancement of the basal meninges and hydrocephalus affecting the lateral and third ventricles. Clinical and radiological recovery occurred over 5 weeks. We propose that this was a manifestation of immune-mediated encephalomyelitis induced by the infection rather than direct invasion of the central nervous system.
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PMID:Cerebellar syndrome with hydrocephalus due to Mycoplasma pneumoniae infection. 221 14

Paraneoplastic cerebellar degeneration (PCD) is a rare complication of systemic cancer. PCD may present as a "pure", severe pan-cerebellar syndrome of subacute progression or be only one clinical feature in the setting of extensive CNS disease. The most characteristic form of "pure" PCD is associated with the presence of an anti-Purkinje cell antibody (AB), called anti-Yo, in patients with breast or ovarian cancer. The primary tumor is very often unknown when the cerebellar signs occur, and extensive investigations, including laparotomy or prolonged follow-up may be required to demonstrate its presence. More rarely, others AB than anti-Yo are discovered during PCD. Almost 50% of patients with "pure" PCD do not have circulating anti-neuronal AB. In the cases, the primary cancer is more often known and the clinical course of the cerebellar syndrome may be slower. Cerebellar degeneration may also occur during paraneoplastic encephalomyelitis. In this setting, the cerebellar signs which may be isolated at the onset, become associated with other signs of neuraxis involvement (limbic encephalitis, brainstem encephalitis, myelitis and particularly, subacute sensory neuronopathy) during the course of the disease. When a paraneoplastic encephalomyelitis is associated with a small cell lung cancer, an antineuronal AB called anti-Hu is frequently found. Finally PCD may be associated with the opsoclonus-myoclonus syndrome with the Lambert-Eaton syndrome.
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PMID:[Paraneoplastic cerebellar degeneration]. 786 50

We conducted the current review of the paraneoplastic neurologic syndromes (PNSs) associated with gynecologic and breast carcinomas to describe their clinical and immunologic characteristics and their relative frequency. We retrospectively reviewed 92 patients whose serum was sent to our laboratories to detect onconeural antibodies and who were diagnosed as having PNSs associated with breast or gynecologic tumors. PNSs were defined as "definitive" and "possible" (atypical PNS, no onconeural antibodies, and no improvement after tumor treatment). Forty-nine patients had breast and 43 had gynecologic cancer. Sixty-three patients had onconeural antibodies (50 Yo-ab, 5 Hu-ab, 5 Ri-ab, and 3 amphiphysin-ab). Cerebellar ataxia represented 57 (62%) of all PNSs and was associated with anti-Yo in 88%. All Yo-abnegative patients had breast cancer; 4 of them had a mild cerebellar syndrome that improved after tumor treatment. Sensorypredominant neuropathies were present in 17 (18%) patients. Seven of them had Hu-ab (5) or amphiphysin-ab (2). Other PNSs were opsoclonus-myoclonus syndrome (4 cases, Ri-ab in 2), sensorimotor neuropathy (4 cases), paraneoplastic encephalomyelitis (4 cases, Ri-ab in 3), paraneoplastic retinopathy (2 cases), amyotrophic lateral sclerosis (2 cases), stiff-person syndrome (1 with amphiphysin-ab), and limbic encephalitis (1 case). All patients with gynecologic cancer presented definitive PNS, and onconeural antibodies were diagnosed in 93% of them. In contrast, 20% of PNSs associated with breast cancer were defined as possible and the incidence of onconeural antibodies was 51%, excluding the 2 patients with paraneoplastic retinopathy in whom antiretinal antibodies were not analyzed. In patients with possible PNS, a coincidental association between the tumor and the neurologic disorder cannot be excluded.
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PMID:Spectrum of paraneoplastic neurologic disorders in women with breast and gynecologic cancer. 1279 7

Tick-borne borreliosis (Borrelia burgdorferi) is a common and complex disorder affecting the skin, the joints and the nervous system. It progresses through different clinical stages. The clinical spectrum of neuroborreliosis has expanded since the introduction and widespread application of specific serological tests. We have investigated 41 patients with Bannwarth's meningopolyneuritis (MPN) as the classical form of neuroborreliosis, in a prospective (26 patients) and a retrospective (15 patients) study. When questioned, 19/41 patients reported a tick bite and only 15/41, erythema migrans as the characteristic early skin lesion. In 34/41 patients typical MPN characterized by painful radiculoneuritis and/or cranial neuritis, especially facial palsy, were seen. Among these, 3 had a complicated form with a progressive remitting relapsing course or focal central nervous system involvement (hemiparesis, cerebellar syndrome); 2 had mild meningitis and facial nerve palsy bilaterally without radicular pain; and in 5 radicular pain was the only symptom. MPN associated with Lyme arthritis was observed only once. In 2 patients in the retrospective study (no antibiotics in the acute stage) we saw a chronic spinal cord disorder with spastic paresis several years after uncomplicated MPN, accompanied in 1 of them by acrodermatitis chronica atrophicans (ACA), the typical late-onset borrelia-induced dermatosis. In the acute stage of the disease 40/41 patients had a cerebrospinal fluid (CSF) syndrome compatible with MPN (mononuclear pleocytosis, blood-brain barrier dysfunction, elevated IgG and/or oligoclonal bands). IgG antibody titers against borrelia antigen were elevated in all patients in the serum and in 21/30 also in the CSF. In all patients pain was an early and prominent symptom; the first symptoms are usually felt in the region of the tick bite or the erythema, initially as diffuse myalgia, arthralgia or pain in the connective tissue. In the further course the migrating pain becomes more radicular in character, without being limited to defined dermatomic areas or peripheral nerves. The intense, burning pain is characterized by exacerbation during the night. Peripherally and centrally acting analgesics have only minor effects. Often neurological deficits are still absent at this time. Erythema migrans with radicular pain in the region of the dermatological lesion was observed in 2 patients. This is an early manifestation of MPN. After MPN and/or Lyme arthritis a sympathetic reflex dystrophy (SRD) developed in 2 patients. In a further patient SRD was observed right at the beginning of the illness, immediately before MPN. There is a close clinical similarity between SRD and the acute stage of ACA. Therefore, borreliosis can be assumed to produce a painful skin dystrophy like SRD or ACA by direct injury to the sympathetic nerves even in the early clinical stage of the infection. The main conditions to be considered in the differential diagnosis are polymyalgia rheumatica; lumbar disk herniation; inflammatory radiculopathies of other origin (e.g. herpes zoster); painful neuropathies, including the diabetic thoraco-abdominal form; internal disorders of chest and abdomen with referred pain; lymphocytic meningitis of other origin, encephalomyelitis; and sympathetic reflex dystrophy. High-dose penicillin G i.v. is a potent analgesic in all patients with tick-borne neuroborreliosis.
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PMID:[Pain syndromes in tick-borne neuroborreliosis. Clinical aspects and differential diagnosis.]. 1841 56

Paraneoplastic retinopathy including cancer-associated retinopathy (CAR) and melanoma-associated retinopathy (MAR), and paraneoplastic optic neuropathy (PON) are visual disorders associated with systemic cancer. Patients with CAR typically present with progressive loss of vision and photopsia, which are related to dysfunction of both cones and rods in photoreceptors. The triad of photosensitivity, ring scotoma, and a reduced caliber of the retinal arteriole along with undetectable signals in electroretinogram (ERG) are specific manifestations of CAR. CAR is associated most commonly with small-cell lung cancer (SCLC) and occasionally with gynecologic tumors, and it is usually caused by autoantibodies against recovering, which is a calcium-binding photoreceptor protein that participates in the transduction of light. MAR is characterized by shimmering, flickering, or pulsating photopsias, and usually occurs in the patients with a cutaneous melanoma. MAR differs from CAR in terms of visual acuity and color vision and is associated with a characteristic pattern detected in ERG. Autoantibodies against the bipolar cells of the retina have been identified in patients with MAR. Patients with PON frequently present with progressive visual loss and optic disc edema, or with other paneoplastic neurologic syndromes related to SCLC, such as paraneoplastic encephalomyelitis or retinitis, ophthalmoplegia, and subacute cerebellar syndrome. Autoantibodies against collapsin-responsive mediator protein-5 (CRMP-5, also called anti-CV2) are considered to be as the causative factor. Treatments with corticosteroids, plasma exchange, and intravenous immune globulin as well as treatment of the tumor itself, occasionally improves these paraneoplastic visual syndromes. However, the prognosis depends on their underlying malignancy.
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PMID:[Paraneoplastic retinopathy and optic neuropathy]. 2042 Jan 77

Sepsis-associated encephalopathy (SAE) is a diffuse brain dysfunction due to a systemic response to infection. We report the case of a 4-year-old girl with fever and vomiting for 48h, brought to the university hospital of Grenoble because of vigilance disorders, loss of verbal fluency, and a cerebellar syndrome. She had a biological infectious syndrome. Infectious encephalitis was suggested first, but the cerebral scan and the lumbar punction were normal. Magnetic resonance imaging (MRI) showed a diffuse brain edema with extended involvement of cortical and basal ganglia. The electroencephalogram was globally slow. The infectious syndrome was explained by perforated appendicitis with peritonitis, treated by surgery and antibiotic therapy. Other infectious explorations were negative. No metabolic or autoimmune diseases were found. Hence, our final diagnosis was sepsis-associated encephalopathy. After 1 year of follow-up care, her clinical exam, MRI, and EEG were normal. Sepsis-associated encephalopathy has been increasingly described in the adult population, but until today only three pediatric cases have been published. It is diagnosed when the patient has a severe infectious syndrome associated with neurologic symptoms, mostly vigilance or consciousness disorders, no signs of shock, and only when other potential reasons have been ruled out. The MRI shows non-specific diffuse lesions with vasogenic edema on the subcortical substance or on the basal ganglia and the thalami. The electroencephalogram is slowed down on the whole. The main differential diagnoses are infectious encephalitis, acute disseminated encephalomyelitis, and cerebral vasculitis. Posterior reversible encephalopathy syndrome is an MRI diagnosis that presents characteristics similar to SAE. In the future, it could be discovered that it is the same physiopathology. At the moment, we only treat the symptoms and the causative infection. Most of the time, patients have neurologic sequelae that affect their verbal fluency. It can persist from a few months up to 6yrs. Although quite slow, the neurologic progression is good. The mechanisms are studied and there are hopes for specific treatments. The main explanation seems to be immune with alterations of the blood-brain barrier. Cytokines and activated leukocytes may attack the cerebral substance.
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PMID:[A child with sepsis-associated encephalopathy]. 2395 25