Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
 3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients, aged 24 and 19 years, who had undifferentiated lymphoma, developed the acute onset of focal neurologic deficits 10 days after treatment with moderate-dose methotrexate (2.76 g/m2 by 42-hour intravenous infusion) and 12.5 mg of intrathecal methotrexate. Prior chemotherapy also included intravenous cyclophosphamide, doxorubicin, vincristine, oral prednisone, and intrathecal cytosine arabinoside. Dysarthria with left hemiparesis was noted in one patient and inability to speak and quadriparesis in the other. Cerebrospinal fluid and cranial computerized tomography results were normal; however, EEGs showed focal abnormalities in both patients. Full neurologic recovery occurred within 48 hours. These symptoms developed early in the course of treatment and have not recurred in one patient who continued to receive the same regimen. The mechanism for this neurologic dysfunction is unclear. A similar picture has been reported in patients receiving high-dose methotrexate (8-10 g/m2) for osteogenic sarcoma.
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PMID:Transient neurologic dysfunction following moderate-dose methotrexate for undifferentiated lymphoma. 647 34

Twenty-four patients with leukemia or lymphoma refractory to conventional chemotherapy were given a course of systemic, high-dose cytosine arabinoside (3 gm/m2 every 12 hours for twelve doses). Four patients developed cerebellar degeneration during treatment. Ataxia of gait and limb movements, dysarthria, and nystagmus appeared five to seven days after the first dose, worsened over the next two to three days, and then remained stable for two to six days. Incomplete improvement occurred over the following one to two weeks. Postmortem examination disclosed loss of Purkinje cells in the depths of cortical sulci with relative preservation of those at the crests of folia and those in the most posterior inferior portions of the cerebellum. Other patients developed a mild, reversible cerebellar syndrome over the same time course as that of the irreversible disorder. Manifestations ranged from nystagmus alone to dysarthria and unsteadiness of gait without limb ataxia. We conclude that cytosine arabinoside at this dosage causes a cerebellar degeneration with characteristic clinical and pathological features. Among the present patients with refractory hematological malignancies, such degeneration occurred with an incidence of 16.7%, more than twice that reported in previous series.
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PMID:Cerebellar degeneration caused by high-dose cytosine arabinoside: a clinicopathological study. 665 Dec 39

Paraneoplastic cerebellar degeneration (PCD) is a rare manifestation of cancer, characterized clinically by subacute progressive ataxia, dysarthria and nystagmus. The pathological hallmark of PCD is a severe, diffuse loss of Purkinje cells. PCD occurs most frequently in association with small cell carcinoma of the lung and adenocarcinoma of the ovary, but it has also developed in patients with carcinoma of the breast, malignant lymphoma, and various cancers. Autoantibodies against cerebellar Purkinje cells have been frequently observed in the serum or cerebrospinal fluid (CSF) from patients with PCD. The cause of PCD is unknown, but the presence of these autoantibodies in some patients suggests that the pathogenesis may be immune mediated. The potential role of the autoantibody in the pathogenesis of PCD is discussed.
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PMID:[Paraneoplastic cerebellar degeneration]. 799 1

A 68 year old man with a 10 year history of apparently benign IgM kappa paraproteinaemia presented with dysarthria, left hemiparesis, and a sensory peripheral neuropathy. A calcified right temporoparietal extradural mass was shown by scintigraphy with 123I-serum amyloid P component to contain amyloid. There were no extracranial amyloid deposits. Clinical improvement followed craniotomy and partial resection of tissue which consisted of amyloid and a mixed mononuclear cell infiltrate. The amyloid fibrils consisted of the framework 1 region of the variable domain of monoclonal kappa IV immunoglobulin light chains. There was a prominent B-cell clonal immunoglobulin gene rearrangement in the tumour tissue, supporting a diagnosis of lymphoplasmacytic lymphoma, but no sign of systemic lymphoma. Neurological state, tumour volume, and quantity of amyloid have remained static for two years after treatment with chlorambucil.
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PMID:AL kappa amyloid in a solitary extradural lymphoma. 800 62

Neurological symptoms of transient unsteadiness, dysarthria, dysphasia, dysbasia, transient monoor hemiparesis, hemiparesis, scintillating scotomas, amaurosis fugax, vertigo, dizziness, migraine accompaniments, syncope and seizures were the presenting manifestations of thrombocythemia in various myeloproliferative disorders. Erythromelalgia preceded or followed the neurologic ischemic attacks. The neurologic and ocular attacks usually had a sudden onset, lasted for a few seconds to several minutes and occurred independently or sequentially rather than simultaneously. This clinical syndrome is caused by platelet-mediated ischemic and thrombotic processes in the end-arterial microvasculature and reflects the existence of a platelet dependent and aspirin responsive arterial thrombophilia in thrombocythemia as novel disease entity, which confirms and elucidates Mitchell's hypothesis.
Leuk Lymphoma 1996 Sep
PMID:Atypical transient ischemic attacks in thrombocythemia of various myeloproliferative disorders. 895 74

We report a 29-year-old HIV-positive patient admitted with dysarthria, ataxia and somnolence. Imaging findings were typical of Wernicke's encephalopathy, but autopsy revealed cytomegalovirus encephalitis and primary cerebral lymphoma.
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PMID:Cytomegalovirus encephalitis and primary cerebral lymphoma mimicking Wernicke's encephalopathy. 912 41

We report a patient with acute promyelocytic leukemia (APL) involving the central nervous system. A 55-year-old male was admitted to our hospital with dysarthria and incomplete right hemiplegia. A CT scan of the brain revealed a low density area in the left cerebrum. APL was diagnosed by bone marrow aspiration and chromosomal analysis. The patient received all-trans retinoic acid (ATRA) in combination with chemotherapy. Complete hematological remission (CR) was obtained, and the patient's neurological symptoms improved. However, a cytospin smear of the cerebrospinal fluid after CR showed immature myelocytes ("intermediate cells") that had possibly been derived from leukemic promyelocytes. Comprehensive intrathecal treatment as well as cranial irradiation, caused a further reduction in dysarthria and a complete disappearance of hemiplegia with no atypical cells in the cerebrospinal fluid. The patient has undergone maintenance chemotherapy as an out-patient.
Leuk Lymphoma 2000 Sep
PMID:Detection of acute promyelocytic leukemia (APL) cells intermediately differentiated by all-trans retinoic acid in the cerebrospinal fluid: central nervous system involvement in APL. 1097 2

Primary leptomeningeal lymphoma (PLML) is a rare disease. The most common presentation is symptoms of increased intracranial pressure. Confusion, dysarthria, hearing loss, paraparesis and lumbosacral spinal root symptoms have also been reported. Chemotherapy and radiotherapy have been tried, but its prognosis is usually poor. We experienced a case of PLML with a relatively benign course in an 18-year-old girl. Initial diagnosis was made as idiopathic intracranial hypertension. Lumbosacral shunt was done with good response for 3 years. When headache recurred, she was reevaluated and was correctly diagnosed as PLML.
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PMID:Primary leptomeningeal lymphoma with long-term survival: a case report. 1102 96

The development of central pontine myelinolysis (CPM) has rarely been reported in association with hemophagocytic syndromes (HPS). Here we report a unique case of Epstein-Barr Virus (EBV)-related HPS which was accompanied with CPM. A 72-year-old man who had no significant medical history was admitted to our hospital due to high fever and progressing dysphasia and dysarthria. Physical examination revealed anisocoria of the right pupil, fixed reaction to light, and paralysis of the left vagus nerves. Magnetic resonance imaging revealed low signal intensity on T1-weighted images and high signal intensity T2-weighted images in the patient's central midpontine lesion. Initial work-up showed anemia and thrombocytopenia with elevated levels of serum ferritin, lactate dehydrogenase, and soluble IL-2 receptor. Bone marrow aspiration revealed hemophagocytosis. The EBV genome was detected in the peripheral blood using the polymerase chain reaction method. He was diagnosed as having EBV-related HPS and CPM. Despite intensive treatment with methylpredonisolone, immunoglobulin, and etoposide, he died due to progressive disease and fungal septicemia. The etiology and relation between CPM and HPS are discussed.
Leuk Lymphoma 2002 Oct
PMID:EBV associated hemophagocytic syndrome accompanied by central pontine myelinolysis. 1248 7

We report a 49-year-old previously healthy woman with acute onset of decrease in attention, dysarthria and ataxia, accompanied by drowsiness. On admission, there were cloudness of consciousness, hallucination and left hemiparesis. Cerebrospinal fluid study revealed a cell count of 1/mm3, and the cytology was class I with a slight increase in protein. MRI of the brain performed on admission showed multiple gadolinium-enhanced lesions with a T2 weighted high intensity area in the cerebral white matter. At first the patient was diagnosed as acute disseminated encephalomyelitis (ADEM), and treated with methylprednisolon pulse therapy. Soon after, she showed transient clinical improvement, but her condition soon worsened. MR spectroscopy revealed elevated choline peak, decreased NAA peak and lactate peak, which indicated a neoplastic lesion. The brain biopsy disclosed diffuse intravascular lymphoma (IVL). MRS was useful in the differential diagnosis of IVL from ADEM.
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PMID:[MR spectroscopy findings of a case of intravascular malignant lymphoma: usefulness for differential diagnosis]. 1288 28


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