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)

A 10 year old boy, who was thought to have had a traumatic intracranial hemorrhage, was transferred to our Children's Medical Center. In spite of conservative treatment, he developed dysarthria, systemic convulsions, unconsciousness, quadriplegia, and consecutive paralysis of the cranial nerves. Magnetic resonance imaging scans demonstrated areas of increased signal intensity around the brain stem. The cerebrospinal fluid (CSF) contained a few large cells with abundant melanin-like granules, and numerous bizarre cells. The latter were considered to be malignant melanoma cells on immunocytological examination. Chemotherapy with dimethyltriazenoimidazole carboxamide (DTIC) and interferon beta (IFN-beta) was ineffective and he expired. Autopsy revealed diffuse infiltration of malignant melanoma cells into the meninges. We think that immunocytological examination of CSF is advisable for correct and rapid diagnosis.
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PMID:Meningeal malignant melanoma in a child: immunocytological diagnosis. 162 23

A 32-year-old male patient with chronic myelocytic leukemia in accelerated phase received a bone marrow allograft from his 42-year-old HLA/MLC-identical sister. He recovered from acute graft-versus-host disease (GVHD) grade III-IV of skin, liver and gut, but chronic GVHD of progressive onset developed. On day 556 post-graft severe thrombocytopenia was resistant to prednisolone, cyclophosphamide and high dose immunoglobulin. Splenectomy was followed by a normalization of platelet counts. The subsequent clinical course was characterized by progressive muscular atrophy and weight loss. Dysphagia, dysarthria, cachexia and ultimately recurrent pneumonic episodes ensued. The cachectic patient developed a highly abnormal breathing pattern with hypoventilation and intermittent apnea requiring mechanical ventilation. Auditory evoked potentials revealed a considerable dysfunction of the brainstem. The patient died on day 1120 post-graft from pneumonia, aggravated by thoracic muscular insufficiency. Postmortem examination revealed diffuse predominantly lymphoid perivascular infiltration in meninges and CNS tissue; proliferation of activated microglial cells expressing the HLA-DR antigen was prominent in the brainstem. These histologic changes are similar to those observed in the CNS in experimental GVHD. We suggest that this case represents the first documentation of CNS involvement in chronic GVHD.
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PMID:Fatal encephalitis in a patient with chronic graft-versus-host disease. 239 Jun 33

A rare case of glioblastoma fed by meningeal branches of the external carotid artery was reported. A 63-year-old female was transferred to our hospital suffering from gait disturbance and dysarthria. CT and MRI revealed brain tumor and paratumoral hemorrhage with a large cyst that was heterogeneously enhanced and existed in the right fronto-temporal region. Right external carotid arteriography demonstrated the tumor stain markedly fed by the right middle meningeal artery and the accessory meningeal artery. Subtotal removal operation was carried out uneventfully using the right fronto-temporal craniotomy. The histological diagnosis was glioblastoma. After the operation the patient was in good condition, and was transferred to another hospital for the purpose of the synchronized chemoradiotherapy. It is well known that any glioma invades the meninges. However, we rarely encountered an intra-axial glioma fed by a meningeal blood supply. A meningeal-invaded glioma may make difficult its differentiation from meningioma. We concluded that there is necessity for close examination of the intra-axial brain tumors invaded and fed by meningeal blood supply.
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PMID:[Glioblastoma fed by meningeal branches of the external carotid artery: a case report]. 1036 56

A rare case of sarcoid meningoencephalitis with no systemic lesion is reported here. A 58-year old man was admitted experiencing dull headache and speech disturbance. He had never received a diagnosis of systemic sarcoidosis. On admission, neurological examination revealed dysarthria, a defect of the right-side visual field and accelerated right Achilles tendon reflex. A T2-weighted MRI showed a high-intensity signal in the white matter of the left parieto-occipital lobe surrounded by severe brain edema with a mass effect. The meninges around the lesion were enhanced by gadolinium, but no enhancement was observed in the basal portion. Angiotensin-converting enzyme (ACE) activities of cerebrospinal fluid (CSF) and serum were within normal range. The level of interleukin-6 in the CSF was slightly elevated. Chest X ray films and chest CT revealed no abnormal lesions. Whole body gallium scanning showed a hot region only in the intracranial lesion. A brain biopsy was performed. Histological examination revealed typical granuloma of sarcoidosis accompanied by microvasculitis and epithelioid cell granuloma without caseous necrosis. Oral administration of prednisolone improved all symptoms and MRI findings. These observations suggest that release of cytokines from macrophages and epithelioid cells, as well as disruption of the blood-brain barrier due to microvasculitis, are involved in the mechanism responsible for producing lesions of sarcoid meningoencephalitis.
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PMID:[A case of sarcoid meningoencephalitis with an isolated supratentorial lesion]. 1125 86

We report the case of a 23-year-old male patient who suddenly developed right hemiparesis, cerebellar ataxia, dysarthria, and bilateral dysmetria. Brain magnetic resonance (MR) examination demonstrated hyperacute ischaemic lesions within the pons. CSF analysis revealed a high protein content, lymphocytic pleocytosis, and oligoclonal IgG bands not present in the serum. Elevated IgM and IgG anti-Borrelia burgdorferi antibodies were shown in both serum and CSF samples, associated with an intrathecal synthesis of these antibodies. Ischaemic CNS lesions have been rarely observed as the first manifestation of Lyme neuroborreliosis. The putative mechanism for parenchymal ischaemia is the local extension of inflammatory changes from meninges to the wall of penetrating arterioles.
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PMID:Acute ischaemic pontine stroke revealing lyme neuroborreliosis in a young adult. 1911 74

Sarcoidosis is a chronic disease of unknown aetiology. Neurosarcoidosis is registered in 5% of patients with sarcoidosis. Clinical manifestations of sarcoidosis are numerous and diverse. Manifestation of Neurosarcoidosis includes partial- and grand-mal seizures, low-grade fever, headache, increased intracranial pressure, visual disturbances, diabetes insipidus, amenorrhea- galacterorrhea syndrome and pituitary failure, hypogonadotropic hypogonadism, hyperprolactinemia, unilateral and bilateral facial palsy, infiltration of meninges (aseptic meningitis) and nerve roots, leptominingitis, pachymeningitis with cranial neuropathies, pseudotumor, mild cognitive disorder, psychosis, delirium, dementia, disorientation, amnesia, progressive visual deterioration and proptosis, axonal polyneuropathies, mononeuropathies, chronic polyradiculoneuritis, peripheral neuropathy, cranial nerve abnormalities, radiculopathies, peripheral neuropathy, mononeuritis multiplex, progressive numbness and deep sensation disturbance in bilateral lower extremities, hemiplegia, hyperreflexia with pathological reflexes and hypesthesia, upward gaze palsy, spinal cord compression, dysarthria, dysphagia, weakness, episodes of blurred vision, diplopia, intracerebral hemorrhage, neuro-ophthalmic manifestations, intranuclear ophthalmoplegia, dysorientation, vasculitis presenting with strokes, intracranial hypothalamic lesion, paresthesis, hemiparesis, myelopathy in the cervico-thoracic region, lumbar pain, sensory level and inability of lateral gaze (Tab. 2, Ref. 60).
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PMID:Clinical manifestations of neurosarcoidosis. 1982 43

Central nervous system involvement in rheumatoid arthritis is infrequent. Inflammatory lesions described in the literature are limited to rheumatoid nodules and vasculitis. We report on a 71-year-old woman who presented with a 1-month history of headache and dysarthria, and who had suffered seropositive rheumatoid arthritis without extra-articular complications, for 15 years. Magnetic resonance imaging showed a high-intensity image in FLAIR-weighted sequences in the right cerebral hemisphere, with meningeal gadolinium enhancement. A brain biopsy revealed necrotizing and lymphocytic vasculitis in the meninges as well as cerebral parenchyma. The patient received treatment with high-dose intravenous methylprednisolone with radiological improvement at 6-month follow-up. She remained neurologically asymptomatic in subsequent years. Three years after the onset of neurological symptoms, she was admitted to the hospital with choluria and jaundice. On the fiftieth day of hospitalization, she died from bronchopneumonia. The autopsy showed no signs of vasculitis. Cerebral vasculitis is an infrequent complication in RA. High-doses of intravenous corticosteroids may be an effective treatment.
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PMID:Isolated cerebral vasculitis associated with rheumatoid arthritis. 2047 84

A 44-year-old woman with a history of transient right hemiparesis presented with personality change. One year later, she was admitted with ophthalmoparesis, dysarthria and regression phenomenon. MRI indicated acute infarction of the paramedian region of the midbrain and a nodular lesion in the interpeduncular fossa with contrast enhancement. Two years later, the patient was admitted with sudden onset of right hemiplegia. MRI showed acute infarction in the left side of the pons, diffuse brain atrophy, and abnormal contrast enhancement in the nodular lesion of interpeduncular fossa and leptomeninges of the ventral pons. MR angiography revealed that cerebral main tracts were intact, and cerebrospinal fluid analysis revealed mild pleocytosis and slightly elevated protein levels. Cervical lymph node biopsy demonstrated caseating granuloma with acid-fast bacilli. The patient was diagnosed with chronic tuberculous meningitis, even though tuberculous bacilli were not detected on polymerase chain reaction (PCR) or in culture. Antituberculous medication resulted in radiological resolution and neurological improvement. Although the patient had mild headache and pyrexia at the first admission, no signs of meningeal irritation were confirmed throughout the clinical course. We suspect that a paucity of tuberculous bacilli released from the tuberculous foci in the meninges to the subarachnoid space caused prolonged clinical course and lack of meningeal irritation signs.
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PMID:[Chronic tuberculous meningitis presenting recurrent brainstem infarction without features of meningitis]. 2470 35

Vogt-Koyanagi-Harada disease is a rare multisystemic granulomatous autoimmune disorder affecting pigmented tissues such as the choroid, meninges, inner ear, and the skin. Neurologic symptoms are usually mild. Clinical manifestations include generalized muscle weakness, headache, meningismus, vertigo, decreased visual acuity, hearing loss and mental changes ranging from mild confusion to psychosis, hemiparesis, dysarthria, and aphasia. Seizures are very rare. We describe a case of 18F-fluorodeoxyglucose (F-18 FDG) positron emission tomography (PET) and software-fused PET-magnetic resonance imaging (MRI) in Vogt-Koyanagi-Harada disease with seizure.
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PMID:F-18 FDG PET Findings for Vogt-Koyanagi-Harada Disease. 2855 46

Primary central nervous system lymphoma (PCNSL) is a very rare tumor of increasing incidence. It is often misdiagnosed due to the unspecific presentation or unavailable biopsy, and results in poor prognosis. PCNSL involved the spinal cord is extremely sparse. Here we report a gentleman presented with one-year history of progressive tremor in the left limbs and slight dysarthria as well as three-month history of paraparesis, tinnitus and insomnia. MR images disclosed the swollen cerebellum and cauda equine, with contrast enhancement in both meninges and nerve roots. The cerebrospinal fluid (CSF) revealed extremely high protein level. Tubercular meningitis was considered and anti-tuberculosis therapy was given for weeks but without relief. With progressive deterioration, the PCNSL was eventually presumed according to positive CSF cytology and exclusion of systemic involvement. However, the patient passed away within days. We then reviewed the current diagnostic methods of PCNSL. The biopsy, as the gold standard for PCNSL diagnosis, is not eligible for all patients suspected PCNSL. The presurgical diagnostic algorithm of PCNSL has been fixed by clinicians and we suggest the early and repeated CSF cytology should be included for definitive diagnosis.
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PMID:Spinal primary central nervous system lymphoma: Case report and literature review. 2942 85


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