Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 11-year-old boy of short stature had recurrent right temporal pounding headaches of 7 months' duration, and progressive visual loss for 3 days. There was a left hemianopia, alexia without agraphia, and diffuse muscle weakness. Investigation established the presence of a mitochondrial myopathy with pyruvate and lactic acidemia and increased serum content of sarcoplasmic enzymes. On treatment with prednisone, the patient's strength and reading skill improved, symptoms resolved, and muscle enzymes returned to normal. Three attempts to reduce steroids resulted in accentuation of symptoms, seizures, weakness, regression of reading skills, and elevation of serum enzymes. The alexia was also reversible.
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PMID:Reversible alexia, mitochondrial myopathy, and lactic acidemia. 57 70

Frontal agraphia has always been a subject of interest, although very few documented case reports have been published. A 57 year old male clerk, suffering from adenocarcinoma of the signoid colon, presented with mild headache and difficulty in writing. Minor features of dysphasia could be detected, but there were no alexia, agnosia or apraxia. Brain scan and angiography confirmed a metastasis in the posterior part of the left prefrontal region. The patient was able to write single letters, and had no difficulty with automatic writing or copying. The writing of words and of sentences was more affected. After a brief review of the literature and discussion, the authors concluded that frontal agraphia is related to frontal lobe diorders, especially the inability to translate verbal signals into motor behaviour (i.e. writing).
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PMID:Frontal agraphia, (including a case report). 121 2

We report here a case of multiple sclerosis (MS) showing pure alexia. The patient was a 28 year-old, right-handed male student of medicine. He had been healthy prior to college, but when he tried to read the newspaper one morning in January of 1983, he suddenly became aware that he could not. He could speak fluently and had no disturbances of auditory comprehension. No signs of abnormality were noted in his writing ability. Thereafter, the patient occasionally experienced difficulty in reading, together with right homonymous hemianopsia. These symptoms, however, usually vanished following sleep. In April, 1984, the patient experienced headache and ataxia when walking, followed by repeated remission and exacerbation of the symptoms. Corticosteroid therapy produced complete disappearance of the headache and ataxia. In September of 1985, he was hospitalized at our department due to the clouding of consciousness accompanied by convulsions. Neurologically, no abnormalities of the ocular fundus were noted and Babinski's sign was negative, but left hemiplegia was observed. Neuropsychologically, his intelligence was normal and he had no difficulty in writing, but paralexia was noted. There were no disorders of spontaneous speech or auditory comprehension. Examination by CT scan and magnetic resonance imaging revealed a number of lesions in the white substance of the cerebrum, including the left occipital lobe. The abnormal signs seen radiographically vanished when cortical hormones were administered. It has been thought that the symptoms of multiple sclerosis are due mainly to disorders of the white substance, and that MS seldom produces symptoms of aphasia, due to the fact that the lesions in this disease are generally small.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of multiple sclerosis with pure alexia]. 260 43

Marked non-communicating hydrocephalus may rarely cause ventricular rupture producing either a dilated cystic cavity (ventricular diverticulum) or communication between ventricle system and subarachnoid space (spontaneous ventriculostomy). Ventricular diverticulum has been believed to be collection of cerebrospinal fluid which escaped beneath the pia mater after rupture of ependymal layers and cerebral parenchyma. We proposed herein to report a case of subtentorial ventricular diverticulum which accompanied with choroid plexus papilloma of the lateral ventricle. A nine year-old girl admitted to our hospital complaining of clumsiness of hands and walking, disability of reading, headache and vomiting. The neurological examination revealed alexia, papilledema, anisocoria, righ hemianopsia, weakness of right upper limb, and cerebellar ataxia. CT brain scan showed a large high density area at the trigone of the left lateral ventricle with non-communicating hydrocephalus and an extra-axial low density area in the posterior fossa. The medial space of the left trigone was especially ballooned and is just shifted above incisura tentoria by the tumor. The intraventricular tumor was totally removed by operation and proved to be benign choroid plexus papilloma microscopically. The subtentorial mass was confirmed to be a cyst contiguous to the medial trigone of the lateral ventricle, namely ventricular diverticulum. Three special features were recognized in this case. The first, this was the first example of ventricular rupture accompanied with tumor in the lateral ventricle reviewing all reports of both ventricular diverticulum and spontaneous ventriculostomy. The second, this ventricular diverticulum was not produced merely by hydrocephalus alone but mainly by direct effects of the tumor.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of subtentorial ventricular diverticulum accompanied with choroid plexus papilloma in the lateral ventricle]. 310 97

A 36-year-old homosexual man with 6 months of visual symptoms and headaches had right homonymous hemianopia, mild new learning impairment, and alexia with agraphia. The initial brain magnetic resonance imaging (MRI) scan was reported consistent with left occipital infarction. Subsequent MRI demonstrated abnormal demyelination in subcortical white matter and deep parieto-occipital white matter bilaterally, but primarily left. Human immunodeficiency virus testing and cerebrospinal fluid polymerase chain reaction for JC virus DNA were both positive, consistent with progressive multifocal leukoencephalopathy (PML) with AIDS. His clinical status steadily deteriorated, and MRI white matter abnormalities worsened despite high-dose antiretroviral therapy. After the antiretroviral regimen was intensified by the addition of a protease inhibitor, rapid clinical and radiographic improvement occurred with subsequent MRI studies revealing only residual left parieto-occipital encephalomalacia. PML in AIDS patients has been associated with a nearly uniformly poor prognosis until recent reports of improved outcomes after highly active antiretroviral therapy. This patient with PML and AIDS similarly showed a robust clinical and MRI response to intensive antiretroviral combination therapy, which has been maintained for more than 3 years.
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PMID:Clinical and magnetic resonance imaging regression of progressive multifocal leukoencephalopathy in an AIDS patient after intensive antiretroviral therapy. 1146 8

Alexia without agraphia is a rare disconnection syndrome characterized by the loss of reading ability with retention of writing and verbal comprehension. We report a patient who developed alexia without agraphia after undergoing a biopsy for a malignant glioma involving the left thalamus. A 15-year-old right-handed male presented with 3 days of severe headache, and vomiting, and 1 month of blurry vision in his right visual field. Magnetic resonance imaging of the brain disclosed a large exophytic mass originating in the left thalamus, with mass effect and hydrocephalus. The patient underwent biopsy of the left thalamic mass via a transcallosal approach. Postoperatively, the patient complained of inability to read or identify letters. Examination revealed alexia without agraphia. The syndrome of alexia without agraphia can be rarely caused after surgery. A transcallosal procedure through the splenium of the corpus callosum may disrupt the visual association fibers traveling from the right occipital cortex to the left angular gyrus. In our case the syndrome occurred because of a preexisting right homonymous hemianopia resulting from a left thalamic tumor.
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PMID:Alexia without agraphia following biopsy of a left thalamic tumor. 1498 10

A 75-year-old woman with a dural arteriovenous fistula (DAVF) presented with progressive cognitive impairment including amnesia and alexia. Neuroradiological studies showed a relatively confined DAVF lesion in the left temporal lobe. The patient did not have a history of trauma and did not complain of headache or tinnitus. Amnesia and alexia dramatically improved upon treatment of the DAVF, and this was associated with attenuation of an abnormal MRI signal in the left temporal lobe. The results suggest that gradually impaired cerebral circulation due to focal venous hypertensive encephalopathy localized to the left temporal lobe and resulting from a DAVF could be involved in slowly progressive amnesia and alexia. The case also shows that an intracranial DAVF may present as a variety of neurological symptoms, depending on its localization, size and clinical stage.
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PMID:Focal dural arteriovenous fistula (DAVF) presenting with progressive cognitive impairment including amnesia and alexia. 1770 15

This study examined a 24-year-old patient with delayed encephalopathy, who was admitted to hospital with complaints of headache and visual impairment 1 week after acute carbon monoxide poisoning. The results of a visual field assessment, electroencephalography and head magnetic resonance imaging indicated damage to the cerebral cortex. After a 2-week treatment period, the patient had recovered from the visual impairment, but exhibited digit- and letter-reading difficulty. The Chinese aphasia battery and the number and letter battery supplement were conducted. The results revealed that the patient exhibited digit and letter alexia, while the ability to read Chinese characters was preserved. In contrast, the patient exhibited a deficit in Chinese character writing, while number and letter writing remained intact. Following treatment, reading and writing ability was improved and electroencephalographic abnormalities were ameliorated. Overall, our experimental findings demonstrated that delayed encephalopathy following acute carbon monoxide poisoning was characterized by digit and letter alexia.
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PMID:Digit and letter alexia in carbon monoxide poisoning. 2565 9

A 41-year-old right-handed woman presented abnormal behavior two weeks after suffering from headache and fever. Anti-glutamate receptor antibodies in the serum and cerebrospinal fluid were positive and we diagnosed anti-glutamate receptor antibody-related encephalopathy. The patient improved after administration of corticosteroid and was discharged without neurological deficit. After discharge, pure alexia and foreign accent syndrome-like language disturbance appeared consecutively. The serial fluorodeoxyglucose-positron emission tomography scans suggested that pure alexia and FAS-like language disturbance may have been caused by low function of the occipital lobes and the left frontal lobe, respectively. FAS has been linked to various lesions in the brain. The background mechanism may therefore be heterogeneous. On the other hand, patients with this syndrome recover spontaneously with our case. FAS may therefore be a temporal phenomenon resulting from imbalance in language processing rather than a specific deficit.
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PMID:[A case of pure alexia and foreign accent syndrome following acute encephalopathy in the presence of anti-glutamate receptor antibodies]. 2628 58