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 56-year-old woman attended our hospital because of acute severe (thunderclap) headache. Neurological examination was normal and no abnormality was found on head CT or by cerebrospinal fluid examination. A few days later, she experienced a recurrence and suffered a seizure in her left upper and lower extremities. On neurological examination, she had conjugate deviation of the eyes toward the right side and left lower limb paralysis with Chaddock sign. MRI showed multiple hyperintense lesions in the bilateral occipital and parietal lobes, predominantly in the subcortical white matter at the right side on T2-weighted and FLAIR images. We diagnosed posterior reversible encephalopathy syndrome (PRES) because the ADC map showed a vasogenic edema pattern (increased ADC values in the hypodense lesions on diffusion-weighted image). Her blood pressure was normal and there were no underling diseases. As MRA showed vasoconstriction especially in bilateral posterior cerebral arteries, we initiated a therapy with a Ca-channel blocker. On follow-up MRI, the hyperintense lesions on T2-weighted and FLAIR images had almost disappeared, and vasoconstriction was also improved on MRA. This case suggested that cerebral vasoconstriction could underlie both thunderclap headache and PRES.
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PMID:[Case of posterior reversible encephalopathy syndrome with cerebral vasoconstriction]. 1854 Mar 85

A case of visual hallucination, headache and left hemiparesis is reported. The patient had a history of recurrent attacks of similar semiology for the previous 15 years. MRI brain revealed a cortical hyperintensity on T2W, FLAIR and diffusion weighted imaging (DWI) in the right cerebral hemisphere with a normal ADC (apparent diffusion coefficient) map and MR angiogram. Detailed workup for MELAS was negative. A diagnosis of sporadic hemiplegic migraine was made and he was managed conservatively. He made a gradual complete recovery over 2 weeks. He was discharged on flunarizine for prophylaxis and has remained asymptomatic over the ensuing 4 months. This interesting condition is reviewed and discussed herein.
J Headache Pain 2008 Dec
PMID:Sporadic hemiplegic migraine: report of a case with clinical and radiological features. 1881 Mar 16

Vasculitides are characterized by inflammation and necrosis of the blood vessel wall. Large vessels including the aorta are affected in giant-cell arteritis, medium-size arteries in classic polyarteritis nodosa. The small-vessel vasculitides are separated in those with antineutrophil cytoplasm antibodies (ANCA) and those without. The primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on laboratory and imaging findings. When cerebral affection occurs in systemic vasculitis an acute inflammatory response with raised erythrocyte sedimentation rate and increased values of C-reactive protein is present. In many cerebral vasculitides including PACNS, CSF studies reveal inflammatory findings. Magnetic resonance imaging, including ADC maps, diffusion and gradient echo sequences, is the investigation of choice to detect and monitor cerebral involvement. Certain MRI techniques and 18-fluorodeoxyglucose positron emission tomography allow the visualization of vessel wall inflammation when the lumen is still unaffected on angiography. The treatment recommendations for cerebral angitis are derived from protocols for systemic vasculitides. In general, a combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. An alternative option is the use of the anti- CD20 antibody rituximab. Methotrexate, azathioprine and mycophenolate mofetil are recommended as alternatives to CYC once remission is achieved.
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PMID:Diagnosis and treatment of cerebral vasculitis. 2118 Jun 34

Here, we demonstrate a first case of tick-borne encephalitis (TBE) associated with an isolated reversible splenial corpus callosum lesion (IRSL) and highlight the wide range of different clinical entities in which such alterations have been observed. A 42-year-old man showed fever, cephalgia and mild disturbance of coordination and gait. Diagnosis was ascertained by slight CSF-pleiocytosis and positive TBE-IgG as well as by positive intrathekal specific antibody index on follow-up. MRI demonstrated a single ovoid hyperintensity in T2 and DWI with reduction in ADC in the splenium of corpus callosum which was abrogated in follow-up after 6 weeks. Most entities of IRSL presented with excellent prognosis, including our novel case of TBE. We discuss different possible pathomechanisms and the so far unexplained propensity of the splenium for such alterations. Clinicians should be familiar with this phenomenon to avoid unnecessary diagnostic or therapeutic efforts.
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PMID:Isolated reversible splenial lesion in tick-borne encephalitis: a case report and literature review. 2137 18

Familial Creutzfeldt-Jakob disease (CJD) with V180I shows different clinical characteristics from classical CJD and is difficult to diagnose in the early stage. We report a CJD180 patient in whom results of diffusion-weighted imaging (DWI) led us to suspect CJD before symptoms started. A 68-year-old woman presented to our hospital with headache and nausea and underwent magnetic resonance imaging. DWI showed cortical hyperintensity. Three months later, cognitive function started to decline and CJD180 was diagnosed following genetic examination. In the early stage, ADC values were not decreased and single positron emission computed tomography demonstrated a decreased pattern like Alzheimer disease.
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PMID:Early detection of familial Creutzfeldt-Jakob disease on diffusion-weighted imaging before symptom onset. 2264 Sep 3

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a maternally inherited disease due to mitochondrial DNA (mtDNA) point mutations. The clinical phenotype varies in relation to the systems affected, age at onset and disease severity. The characteristic signs of MELAS are nausea and vomiting due to acidosis, headache, epilepsy, ataxia or generalized weakness, ophthalmoplegia, motor and sensory focal neurological deficits. The clinical course may improve due to partial regression of the typical lesions, but the prognosis is usually adverse. A 19-year-old man with a diagnosis of benign occipital epilepsy and resumption of seizure activity with focal occipital attacks since the age of 14 years came to our attention for the recent onset of drug-resistant electroclinical seizures of long duration with complex symptoms, where the dominant clinical feature was an intense, persistent bilateral periorbital migraine with nausea and vomiting, scintillation scotomata and blurring of vision. MR studies were performed at our institution in the immediate post-seizure phase and then at one week, three and six months. The acute-phase morphological scans showed a right cortical-subcortical area with altered signal in the occipitopolar region that was hypointense on T1 and hyperintense on T2 and FLAIR, with cortical thickening and effacement of the sulci. Contrast-enhanced scans did not demonstrate BBB alterations. The DWI scans showed a right temporo-occipital cortical area with higher signal intensity. In the subsequent examinations the area with altered signal shrank gradually and significantly in parallel with improvement in clinical conditions. The diagnostic hypothesis of benign occipital epilepsy was consistent neither with the clinical course, characterized by persistent headache, visual disturbance and refractoriness to antiepileptic drugs, nor with the temporal-occipital cortical MR findings, which resembled ischemic lesions but displayed a non-territorial pattern as well as reversibility over time. These elements guided in the diagnosis of MELAS, which was subsequently confirmed by identification of the typical gene mutation. On DWI the stroke-like lesions of MELAS are seen more frequently as focal hyperintense areas compared with healthy parenchyma. Such high signal intensity likely corresponds to T2 shine-through rather than cytotoxic edema. Indeed, several studies have demonstrated that in acute-phase scans of MELAS stroke-like lesions DWI hyperintensity is associated with increased ADC values that are not associated with restricted water diffusivity, reflecting the metabolic rather than anoxic-ischemic nature of these changes. In the present case, morphological MR associated with DWI was very helpful in guiding the diagnosis by demonstrating some pathognomonic features of MELAS stroke-like lesions such as cortical-subcortical involvement of the posterior hemispheres, the non-territorial pattern, lesion reversibility and the pathophysiological role of vasogenic edema in inducing an increase in extracellular water and thus in diffusion values.
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PMID:Atypical Clinical Picture in a Patient with Benign Occipital Epilepsy: Diagnostic Contribution of Morpho-Functional MR. A Case Report. 2429 88

A 26-year-old male was admitted because of a fever, headache and disturbance of consciousness with lymph node swelling of the neck two days after developing a rash. A neurological examination revealed restlessness with irritability in response to sensory stimuli, such as an injection. Diffusion-weighted brain magnetic resonance imaging (MRI) revealed a hyperintense ovoid lesion in the splenium of the corpus callosum, which showed a low coefficient in the ADC map: the lesion disappeared after 22 days. An enzyme immunoassay (EIA) of the serum and cerebrospinal IgM were positive for rubella virus. The patient was therefore diagnosed with rubella encephalitis. He recovered gradually and was discharged on day 19 after the onset of symptoms without any sequelae. To our knowledge, this is the first case of rubella encephalitis presenting as clinically mild encephalitis/encephalopathy with a reversible splenial lesion (MERS). Although the exact mechanism underlying the development of rubella encephalitis is not well established, this case indicated that our patient had an immune-mediated secondary encephalitis. According to the survey of the pandemic of rubella from 2012 to April 2013 in Japan, the incidence of rubella encephalitis is thought to be relatively higher than was previously noted. This emphasizes the importance of vaccination for preventing encephalitis.
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PMID:[A case of rubella encephalitis presenting as clinically mild encephalitis/encephalopathy with a reversible splenial lesion]. 2514 41

A 44-year-old right-handed Caucasian male was initialy diagnosed in 2007 with dermatomyositis (DM) and in 2009 with systemic lupus erythematosus (SLE) (overlap syndrome). He was treated with Methylprednisolone and Hydroxychloroquine. He interrupted the treatment in the last three years. The patient presented with fever (39.8 degrees C), left zoster ophthalmicus, headache and psychomotor agitation. The cerebral CT scan showed left hemispheric hypodense lesions. Herpetic encephalitis was suspected. The patient was referred to the Institute of Infectious Diseases. The patient's neurological status worsened, he presented spastic tetraparesis and aphasia. DW-MRI, ADC, DS and AngioMRI were done, the patient proved to have an ischemic stroke due to acute thrombosis of the left internal carotid artery and multiple watershed infarctions. An infectious pathology, including HSV-1, was excluded by PLEX ID performed from CSF. Acyclovir, anti vitamin K, steroidal intravenous pulse therapy was started. The patient was referred after two weeks to the Department of Neurology. Mild inflammatory syndrome, tests for anti-double stain DNA (dsDNA), anti-Sm, anti-SSA, IgM and IgG anti-cardiolipin antibodies and lupus anticoagulant were positive. He was currently treated with Methylprednisolone (48 mg/d), anti vitamin K, statin, symptomatics. The outcome was favorable, with good laboratory response. Overlap syndrome may be associated with a significant increase in the risk of stroke. Our case presented without clinically susceptible symptoms of stroke but found to have stroke after neurological assessment associated with overlap syndrome (DM and SLE).
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PMID:Ischemic strokes in a young patient with dermatomyositis, systemic lupus erythematosus and secondary antiphospholipid syndrome mimicking herpetic encephalitis. 2550 61

This report presents the case of a 60-year-old woman who was diagnosed with stage IV lung adenocarcinoma with asymptomatic brain metastases and commenced chemotherapy with cisplatin/pemetrexed (CDDP/Pem). She experienced tonic-clonic convulsions on day 9 of the first cycle, which were accompanied by increased blood pressure (173/69 mm Hg) and headache. Therefore, brain MRI was performed to check for stroke or progression of brain metastatic foci. T2-weighted, FLAIR, and ADC map images showed high-intensity areas in the subcortical region of the bilateral parieto-occipital lobes, leading to a diagnosis of posterior reversible encephalopathy syndrome (PRES). The symptoms improved after treatment with antihypertensive and antiepileptic drugs. Clinicians should keep it in mind that central nervous system symptoms during anticancer therapy containing Pem may indicate possible PRES.
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PMID:A Case of Posterior Reversible Encephalopathy Syndrome Induced by Cisplatin/Pemetrexed Chemotherapy for Lung Cancer. 2861 37