Cases reported "Paresis"

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1/15. Arteriovenous and lymphatic malformations, linear verrucous epidermal nevus and mild overgrowth: another hamartoneoplastic syndrome?

    We report a 22 year old female presenting with slowly progressive paraparesis, who appeared to have many (mainly subcutaneous) hamartomas. The neurological symptoms were caused by intraspinal masses and arteriovenous malformations. In addition, she had mild overgrowth of one leg and lymph vessel malformations. This combination of symptoms resembles proteus syndrome, but is different in symptomatology and progression and may be yet another hamartoneoplastic syndrome.
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2/15. Familial occurrence of cerebral arteriovenous malformation in sisters: case report and review of the literature.

    Cerebral arteriovenous malformations (AVMs) are considered to be congenital disorders. However, their familial occurrence has so far been described in only 19 families in the literature. The authors report on two cases in one family and review the literature. A 45-year-old female subject with sudden onset of headache and vomiting due to a subarachnoid haemorrhage from a small AVM in the posterior part of the corpus callosum near the midline on the left side was studied. Irradiation of the AVM using Leksell's gamma knife led to its complete obliteration. Her older sister presented with temporal seizures at the age of 49 and later also with left hemiparesis, left hemihypaesthesia and dizziness - caused by a large AVM in the right temporal lobe. This AVM was treated by a combination of embolization and irradiation by the Leksell's gamma knife.
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3/15. Postoperative confusion preceded by decreased frontal lobe haemoglobin oxygen saturation.

    We describe a 58-year-old male patient with confusion and prolonged recovery after liver transplantation. A cause was not apparent for the confusion, but during surgery, monitoring of the frontal lobe cerebral haemoglobin oxygen saturation by near-infrared spectrophotometry showed cerebral hypo-oxygenation despite optimization of conventional cardiovascular parameters. It is possible that intraoperative cerebral ischaemia is the cause of postsurgical confusion and with near-infrared spectrophotometry this hypothesis may be tested clinically.
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4/15. Interhemispheric intracranial pressure gradients in massive cerebral infarction.

    We report continuous bilateral intracranial pressure (ICP) monitoring immediately after transtentorial herniation in a patient with massive cerebral infarction to: 1) determine presence and time course of compartmental ICP differences, and 2) to study effects of therapy on both hemispheres. A 55-year-old man admitted with watershed infarctions in the left anterior-middle-posterior cerebral arteries distribution. Initial investigations demonstrated highly narrowed left extracranial internal carotid artery. Eight days later he developed unexplained lethargy and anisocoria. Head computerized tomography (CT) showed massive left hemispheric infarction, edema, and midline shift. Bilateral subarachnoid bolts demonstrated equally elevated ICP in both hemispheres. hyperventilation and osmotic therapy produced near-identical ICP reduction bilaterally with resolution of anisocoria. Later, plateau waves and autonomic instability developed. Shortly before loss of brainstem function, interhemispheric ICP gradients (left greater than right) of 30-40 mm Hg developed. intracranial pressure did not equalize prior to brain death determination. Bilateral ICP monitoring did not reveal an interhemispheric ICP gradient soon after transtentorial herniation in massive MCA infarction. The presence of interhemispheric ICP gradients in massive stroke remains unproven and further clinical study is necessary.
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5/15. Preoperative assessment of motor cortex and pyramidal tracts in central cavernoma employing functional and diffusion-weighted magnetic resonance imaging.

    BACKGROUND: Functional MRI (fMRI) combines anatomic with functional information and has therefore been widely used for preoperative planning of patients with mass lesions affecting functionally important brain regions. However, the courses of functionally important fiber tracts are not visualized. We therefore propose to combine fMRI with diffusion-weighted MRI (DWI) that allows visualization of large fiber tracts and to implement this data in a neuronavigation system. methods: DWI was successfully performed at a field strength of 1.5 Tesla, employing a spin-echo sequence with gradient sensitivity in six noncollinear directions to visualize the course of the pyramidal tracts, and was combined with echo-planar T2* fMRI during a hand motor task in a patient with central cavernoma. RESULTS: Fusion of both data sets allowed visualization of the displacement of both the primary sensorimotor area (M1) and its large descending fiber tracts. Intraoperatively, these data were used to aid in neuronavigation. Confirmation was obtained by intraoperative electrical stimulation. Postoperative MRI revealed an undisrupted pyramidal tract in the neurologically intact patient. CONCLUSION: The combination of fMRI with DWI allows for assessment of functionally important cortical areas and additional visualization of large fiber tracts. Information about the orientation of fiber tracts in normal appearing white matter in patients with tumors within the cortical motor system cannot be obtained by other functional or conventional imaging methods and is vital for reducing operative morbidity as the information about functional cortex. This technique might, therefore, have the prospect of guiding neurosurgical interventions, especially when linked to a neuronavigation system.
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6/15. Late onset seizures, hemiparesis and blindness in hemolytic uremic syndrome.

    Neurologic complications of hemolytic uremic syndrome, including seizures, usually occur early during the acute phase of the illness. We report a3-year-old girl with classic diarrhea-associated hemolytic uremic syndrome who developed late onset seizures, hemiparesis and transient blindness on the 17th hospital day, at which time her recovery was characterized by improvement in her blood pressure, serum electrolytes, renal function, hematocrit and platelet count. A CT and MR revealed brainstem and posterior parietal and occipital infarct/edema. The association of these radiologic findings within the posterior distribution along with visual loss and seizures are unique to posterior reversible encephalopathy syndrome. Within 7 days, she regained motor function and vision and had no further seizure activity. At 6 months follow-up, physical examination revealed normal motor function and vision and a repeat MR showed near resolution of the previous findings with minimal occipital lobe gliosis. This case report describes the uncommon finding of late onset seizures occurring during the recovery phase of hemolytic uremic syndrome with MR findings consistent with posterior reversible encephalopathy syndrome.
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7/15. A variant of multifocal motor neuropathy with acute, generalised presentation and persistent conduction blocks.

    OBJECTIVE: Multifocal motor neuropathy with persistent conduction blocks is classically described as a chronic neuropathy with progressive onset, and acute forms have not previously been characterised. We report four cases of severe motor impairment with acute and generalised onset and with persistent motor conduction blocks. patients AND RESULTS: An acute tetraparesis with diffuse areflexia but little or no sensory disturbance was the clinical picture. Serial electrophysiological tests showed persistent multifocal motor conduction blocks with absent F waves in most tested motor nerves. No or minor abnormalities of the sensory nerve action potentials were observed. cerebrospinal fluid contained normal or mildly increased protein levels (<1 g/l) without cells. campylobacter jejuni serology was negative in three patients and consistent with past infection in one patient. Anti-ganglioside antibodies were positive in three patients. A five day course of intravenous immunoglobulins produced nearly complete symptom resolution in three patients and was ineffective in one patient. CONCLUSION: Because of the persistence of multifocal motor conduction blocks for several weeks or months as the isolated electrophysiological feature, these cases could not be consistent with guillain-barre syndrome or chronic inflammatory demyelinating polyneuropathy. They suggest an original variant of multifocal motor neuropathy with an acute and generalised initial presentation and persistent motor conduction blocks affecting all four limbs.
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8/15. Intraoperative cortical mapping has low sensitivity for the detection of motor function in proximity to a tumor in the primary motor area.

    Six patients with brain tumors within or near the primary motor cortex underwent preoperative functional magnetic resonance imaging (fMRI) and intraoperative cortical mapping, and the accuracy of those techniques for localization of the primary motor cortex and motor function beside the tumor were determined by comparison against neuroanatomical correlates from pre-, intra- and postoperative neurological observations. The location of the primary motor cortex was detected by intraoperative cortical mapping in 5 of 6 cases and by fMRI in all 6 cases. brain mapping provided equivocal information on the cortical representation of motor territories, and with the technique used in close proximity to the tumor, the motor territories were not detected in all but 1 case. In contrast, the areas controlling motor function in close proximity to the tumor were detected by fMRI in 4 of 6 cases.These data indicate that intraoperative cortical mapping has a low sensitivity for the detection of motor function in the area beside the tumor. Therefore, this technique may not be sufficient to prevent compromise of motor areas during tumor resection.
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9/15. Postictal spectroscopy and imaging findings mimicking brain tumor recurrence.

    (1)H magnetic resonance spectroscopic imaging (MRSI) was performed on a patient with an admission diagnosis of recurrent astrocytoma. The patient had undergone surgical resection and radiation therapy for a left occipital astrocytoma WHO grade III 12 years previously, and presented with aphasia, right-sided hemiparesis, and severe headache. Postcontrast T1-weighted images showed cortical enhancement of the left parietotemporal lobe near the post-resection cavity. MRSI revealed a marked increase of trimethylamines (TMA), elevated creatine/creatinephosphate (tCr), and reduced N-acetyl-aspartate (tNAA) in the same brain region. The spectroscopic data were consistent with tumor recurrence. However, the pattern of contrast enhancement on magnetic resonance imaging (MRI), evidence of an epileptic focus on electroencephalography (EEG), and spontaneous regression of the symptoms argued against tumor recurrence. In a 4-week follow-up, the contrast enhancement disappeared on MRI and the EEG abnormalities and neurological symptoms resolved. Follow-up spectroscopic data showed a decrease in TMA compared to normal values. The tCr signal remained elevated but returned to normal values after 5 months. In conclusion, postictal neurological deficits with a temporary increase in TMA and tCr were diagnosed. This is the first report of seizure-induced MRS abnormalities mimicking tumor recurrence.
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10/15. Restored activation of primary motor area from motor reorganization and improved motor function after brain tumor resection.

    BACKGROUND AND PURPOSE: Reorganization of brain function may result in preservation of motor function in patients with brain tumors. The goal of the present study was to investigate whether function of the primary motor area (M1) was restored and whether motor function improved after brain tumor resection. methods: Five patients with metastatic brain tumors located within or near M1 underwent awake surgery with intraoperative cortical mapping and continuous task monitoring. Preoperative and postoperative functional MR imaging (fMRI) was performed during hand clenching, and diffusion tensor imaging (DTI) was performed in 1 case to further characterize the area activated in fMRI. RESULTS: Preoperative fMRI performed during hand clenching demonstrated reorganization of motor function. In patients with severe paresis (cases 3, 4, and 5), clenching of the affected hand induced a large blood oxygen level-dependent response in the right hemisphere, mainly in the anterior temporal lobe, despite the location site of the tumor. Postoperative fMRI during hand clenching demonstrated activation of the contralateral M1. Furthermore, in case 5, DTI detected tracts, possibly the inferior longitudinal fasciculus, arising from anterior temporal activated area as well as tracts connecting the premotor and M1 activated area. This patient demonstrated mirror movement of the hand during the course of motor function recovery. CONCLUSIONS: Tumor resection resulted in restoration of M1 function and improved motor function in patients with preoperative reorganization of M1 function. Furthermore, the preoperative reorganization of motor function in cases with severe paresis may be related to changes in the right hemisphere, including the temporal lobe.
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