MRI abnormalities associated with partial status epilepticus

OBJECTIVE: To report neuroimaging findings in patients with complex partial status epilepticus. BACKGROUND: During status epilepticus, neuroimaging may be used to exclude other neurologic conditions. Therefore, it is important to identify the neuroimaging features that are associated with status epilepticus. In addition, MRI characteristics may provide insight into the pathophysiologic changes during status epilepticus. METHODS: The history and neuroimaging examination results of three patients with complex partial status epilepticus were reviewed. Studies obtained during status epilepticus included diffusion-weighted MRI (DWI), MR angiography (MRA), postcontrast T1-weighted MRI, T2-weighted MRI, and CT. Follow-up MRI was obtained in two patients, and autopsy results were available for the third. RESULTS: Some of the MRI and CT findings during partial status epilepticus mimicked those of acute ischemic stroke: DWI and T2-weighted MRI showed cortical hyperintensity with a corresponding low apparent diffusion coefficient, and CT showed an area of decreased attenuation with effacement of sulci and loss of gray-white differentiation. However, the lesions did not respect vascular territories, there was increased signal of the ipsilateral middle cerebral artery on MRA, and leptomeningeal enhancement appeared on postcontrast MRI. On follow-up imaging, the abnormalities had resolved, but some cerebral atrophy was present. CONCLUSIONS: The radiologic characteristics of status epilepticus resemble those of ischemic stroke but can be differentiated based on lesion location and findings on MRA and postcontrast MRI. The MRI abnormalities indicated the presence of cytotoxic and vasogenic edema, hyperperfusion of the epileptic region, and alteration of the leptomeningeal blood-brain barrier. These changes reversed, but they resulted in some regional brain atrophy.     

EEG in dengue virus infection with neurological manifestations: a clinical and CT/MRI correlation.

OBJECTIVE: To evaluate electroencephalography (EEG) changes in dengue virus infection and correlate these with clinical, laboratory and CT/MRI changes. METHODS: Consecutive patients with dengue virus infection were prospectively evaluated. A detailed history and clinical examination were carried out. Level of consciousness was assessed by Glasgow Coma scale (GCS). Cranial CT scan or MRI or both were carried out. EEG was carried out within 24h of hospitalization and repeated after 15-30 days. Outcome was defined at 1 month into death, poor, partial and complete recovery. RESULTS: Twenty-four patients aged 5-65 years with dengue virus infection having neurological manifestations were included; of whom six were females. EEG revealed theta to delta slowing in 11 out of 20 patients. Five of these patients had seizures, 12 altered sensorium, 3 hypotension, 9 CSF pleocytosis, 3 moderate to severe liver dysfunction and 2 MRI abnormalities who had Japanese encephalitis virus coinfection. Presence of EEG slowing correlated with altered sensorium and depth of coma but not with outcome. At 1 month, three patients died and 16 had complete, three partial and two poor recovery. CONCLUSIONS: EEG revealed non-specific slowing in 55% patients with dengue virus infection, which correlated with level of consciousness but not with CT/MRI abnormalities or clinical recovery. SIGNIFICANCE: Dengue virus infection results in non-specific EEG slowing in 55% patients, which may be due to dengue virus encephalitis per se or associated confounding variables such as seizure, metabolic encephalopathy, or structural brain lesion.     

Parkinsonism due to predominant involvement of substantia nigra in Japanese encephalitis

OBJECTIVE: To study the clinical correlates of lesions seen predominantly in the substantia nigra in some patients with Japanese encephalitis (JE). BACKGROUND: JE typically involves thalamus, brainstem, spinal cord, and cerebral cortex. Rarely, basal ganglia and cerebellum may be affected. Lesions are often widespread and discrete. Predominant involvement of substantia nigra in JE has not been previously reported. METHODS: Of 52 patients with JE seen in an endemic zone, five were selected on the basis of isolated lesions in the substantia nigra on MRI; all were subjected to detailed clinical and laboratory evaluation. RESULTS: Presenting symptoms were fever, alteration of consciousness, neck stiffness, and decreased body movements. Examination during acute illness revealed restricted eye movements, opsoclonus, upbeating nystagmus, and cogwheel rigidity. There was early and complete recovery of consciousness and eye signs. Parkinsonian features such as positive glabellar tap sign, masklike face, bradykinesia, tremors, and postural instability became apparent as these patients started walking. Reversible mutism was observed in three patients during the acute phase. Response to levodopa, amantadine, and trihexiphenedyl was partial. Three patients were followed for more than 1 year, during which time their parkinsonian features recovered completely. Substantial recovery was also observed in the two other patients 2 months after regaining consciousness. CONCLUSIONS: Some patients with Japanese encephalitis may have lesions predominantly in the substantia nigra. After recovery from acute encephalitic illness, they manifest clinically with typical parkinsonian features. Although several viruses are known to cause parkinsonism, this is the first demonstration of a virus producing lesions predominantly in the substantia nigra and causing parkinsonism.     

Seizures in human immunodeficiency virus infection

Among 630 patients with human immunodeficiency virus infection, 70 patients with new-onset seizures were studied. Generalized seizures occurred in 66 patients (94%): they occurred as the initial seizure in 56 patients (80%) and during follow-up in another 10 patients (14%). Partial seizures (18 patients), status epilepticus (10 patients), and recurrent seizures (38 patients) were also noted. Identified processes included cerebral toxoplasmosis in 11 patients, cerebral lymphoma in 8, metabolic derangement in 8, cryptococcal meningitis in 7, and vascular infarction in 4. In 32 patients (46%) seizures were not associated with identifiable brain lesions and were believed to result from human immunodeficiency virus cerebral infection. Phenytoin treatment was associated with adverse drug reactions in 16 of 62 patients who received it. Our results suggest that the majority of patients with human immunodeficiency virus and seizures do not have secondary focal brain lesions as the cause of the seizures and that human immunodeficiency virus infection alone can, and often does, cause seizures.     

Transient Focal Abnormalities of Neuroimaging Studies During Focal Status Epilepticus

We report transient changes in computed tomography (CT) and magnetic resonance imaging (MRI) scans in a patient with focal status epilepticus, referred to us with a tentative diagnosis of neoplasm based on CT and angiographic findings. EEG seizures originated independently from each temporal-occipital area, predominantly from the right. Previous EEGs had shown almost exclusively right temporo-occipital epileptogenic activity. MRI showed increased signal intensity, and CT showed decreased right hemisphere attenuation without enhancement. One month later, there was resolution of the radiological and clinical abnormalities. The transient CT and MRI changes probably represented focal cerebral edema, developing during focal status epilepticus. Lack of change in the left hemisphere probably reflected the quantitative difference in epileptic activity. Clues to the diagnosis of focal edema due to status include: (1) changes on electrical and imaging studies that correlate anatomically with the clinical status, and (2) resolution of abnormalities with appropriate seizure control. In patients with suspected seizure disorders, electrical and clinical data should be correlated before interpretation is made of focal lesions seen by neuroimaging techniques.     

Kainic-acid-induced seizures: a developmental study

Developmental dose-response curves for kainic-acid-induced seizures were generated in rats. Rats at 15-18 days (pups), 33-37 days ( pubescents ) and over 90 days (adults) were administered kainic acid intraperitoneally. Seizures were elicited in all 3 age groups, but some of the behavioral manifestations differed in the pups. This group also had the lowest convulsive threshold, the most severe seizures and the highest mortality. Forelimb convulsions and status epilepticus were associated with the occurrence of necrotic lesions in the adults and pubescents but not in the pups. Deoxyglucose (DG) autoradiographic studies of the convulsing rats disclosed differences in the DG uptake pattern of the substantia nigra across the 3 age groups. Increases in the DG uptake were present in the two older age groups but not in the pups. Since recent data have implicated the substantia nigra as a crucial site in a seizure modifying circuitry in adult animals, our results suggest that the lack of substantia nigra involvement in the pups may account for the early generalization and the increased severity of seizures in this age group.     

Generalized tonic-clonic status epilepticus: causes, treatment, complications and predictors of case fatality

We retrospectively reviewed the clinical course of 66 patients treated for generalized tonic-clonic status epilepticus at the Ege University neurological intensive care unit from 1988 to 1997. Seventy-two per cent of the study group had a pre-existing seizure disorder, and antiepileptic drug withdrawal was the most prominent cause of status epilepticus. The other causes included drug toxicity, central nervous system infection, cerebrovascular disease, tumour and trauma. Seventy-three per cent of all patients responded to the first-line therapy (diazepam and/or phenytoin), and the remainder were considered to have refractory status epilepticus and required pentobarbital anaesthesia. Overall case fatality was 21%, but death could be attributed directly to status epilepticus and/or treatment complication in 10% of the study group. Major determinants of fatal outcomes were: increasing age, longer duration of status epilepticus before initiation of therapy and central nervous system infection as a causal factor. Received: 9 December 1997 Received in revised form: 23 February 1998 Accepted: 30 March 1998     

Characterization of Benzodiazepine Receptor Binding in Immature Rat Brain After Kainic Acid Administration

Summary: Purpose : To evaluate the effects of status epilepticus on benzodiazepine (BDZ) receptor binding in immature rat brain.
Methods : Twenty-four immature (15 days old) and six adult (90 days old) rats were used in this study. Status epilepticus was induced in immature animals by administration of kainic acid (7 mg/kg intraperitoneal), whereas adults rats received saline. Animals were killed 72 hours or 35 days after treatment, and their brains were used for in vitro autoradiography experiments to determine BDZ binding.
Results : In basal conditions and compared with the adult group, immature animals presented reduced BDZ binding in the entorhinal cortex, substantia nigra pars reticulata, and periaqueductal gray. Seventy-two hours after kainic acid–induced status epilepticus, immature rats showed significantly increased BDZ in the frontal (48%), cingulate (39%), sensorimotor (39%), piriform (57%), and entorhinal (59%) cortices, the medial (84%) and basolateral (27%) amygdaloid nuclei, the dentate gyrus (51%), and the substantia nigra pars reticulata (43%). Thirty-five days after status epilepticus, immature rats displayed decreased BDZ binding in the entorhinal cortex (48%), dentate gyrus (36%), and fields CA1, CA2, and CA3 of Ammon's horn (30%).
Conclusions : The present study demonstrates that status epilepticus and temporal lobe epilepsy produce a characteristic pattern of BDZ binding changes in the immature rat brain that differs from the one previously seen in adults.     

Kainic acid-induced substantia nigra seizure in rats: behavior, EEG and metabolism

RATIONALE: In order to clarify the role of substantia nigra pars reticulata (SNr) upon the development of epileptic seizure, kainic acid (KA) was injected into a unilateral SNr. MATERIALS AND METHODS: Wistar rats weighing 250-350 g were used. A stainless-steel cannula and depth electrode were inserted stereotaxically into the left substantia nigra pars reticulata (SNr). At 7 days after surgery, 1.0 microg of KA was injected into the left SNr. Experiment 1: In eight rats, behavior and electroencephalograms (EEG) were continuously recorded for about 30 h, and intermittently monitored following 1 month. Experiment 2: Two hours after KA injection into SNr, rats demonstrated status epilepticus. Then, 100 microCi/kg of [(14)C]2-deoxyglucose (2-DG) was intravenously injected in seven rats, and the rats were processed for autoradiographic study. RESULTS: Changes in behavior and EEG: On EEG, a secondary generalized seizure status was observed at about 70 min after KA injection. In video, limbic seizure manifestations such as salivation were observed as a initial symptom and followed by rolling and generalized tonic seizures. [(14)C]deoxyglucose autoradiographic study demonstrated increased local cerebral glucose metabolism in the medial and lateral septal nucleus, substantia nigra, hippocampus, parietal cortex, piriform cortex, medial and lateral geniculate nucleus, anterodorsal, lateral and ventral nucleus of the thalamus, amygdala and midbrain reticular formation. SUMMARY: The result suggested that the substantia nigra played an important role in the secondary generalization in the substantia nigra seizure model due to the decreased function of the GABAergic projection system induced by an excessive epileptic excitation of SNr.     

Free‐water and BOLD imaging changes in Parkinson's disease patients chronically treated with a MAO‐B inhibitor

Rasagiline is a monoamine oxidase type B inhibitor that possesses no amphetamine‐like properties, and provides symptomatic relief in early and late stages of Parkinson's disease (PD). Data in animal models of PD suggest that chronic administration of rasagiline is associated with structural changes in the substantia nigra, and raise the question whether the structure and function of the basal ganglia could be different in PD patients treated chronically with rasagiline as compared with PD patients not treated with rasagiline. Here, we performed a retrospective cross‐sectional magnetic resonance imaging (MRI) study at 3 T that investigated nigrostriatal function and structure in PD patients who had taken rasagiline before testing (～8 months), PD who had not taken rasagiline before testing, and age‐matched controls. The two PD groups were selected a priori to not differ significantly in age, sex, disease duration, severity of symptoms, cognitive status, and total levodopa equivalent daily dose of medication. We evaluated percent signal change in the posterior putamen during force production using functional MRI, free‐water in the posterior substantia nigra using diffusion MRI, and performance on a bimanual coordination task using a pegboard test. All patients were tested after overnight withdrawal from antiparkinsonian medication. The rasagiline group had greater percent signal change in the posterior putamen, less free‐water in the posterior substantia nigra, and better performance on the coordination task than the group not taking rasagiline. These findings point to a possible chronic effect of rasagiline on the structure and function of the basal ganglia in PD. Hum Brain Mapp 37:2894–2903, 2016. © 2016 Wiley Periodicals, Inc .     

Complex partial status epilepticus associated with adult H1N1 infection

In the wake of the worldwide H1N1 pandemic, there has been evidence that the H1N1 influenza virus is associated with neurological complications. This is the first report describing status epilepticus in an adult patient with H1N1 virus infection, to our knowledge. This patient had no prior history of epilepsy and presented with complex partial status epilepticus. This was further illustrated on electroencephalographs and MRI brain changes that corresponded with the patient’s clinical state and which subsequently resolved on follow-up. Although uncommon, H1N1 infections may result in central nervous system complications in adults and it is crucial to treat such patients with urgency.     

Transient focal abnormalities of MRI and angiographic neuroimaging in status epilepticus: case report]

We reported transient changes in computed tomography (CT), angiography and magnetic resonance imaging (MRI) scans in a patient with status epilepticus, referred to us with a tentative diagnosis of neoplasma based on CT, MRI and angiographic findings, MRI showed increased signal intensity, and CT showed decreased left hemisphere attenuation without enhancement. Two months later, resolution of these radiological and clinical abnormalities had been attained. The transient CT and MRI changes probably represented focal cerebral edema, developing during focal status epilepticus.     

Early detection of brain damages in status epilepticus using apparent diffusion coefficient mapping]

We report two patients of status epilepticus, in which detailed brain magnetic resonance image (MRI) studies were performed. In one patient apparent diffusion coefficient (ADC) increased in the right temporopareital regions in the acute phase. This patient finally has good clinical course without neurological deficits, and abnormal findings on MRI also disappeared. In another patient ADC increased in the similar regions in the acute phase, and an MRI demonstrated cortical laminar necrosis on T1-weighted scans in the same regions in the posticteric phase about 3 months later. The patient had a poorer prognosis with moderate dementia. It has been demonstrated that increased ADC reflects extracellular edema, while decreased ADC indicates cytotoxic edema. ADC mapping appears to be useful for predicting the reversibility of brain damages due to status epilepticus.     

Flurothyl status epilepticus in developing rats: behavioral, electrographic histological and electrophysiological studies.

Status epilepticus and repeated seizures have age-dependent morphological and neurophysiological alterations in the hippocampus. In the present study, effects of flurothyl-induced status epilepticus were examined in awake and free moving immature (2 weeks old) and adult rats. Without exception, adult rats died of respiratory arrest before the onset of status epilepticus. We were unable to find a concentration of flurothyl that produced status epilepticus and a low mortality in adult rats. In contrast, immature rats survived flurothyl status epilepticus for up to 60 min with a very low mortality. In rat pups, behavioral manifestations correlated with electrographic seizures in both the cortex and hippocampus. Neuropathological damage (cell loss, pyknotic cells or gliosis) was not observed in the immature hippocampus, thalamus, amygdala, substantia nigra or cortex at 24 h, 2 days or 2 weeks after status epilepticus. In addition, no aberrant mossy fiber reorganization or decrease in cells counts were observed in the hippocampus. Young rats did not show alterations in paired-pulse perforant path inhibition following flurothyl status epilepticus. The present findings are consistent with studies in other seizure models, indicating that immature rats are highly resistant to seizure-induced changes.     

A case report of remarkable improvement of motor disturbances with l-dopa in a patient with post-diffuse axonal injury

A 9-year-old girl with rigidospastic quadriplegia as post-traumatic sequela was reported. The distribution of lesions observed on a MRI implied diffuse axonal injury; involvement of the substantia nigra was also detected. l-Dopa administration was remarkably effective for relief of the rigidity. As a result, she became able to walk on her knees and communicate by writing letters. l-Dopa administration should be considered for patients who show rigidity as sequela of diffuse axonal injury with involvement of the substantia nigra.     

Multinodular polymyositis in a patient with human immunodeficiency and hepatitis C virus coinfection

We report a patient who developed multiple inflammatory muscle masses and generalized polymyositis in the setting of combined human immunodeficiency virus (HIV) and hepatitis C virus (HCV) infection. Magnetic resonance imaging (MRI) of muscles showed patchy edema which was particularly intense within the nodular masses. Polymerase chain reaction (PCR) showed no evidence of either virus within muscle. This report reviews earlier literature on muscle nodules associated with myositis and discusses the differential diagnosis of muscle masses in HIV infection.     

Basal ganglia alterations and brain atrophy in Huntington's disease depicted by transcranial real time sonography

OBJECTIVES AND METHODS: Transcranial real time sonography (TCS) was applied to 49 patients with Huntington's disease and 39 control subjects to visualise alterations in the echotexture of the basal ganglia. For comparison T1 weighted, T2 weighted, and fast spin echo MRI was performed in 12 patients with Huntington's disease with and in nine patients without alterations of the basal ganglia echotexture as detected by TCS and T1 weighted, T2 weighted, and fast spin echo MRI. Furthermore, the widths of the frontal horns, third ventricle, and the lateral ventricles were depicted in TCS examinations and correlations examined with corresponding CT slices. RESULTS: Eighteen out of 45 (40%) of the patients with Huntington's disease with adequate insonation conditions showed hyperechogenic lesions of at least one basal ganglia region. In 12 patients TCS depicted hyperechogenic lesions of the substantia nigra; in six patients the head of the caudate nucleus was affected. The lentiform nucleus (n=3) and the thalamus (n=0) were less often affected or spared. Hyperechogenic lesions were significantly more frequent in patients with Huntington's disease than in 39 control subjects, who had alterations of the echotexture in 12.8% (4/39) of the examinations. The number of CAG repeats and the clinical status correlated with the identification of hyperechogenic lesions of the substantia nigra (p<0.01). Hyperechogenic lesions of the caudate nucleus were associated with an increased signal intensity in T2 weighted MR images (p<0.05). All TCS parameters indicating brain atrophy correlated with CT findings (p<0.0001). CONCLUSIONS: TCS detects primarily abnormalities of the caudate nucleus and substantia nigra in Huntington's disease. These changes in the echotexture may represent degenerative changes in the basal ganglia matrix and are partially associated with CAG repeat expansion and the severity of clinical findings.     

Focal Cerebral Magnetic Resonance Changes Associated with Partial Status Epilepticus

Summary: We report 2 patients with transient abnormalities on magnetic resonance imaging (MRI) associated with partial status epilepticus (SE). A man with a 4-month history of partial seizures had complex partial SE for 9 days, with left temporal maximum on ictal EEG. Left temporal lobe T₂ signal was increased on MRI during SE, but cerebral MRI was normal 9 weeks later. A woman with "cryptogenic" temporal lobe epilepsy for 16 years had complex partial SE for 1 week, with right temporal maximum on ictal EEG. T₂ Signal was increased over the entire right temporal lobe, extending into the insula, without mass effect, on MRI 1 month after SE ended. Repeat MRI 1 month later showed marked decrease in volume of increased T₂ intensity, without gadolinium enhancement, but with mild mass effect over the right anteroinferomesial temporal areas. A gemistocytic astrocytoma was resected. Focal cerebral MRI abnormalities consistent with cerebral edema may be due to partial SE but also may indicate underlying glioma, even in long-standing partial epilepsy. Focal structural imaging changes consistent with neoplasm should be followed to full resolution after partial SE.     

Dopamine transporter binding in Wilson's disease

INTRODUCTION: In Wilson's disease (WD), brain magnetic resonance images (MRI) show increased signal intensity in T2 weighted images in the lenticular nuclei, thalamus and brainstem, including the substantia nigra. A poor therapeutic response to levodopa in WD suggests the mechanism of a postsynaptic abnormality. However positron emission tomography studies show an involvement of the nigrostriatal presynaptic dopaminergic pathway. CASE REPORT: We report the clinical manifestations in a case of WD with akinetic-rigid syndrome and initial hesitation. The brain MRI showed an increased signal intensity lesion in the substantia nigra region, in addition to basal ganglion and thalamic lesions. However, dopamine transporter (DAT) imaging with 99mTc-TRODAT-1 revealed a nonsignificantly increased DAT uptake, suggesting a normal presynaptic nigrostriatal dopaminergic terminal. CONCLUSION: We suggest that significant heterogeneity can be found in WD patients and a normal presynaptic dopaminergic pathway may occur in some patients, even those with typical akinetic-rigid syndrome and evidence of substantia nigra involvement in the brain on MRI.     

Nigral involvement and nigrostriatal dysfunction in Huntington's disease: Evidences from an MRI and SPECT study

BackgroundHuntington disease (HD) is pathologically characterized by a selective neurodegeneration of vulnerable populations of neurons, with an early marked neuronal loss and atrophy in the neostriatum. Dopaminergic innervations of neostriatal neurons originate in the substantia nigra pars compacta. Few studies investigated the neuronal loss and the functional role of the substantia nigra in modulating clinical features in HD.Methods12 patients and 12 age-matched controls underwent SPECT scans with ¹²³I-FP-CIT and a 1.5 T MRI scan with inversion recovery technique. The association between both clinical and neuropsychological features and striatal uptake and volume of substantia nigra was explored.ResultsStriatal (p < 0.05), caudate (p < 0.05), and putaminal (p < 0.01) uptake was significantly lower in patients with respect to controls. Further, the volume of substantia nigra was reduced in HD when compared to controls (p < 0.01). No relationship between the volume of SN and tracer striatal uptake was found as well as between clinical and neuropsychological features with the SPECT and MRI results.ConclusionsOur results confirm that the degeneration of nigrostriatal pathway may occur in symptomatic HD patients. If confirmed by larger studies, the lack of any kind of correlation between clinical and neuropsychological features with striatal uptake and volume of substantia nigra suggests that motor and cognitive aspects in HD are not directly related to nigrostriatal degeneration.