Development Of Cerebral Atrophy Research Articles

Be Alert to ALERD: Acute Leukoencephalopathy with Restricted Diffusion—Atypical Presentation of a Rare Case

AbstractAcute leukoencephalopathy with restricted diffusion (ALERD) is a novel entity observed in children with acute encephalopathy. It is characterized by diffusion restriction on magnetic resonance imaging (MRI), which can be the distinctive and sometimes the only imaging finding. Other routine MRI sequences may appear normal. ALERD can have infectious or noninfectious causes. In this case, we present a patient with infectious ALERD associated with influenza B virus. A 10-year-old boy presented to the emergency room with a brief history of fever and cough, followed by altered sensorium. He was irritable and ataxic. Neck stiffness was elicited upon examination. An MRI of the brain showed extensive symmetric diffusion restriction in the periventricular and deep white matter, corpus callosum, posterior limb of the internal capsule, and cerebral peduncles of both cerebral hemispheres. Cerebrospinal fluid study, blood and urine toxicology panels were noncontributory. The respiratory panel test was positive for influenza B. A diagnosis of infectious ALERD was made. Following treatment with steroids, intravenous immunoglobulin, antiglutamate agents, and supportive measures, he showed clinical and radiological improvement. The reporting of ALERD has risen in recent years due to its increased detection using MRI. Diffusion-weighted image plays the key role in the final diagnosis. Supportive care and prompt treatment of seizures are very important for better outcome. Follow-up imaging is also required to assess reappearance of white matter edema and development of cerebral atrophy or gliosis.

Evolution of Cerebral Atrophy in a Patient with Super Refractory Status Epilepticus Treated with Barbiturate Coma

Introduction. Status epilepticus is associated with neuronal breakdown. Radiological sequelae of status epilepticus include diffusion weighted abnormalities and T2/FLAIR cortical hyperintensities corresponding to the epileptogenic cortex. However, progressive generalized cerebral atrophy from status epilepticus is underrecognized and may be related to neuronal death. We present here a case of diffuse cerebral atrophy that developed during the course of super refractory status epilepticus management despite prolonged barbiturate coma. Methods. Case report and review of the literature. Case. A 19-year-old male with a prior history of epilepsy presented with focal clonic seizures. His seizures were refractory to multiple anticonvulsants and eventually required pentobarbital coma for 62 days and midazolam coma for 33 days. Serial brain magnetic resonance imaging (MRI) showed development of cerebral atrophy at 31 days after admission to our facility and progression of the atrophy at 136 days after admission. Conclusion. This case highlights the development and progression of generalized cerebral atrophy in super refractory status epilepticus. The cerebral atrophy was noticeable at 31 days after admission at our facility which emphasizes the urgency of definitive treatment in patients who present with super refractory status epilepticus. Further research into direct effects of therapeutic coma is warranted.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy

CADASIL is an inherited small vessel disease of the brain caused by mutations of the NOTCH3 gene encoding a receptor of smooth muscle cells and pericytes within the wall of arterioles and capillaries. The mutated gene is responsible for accumulation of NOTCH3 protein and aggregation of various proteins in the vascular wall. The disease occurs during mid-adulthood and is responsible for attacks of migraine with aura, ischemic stroke, mood disorders and cognitive impairment ranging from mild alterations of attentional performances and executive functions to severe dementia. The disease develops in adults with aging and is responsible at the latest stage of gait and balance troubles associated with cognitive impairment that may lead to severe disability and dependence. MRI shows widespread white matter lesions that may involve the anterior part of temporal lobes often associated with small cerebral infarcts and with microbleeds. The clinical severity is related to accumulation of small infarcts and the development of cerebral atrophy over time. The diagnosis of the disease is confirmed by genetic testing or skin biopsy.

Day-of-Injury Computerized Tomography, Rehabilitation Status, and Development of Cerebral Atrophy in Persons with Traumatic Brain Injury

To compare day-of-injury (DOI) computerized tomography (CT) findings with acute injury severity markers, disability at acute hospital admission and discharge from inpatient rehabilitation, injury severity markers, and degree of postacute cerebral atrophy on magnetic resonance imaging (MRI). Retrospective chart review of 240 consecutive traumatic brain injury (TBI) admissions (mean age 31.7 +/- 15.8 yrs) with moderate-to-severe initial brain injury. All DOI CT abnormalities were qualitatively rated. Disability was assessed using the Disability Rating Scale (DRS) and the FIM measure. In a representative subset, cerebral atrophy was determined by the ventricle-to-brain ratio (VBR) method and quantified from MRI scans 25 or more days postinjury. CT classification resulted in nonsignificant differences in DRS and FIM ratings at the time of discharge from the rehabilitation unit, except in brainstem injury subjects who had significantly higher DRS and lower FIM scores at rehabilitation discharge. At 25 or more days postinjury, presence of any DOI CT abnormality was associated with larger VBR. Increased VBR, as an index of cerebral atrophy, was associated with worse rehabilitation discharge DRS and FIM ratings. Other than brainstem injury, DOI CT findings relate poorly to rehabilitation outcome. Presence of DOI CT abnormalities were associated with the development of cerebral atrophy, which was associated with poorer rehabilitation discharge DRS and FIM scores.

Magnetic resonance imaging of the brain in a cohort of extremely preterm infants

To define magnetic resonance imaging (MRI) appearances of the brain in extremely preterm infants between birth and term, a sequential cohort of infants born at a gestational age <30 weeks was studied with a dedicated neonatal magnetic resonance scanner. Images of infants (n = 41) with a median gestational age of 27 weeks (range 23 to 29 weeks) were initially obtained at a median age of 2 days (range 1 to 20 days) and then repeatedly studied; 29 (71%) infants had MRI at a median gestational age of 43 weeks (range 38 to 52 weeks) (term MRI). On the initial MRI scan 28 of 41 infants had abnormalities: either intraventricular hemorrhage, germinal layer hemorrhage, ventricular dilatation, or diffuse and excessive high signal intensity in the white matter on T 2 -weighted images. When magnetic resonance images for preterm infants at term gestation were compared with those of infants in the control group born at term, 22 of 29 infants had dilatation of the lateral ventricles, 24 of 29 had squaring of the anterior or posterior horns of the lateral ventricles, 11 of 29 had a widened interhemispheric fissure or extracerebral space, and 22 of 29 had diffuse and excessive high signal intensity in the white matter. There were no cases of cystic periventricular leukomalacia. We conclude that MRI abnormalities are commonly seen in the brain of preterm infants on whom images are obtained within 48 hours of birth and that further abnormalities develop between birth and term. A characteristic appearance of diffuse and excessive high signal intensity in the white matter on T 2 -weighted images is associated with the development of cerebral atrophy and may be a sign of white matter disease. These MRI appearances may help account for the high incidence of neurodevelopmental impairment in extremely preterm infants. (J Pediatr 1999;135:351-7)

Hypertension as an Etiopathological Factor in the Development of Cerebral Atrophy in Hemodialyzed Patients

Twenty-five patients on long-term regular hemodialysis treatment (RDT) at our dialysis unit who underwent diagnostic cerebral computed tomography (CCT) participated in a study aimed at clarifying the pathogenesis of cerebral atrophy occasionally found at their original scan. The upper age limit was 55 years to exclude the physiological involutive brain changes occurring with age. Cerebral atrophy (CA), as defined morphologically (enlargement of cerebral sulci or an increased Evan’s Index), was detected in all cases. Seventeen patients underwent magnetic resonance imaging (MRI) to define possible white matter changes more accurately. No significant correlation was found between the degree of atrophy and the following uremia-altered hematoseric parameters: creatinine, hematocrit, cholesterol, triglyceridemia, albumin, PTH, calcium, inorganic phosphate. There was no correlation between degree of atrophy and number of months the patients had been on RDT or time that passed between the finding of a creatinine clearance <30 ml/min and the start of RDT. Very high correlations were found between the degree of CA and predialytic blood pressure values, and between CA and the duration of hypertension (n = 13, r = 0.66, p < 0.013). Thus, hypertension seems to be an early cause of cerebral parenchymal damage in RDT patients, and should be promptly corrected.

The potential role of nitric oxide in multiple sclerosis.

Nitric oxide (.NO) and its reactive derivative peroxynitrite (ONOO-) have been implicated in the pathogenesis of multiple sclerosis (MS). They are cytotoxic to oligodendrocytes and neurones in culture by inhibiting the mitochondrial respiratory chain (complexes II/III and IV) and inhibiting certain key intracellular enzymes. Recently .NO has been implicated as a possible aetiological factor in reversible conduction block in demyelinated axons. Inducible nitric oxide synthase (iNOS) is upregulated in the central nervous system of animals with experimental allergic encephalomyelitis (EAE) and in patients with MS. In some EAE models inhibiting iNOS activity decreases disease severity whilst in other models disease activity is exacerbated. Raised levels of nitrate and nitrite, stable end-products of .NO/ONOO-, are found in the cerebrospinal fluid, serum and urine of patients with MS. CSF levels of nitrate and nitrite correlate with blood-brain-barrier dysfunction, which suggests that .NO may play a role in inflammatory blood-brain-barrier dysfunction. In a longitudinal study on 24 patients with relapsing remitting and secondary progressive MS, raised serum nitrate and nitrite levels correlated with a relapsing course and infrequent relapses. However, no correlation was found between raised serum levels of nitrate and nitrite and MRI activity, disease progression, or the development of cerebral atrophy. In autoimmune mediated CNS demyelinating disease .NO may be a double-edged sword, mediating tissue damage on the one hand and on the other hand modulating complex immunological functions which may be protective.

Mass Effect and Atrophy after Stroke

Cerebral edema, resolution of edema, and subsequent development of cerebral atrophy were studied prospectively in 83 patients with ischemic stroke with CT 3 days, 2 weeks, and 6 months post-stroke. Nineteen patients had large (diameter &gt; 3 cm), 25 medium sized (diameter ≥ 1.5 ≤ 3 cm), and 15 lacunar infarcts (diameter &lt; 1.5 cm). In 24 patients no infarcts were seen. Changes of Evans' ratio (ER), septum-caudate distance (S/C), and width of widest cortical sulci (SuW) were taken as markers of mass effect/atrophy. These parameters were within normal limits in most cases. However, when all CT scans performed in each patient were compared, changes of ER, S/C, and SuW became apparent as evidence of mass effect and subsequent atrophy development. Mass effect occurred in 81 percent and atrophy in 58 percent of patients with large infarcts. In patients with medium sized infarcts, mass effect occurred in 38 percent and atrophy in 45 percent.

Mass Effect and Atrophy after Stroke

Cerebral edema, resolution of edema, and subsequent development of cerebral atrophy were studied prospectively in 83 patients with ischemic stroke with CT 3 days, 2 weeks, and 6 months post-stroke. Nineteen patients had large (diameter greater than 3 cm), 25 medium sized (diameter greater than or equal to 1.5 less than or equal to 3 cm), and 15 lacunar infarcts (diameter less than 1.5 cm). In 24 patients no infarcts were seen. Changes of Evans' ratio (ER), septum-caudate distance (S/C), and width of widest cortical sulci (SuW) were taken as markers of mass effect/atrophy. These parameters were within normal limits in most cases. However, when all CT scans performed in each patient were compared, changes of ER, S/C, and SuW became apparent as evidence of mass effect and subsequent atrophy development. Mass effect occurred in 81 percent and atrophy in 58 percent of patients with large infarcts. In patients with medium sized infarcts, mass effect occurred in 38 percent and atrophy in 45 percent.