Targeted magnetic resonance imaging (tMRI) of small changes in the T1 and spatial properties of normal or near normal appearing white and gray matter in disease of the brain using divided subtracted inversion recovery (dSIR) and divided reverse subtracted inversion recovery (drSIR) sequences.

This review describes targeted magnetic resonance imaging (tMRI) of small changes in the T1 and the spatial properties of normal or near normal appearing white or gray matter in disease of the brain. It employs divided subtracted inversion recovery (dSIR) and divided reverse subtracted inversion recovery (drSIR) sequences to increase the contrast produced by small changes in T1 by up to 15 times compared to conventional T1-weighted inversion recovery (IR) sequences such as magnetization prepared-rapid acquisition gradient echo (MP-RAGE). This increase in contrast can be used to reveal disease with only small changes in T1 in normal appearing white or gray matter that is not apparent on conventional MP-RAGE, T2-weighted spin echo (T2-wSE) and/or fluid attenuated inversion recovery (T2-FLAIR) images. The small changes in T1 or T2 in disease are insufficient to produce useful contrast with conventional sequences. To produce high contrast dSIR and drSIR sequences typically need to be targeted for the nulling TI of normal white or gray matter, as well as for the sign and size of the change in T1 in these tissues in disease. The dSIR sequence also shows high signal boundaries between white and gray matter. dSIR and drSIR are essentially T1 maps. There is a nearly linear relationship between signal and T1 in the middle domain (mD) of the two sequences which includes T1s between the nulling T1s of the two acquired IR sequences. The drSIR sequence is also very sensitive to reductions in T1 produced by Gadolinium based contrast agents (GBCAs), and when used with rigid body registration to align three-dimensional (3D) isotropic pre and post GBCA images may be of considerable value in showing subtle GBCA enhancement. In serial MRI studies performed at different times, the high signal boundaries generated by dSIR and drSIR sequences can be used with rigid body registration of 3D isotropic images to demonstrate contrast arising from small changes in T1 (without or with GBCA enhancement) as well as small changes in the spatial properties of normal tissues and lesions, such as their site, shape, size and surface. Applications of the sequences in cases of multiple sclerosis (MS) and methamphetamine dependency are illustrated. Using targeted narrow mD dSIR sequences, widespread abnormalities were seen in areas of normal appearing white matter shown with conventional T2-wSE and T2-FLAIR sequences. Understanding of the features of dSIR and drSIR images is facilitated by the use of their T1-bipolar filters; to explain their targeting, signal, contrast, boundaries, T1 mapping and GBCA enhancement. Targeted MRI (tMRI) using dSIR and drSIR sequences may substantially improve clinical MRI of the brain by providing unequivocal demonstration of abnormalities that are not seen with conventional sequences.

Imaging Cancer in Neuroradiology.

Neuroimaging plays a pivotal role in the diagnosis, management, and prognostication of brain tumors. Recently, the World Health Organization published the fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS5), which places greater emphasis on tumor genetics and molecular markers to complement the existing histological and immunohistochemical approaches. Recent advances in computational power allowed modern neuro-oncological imaging to move from a strictly morphology-based discipline to advanced neuroimaging techniques with quantifiable tissue characteristics such as tumor cellularity, microstructural organization, hemodynamic, functional, and metabolic features, providing more precise tumor diagnosis and management. The aim of this review is to highlight the key imaging features of the recently published CNS5, outlining the current imaging standards and summarizing the latest advances in neuro-oncological imaging techniques and their role in complementing traditional brain tumor imaging and management.

Feasibility and usefulness of brain imaging in catatonia.

Catatonia is a well characterized psychomotor syndrome that has recognizable motor, affective, behavioural and vegetative manifestations. Despite recent demonstration that catatonia is often associated with brain imaging abnormalities, there is currently no consensus or guidelines about the role of brain imaging. In this study, we assessed the feasibility of brain imaging in a series of patients with catatonia in a routine clinical setting and estimated the prevalence of clinically relevant radiological abnormalities. Sixty patients with catatonia were evaluated against sixty non-healthy controls subjects with headache. The MRI reports were reviewed, and MRI scans were also interpreted by neuroradiologists using a standardised MRI assessment. In this cohort, more than 85% of brain scans of patients with catatonia revealed abnormalities. The most frequently reported abnormalities in the catatonic group were white matter abnormalities (n = 44), followed by brain atrophy (n = 27). There was no evidence for significant differences in the frequency of abnormalities found in radiology reports and standardised neuroradiological assessments. The frequency of abnormalities was similar to that found in a population of non-healthy controls subjects with headache. This study shows that MRI is feasible in patients with catatonia and that brain imaging abnormalities are common findings in these patients. Most frequently, white matter abnormalities and diffuse brain atrophy are observed.

Quantitative susceptibility mapping demonstrates different patterns of iron overload in subtypes of early-onset Alzheimer's disease.

OBJECTIVES: We aimed to define brain iron distribution patterns in subtypes of early-onset Alzheimer's disease (EOAD) by the use of quantitative susceptibility mapping (QSM). METHODS: EOAD patients prospectively underwent MRI on a 3-T scanner and concomitant clinical and neuropsychological evaluation, between 2016 and 2019. An age-matched control group was constituted of cognitively healthy participants at risk of developing AD. Volumetry of the hippocampus and cerebral cortex was performed on 3DT1 images. EOAD subtypes were defined according to the hippocampal to cortical volume ratio (HV:CTV). Limbic-predominant atrophy (LPMRI) is referred to HV:CTV ratios below the 25th percentile, hippocampal-sparing (HpSpMRI) above the 75th percentile, and typical-AD between the 25th and 75th percentile. Brain iron was estimated using QSM. QSM analyses were made voxel-wise and in 7 regions of interest within deep gray nuclei and limbic structures. Iron distribution in EOAD subtypes and controls was compared using an ANOVA. RESULTS: Sixty-eight EOAD patients and 43 controls were evaluated. QSM values were significantly higher in deep gray nuclei (p < 0.001) and limbic structures (p = 0.04) of EOAD patients compared to controls. Among EOAD subtypes, HpSpMRI had the highest QSM values in deep gray nuclei (p < 0.001) whereas the highest QSM values in limbic structures were observed in LPMRI (p = 0.005). QSM in deep gray nuclei had an AUC = 0.92 in discriminating HpSpMRI and controls. CONCLUSIONS: In early-onset Alzheimer's disease patients, we observed significant variations of iron distribution reflecting the pattern of brain atrophy. Iron overload in deep gray nuclei could help to identify patients with atypical presentation of Alzheimer's disease. KEY POINTS: • In early-onset AD patients, QSM indicated a significant brain iron overload in comparison with age-matched controls. • Iron load in limbic structures was higher in participants with limbic-predominant subtype. • Iron load in deep nuclei was more important in participants with hippocampal-sparing subtype.

High-Resolution CT Imaging of the Temporal Bone: A Cadaveric Specimen Study.

Objective Super-high and ultra-high spatial resolution computed tomography (CT) imaging can be advantageous for detecting temporal bone pathology and guiding treatment strategies. Methods Six temporal bone cadaveric specimens were used to evaluate the temporal bone microanatomic structures utilizing the following CT reconstruction modes: normal resolution (NR, 0.5-mm slice thickness, 512 2 matrix), high resolution (HR, 0.5-mm slice thickness, 1,024 2 matrix), super-high resolution (SHR, 0.25-mm slice thickness, 1,024 2 matrix), and ultra-high resolution (UHR, 0.25-mm slice thickness, 2,048 2 matrix). Noise and signal-to-noise ratio (SNR) for bone and air were measured at each reconstruction mode. Two observers assessed visualization of seven small anatomic structures using a 4-point scale at each reconstruction mode. Results Noise was significantly higher and SNR significantly lower with increases in spatial resolution (NR, HR, and SHR). There was no statistical difference between SHR and UHR imaging with regard to noise and SNR. There was significantly improved visibility of all temporal bone osseous structures of interest with SHR and UHR imaging relative to NR imaging ( p < 0.001) and most of the temporal bone osseous structures relative to HR imaging. There was no statistical difference in the subjective image quality between SHR and UHR imaging of the temporal bone ( p ≥ 0.085). Conclusion Super-high-resolution and ultra-high-resolution CT imaging results in significant improvement in image quality compared with normal-resolution and high-resolution CT imaging of the temporal bone. This preliminary study also demonstrates equivalency between super-high and ultra-high spatial resolution temporal bone CT imaging protocols for clinical use.

Autoimmune disease of head and neck, imaging, and clinical review.

Autoimmune disease of the head and neck (H&N) could be primary or secondary to systemic diseases, medications, or malignancies. Immune-mediated diseases of the H&N are not common in daily practice of radiologists; the diagnosis is frequently delayed because of the non-specific initial presentation and lack of familiarity with some of the specific imaging and clinical features. In this review, we aim to provide a practical diagnostic approach based on the specific radiological findings for each disease. We hope that our review will help radiologists expand their understanding of the spectrum of the discussed disease entities, help them narrow the differential diagnosis, and avoid unnecessary tissue biopsy when appropriate based on the specific clinical scenarios.

Paraspinal soft tissue edema ratio: An accurate marker for early lumbar spine spondylodiscitis on an unenhanced MRI.

PURPOSE: MRI is currently the gold standard imaging modality in the diagnosis of lumbar spine discitis/osteomyelitis. However, even with supportive clinical and laboratory data, the accuracy of MRI remains limited by several degenerative and inflammatory mimics, such that it continues to represent a challenge for radiologists. This study reports a new quantitative imaging marker of lumbar paraspinal soft tissue edema which shows significant accuracy for spondylodiscitis. METHODS: Thirty-five patients with equivocal MRI findings of lumbar discitis/osteomyelitis vs endplate degenerative changes were reviewed over a 24-month period. Patients with a history of surgery, fractures/recent trauma, signs of advanced infection such as abscesses, phlegmon or severe osseous destruction were excluded. Two ABR board certified neuroradiologists who were blinded to the final diagnosis evaluated a new marker; the superior-inferior paraspinal edema ratio (SI-PER). The SI-PER was obtained by measuring the superior-inferior extent of increased signal/edema in the paraspinal soft tissues on the paraspinal inversion recovery images divided by the vertebral body height measured at midpoint. Cases positive for spondylodiscitis were those confirmed by biopsy, aspiration/drainage, surgery, or clinical improvement following antibiotic treatment. The diagnostic sensitivity and specificity of SI-PER were determined by Receiver operating characteristic (ROC) analysis. RESULTS: In 23/35 (66%) patients, the diagnosis of discitis/osteomyelitis was confirmed. The SI-PER showed a significant association with a positive MRI diagnosis (p = 0.001). Inter-observer correlation for SI-PER was 0.92. ROC analysis showed an area under the curve of 0.84. A SI-PER of 2.5 was 96% sensitive and 75% specific for the diagnosis of discitis/osteomyelitis, with a PPV of 88% and a NPV of 90%. CONCLUSION: In this study, the superior inferior paraspinal edema ratio (SI-PER), a newly defined MRI marker, was found to have high sensitivity for differentiating spondylodiscitis from endplate degenerative changes on lumbar spine MRI.

Autoimmune diseases of the brain, imaging and clinical review.

There is an extensive spectrum of autoimmune entities that can involve the central nervous system, which has expanded with the emergence of new imaging modalities and several clinicopathologic entities. Clinical presentation is usually non-specific, and imaging has a critical role in the workup of these diseases. Immune-mediated diseases of the brain are not common in daily practice for radiologists and, except for a few of them such as multiple sclerosis, there is a vague understanding about differentiating them from each other based on the radiological findings. In this review, we aim to provide a practical diagnostic approach based on the unique radiological findings for each disease. We hope our diagnostic approach will help radiologists expand their basic understanding of the discussed disease entities and narrow the differential diagnosis in specific clinical scenarios. An understanding of unique imaging features of these disorders, along with laboratory evaluation, may enable clinicians to decrease the need for tissue biopsy.

Neuroimaging findings and pathophysiology of dorsal spinal arachnoid webs: illustrative case.

BACKGROUND: Spinal arachnoid webs are uncommon and difficult to diagnose, especially because causative intradural transverse bands of arachnoid tissue are radiographically occult. Left untreated, arachnoid webs may cause progressive, debilitating, and permanent neurological dysfunction. Conversely, more than 90% of patients may experience rapid neurological recovery after resection, even with a prolonged duration of presenting symptoms. Indirect imaging signs such as spinal cord indentation and compression with cerebrospinal fluid (CSF) flow alteration provide crucial diagnostic clues that are critical in guiding appropriate management of such patients. OBSERVATIONS: The authors reported a patient with no significant medical history who presented with back pain, progressive lower extremity weakness, gait ataxia, and bowel and bladder incontinence. They discussed multimodality imaging for determining the presence of arachnoid webs, including magnetic resonance imaging, phase-contrast CSF flow study, computed tomography myelography, and intraoperative ultrasound. They also discussed the detailed anatomy of the spinal subarachnoid space and a plausible pathophysiological mechanism for dorsal arachnoid webs. LESSONS: The authors report on a patient who underwent comprehensive imaging evaluation detailing the arachnoid web and whose subsequent anatomical localization and surgical treatment resulted in a full neurological recovery.

Brain Computed Tomography Angiography Maximum Intensity Projection Images for ASPECTS Derivation and Detection of Large Infarct Volumes: Preliminary Study.

PURPOSE: Non-contrast CT ASPECTS (NCCTasp) has an established role in determining eligibility for mechanical thrombectomy in centers without ready access to perfusion or DWI. Moreover, it has been suggested that CTA source ASPECTS (CTAasp) may be superior to NCCTasp in predicting final infarct volume (FIV). In this study, we hypothesized that CTA maximum intensity projection ASPECTS (MIPSasp) would be superior compared to both NCCTasp and CTAasp in predicting FIV as measured by DWI. MATERIALS AND METHODS: In 41 consecutive patients with MCA territory infarcts, NCCTasp, CTAasp and MIPSasp were visually assessed by 2 neuroradiologists. Disagreements were adjudicated by a third neuroradiologist, and the reconciled data used for all further analysis. MR-DWI was used as the standard for FIV determination. Receiver operating characteristic curve analysis was used to compare the area under the curve for all three CT-based methods in predicting FIV ≥70 ml. RESULTS: MIPSasp (AUC: 0.98, CI: 0.88-1.00) were statistically better than NCCTasp (AUC: 0.87, 95% CI: 0.72-0.95; p=0.01) in predicting FIV ≥70 ml. MIPSasp were also superior to CTAasp (AUC: 0.9, CI: 0.79-.98; p˂0.05). Optimal test performance for predicting FIV ≥70 ml for MIPSasp was ≤6 (sensitivity=100%, specificity=91.4%; Youden's J=0.98). CONCLUSION: Our preliminary study suggests that a novel CTA-MIPS derived ASPECTS better predicts large MCA territory infarcts compared to CTA source and non-contrast ASPECTS. Thus, MIPSasp may be a promising technique for future studies aimed at improving ischemic stroke treatment in centers using ASPECTS for stroke management.

What to Look for on Post-stroke Neuroimaging.

The most feared complication after acute ischemic stroke is symptomatic or asymptomatic hemorrhagic conversion. Neuroimaging and clinical criteria are used to predict development of hemorrhage. Seizures after acute ischemic stroke or stroke-like symptoms from seizures are not common but may lead to confusion in the peristroke period, especially if seizures are repetitive or evolve into status epilepticus, which could affect neuroimaging findings. Malignant infarction develops when cytotoxic edema is large enough to lead to herniation and death. Post-stroke neuroimaging prognosticators have been described and should be assessed early so that appropriate treatment is offered before herniation leads to additional tissue injury.

Safety, immediate and mid-term results of the newer generation of hydrogel coils in the treatment of ruptured aneurysms: a multicenter study.

OBJECTIVE: To assess the 'real-world' performance of the newer generation of hydrogel coils in ruptured aneurysms. METHODS: A multicenter retrospective study was carried out of angiographic and clinical outcome data on consecutive patients with ruptured aneurysms treated with at least 70% of the newer generation of hydrogel coils. Demographics and data on clinical grade, smoking, use of statins, aneurysm size, location, technique used, packing density, immediate angiographic result, angiographic follow-up, rebleeding and clinical outcome were obtained and analyzed. RESULTS: Eighty patients (54F; 26M) with an average age of 55.1 years were entered in the study. Forty-four presented good clinical grade (Hunt and Hess 1 or 2). Forty-two (52.5%) aneurysms were ≤5 mm. 56.7% of the aneurysms were treated with simple coil embolization and 39.6% with balloon-assisted coil. The packing density ranged from 9.3% to 92.6% (mean 48.5%). Immediate occlusion rates (Raymond-Roy Scale) were: complete occlusion (class I) in 57.5%, residual neck (class II) in 32.5% and residual aneurysm (class III) in 10%. Intraoperative rupture occurred in 3 cases (3.75%). Clinical follow-up, available in 73 patients, showed a good outcome (modified Rankin Scale 0-2) in 76.3%. Preliminary data on imaging follow-up were available in 54 patients (average 6.8 months) with complete occlusion in 77.8%, residual neck in 20.3% and residual aneurysm in 1.9%. There was no re-hemorrhage. CONCLUSIONS: Our data show that the use of the newer-generation hydrogel coils in the treatment of ruptured aneurysms is feasible, safe and effective with high immediate and mid-term occlusion rates and low morbidity.

Natural history, current concepts, classification, factors impacting endovascular therapy, and pathophysiology of cerebral and spinal dural arteriovenous fistulas.

Dural arteriovenous fistulas (DAVFs) may occur anywhere there is a dural or meningeal covering around the brain or spinal cord. Clinical manifestations are mostly related to venous hypertension, and may be protean, acute or chronic, ranging from minor to severe, from non-disabling tinnitus to focal neurological deficits, seizures, hydrocephalus, psychiatric disturbances, and developmental delay in pediatric patients. Although low-grade lesions may have a benign course and spontaneous involution may occasionally occur (i.e. cavernous sinus DAVFs), the risk of hemorrhage is considerable in high grade lesions. Angiographic features of DAVFs have been clarified since the 1970s when venous drainage pattern was clearly identified as the most significant risk predictor and as a major determinant of success or failure of treatment. The mainstay of therapy is interruption of arteriovenous shunting, which has traditionally been accomplished surgically. Currently, endovascular therapy is generally considered the first line of treatment, allowing elimination of the lesion in most patients, with surgery and stereotactic radiosurgery reserved for complex situations. This review discusses major aspects of DAVFs, including grading systems, clinical presentation, diagnostic evaluation, various issues impacting endovascular therapy, and pathophysiology.

Carotid artery pseudoaneurysm after orthognathic surgery causing lower cranial nerve palsies: endovascular repair.

PURPOSE: Reported complications following Le Fort osteotomies are rare but can include epistaxis from disruptions or pseudo-aneurysms of the maxillary artery or its distal branches the descending palatine and sphenopalatine arteries, aseptic necrosis of the maxilla, ophthalmic injuries including blindness, ophthalmoplegia, and keratitis sicca, and arteriovenous fistulas or false aneurysms of the carotid arteries (external and/or internal). The mechanism of injury to neurovascular structures can be the result of direct or indirect trauma, such as injuries from surgical instruments, traction injuries during manipulation of the osteotomized bone segments or during inadvertent manipulations of the head and neck, or from fractures extending to the base of the skull, orbit, or pterygopalatine fossa associated with the pterygomandibular dysjunction or maxillary downfracture. CASE REPORT: An 18 year-old male with facial bone dysplasia, apertognathia, maxillary hypoplasia and mandibular hyperplasia was treated with maxillary Le Fort I osteotomy with internal fixation and elastic intermaxillary fixation. Following surgery, the patient developed palsies of the vagus and accessory nerves manifesting as dysphagia, cough, vocal cord paralysis and trapezius muscle atrophy. Cross sectional imaging revealed a small, laterally pointing pseudoaneurysm of the high cervical internal carotid artery (ICA) at the skull base, exerting pulsatile mass effect on adjacent lower cranial nerves. The patient was treated with carotid artery stent reconstruction and pseudoaneurysm coil obliteration, and kept on dual antiplatelet therapy for two months. Partial recovery from cranial nerve palsies was observed within a year. CONCLUSION: A small, broad-based, laterally-pointing ICA pseudoaneurysm at the exit of the carotid canal without surrounding hematoma was clearly demonstrated on CTA, which visualization was difficult on MRA due to considerable metallic artifact from surgical hardware. Angiography exquisitely demonstrated the pseudoaneurysm, which was fully repaired with the combination of stenting and coil obliteration, allowing total preservation of the ICA.

Nicardipine infusion for blood pressure control in patients with subarachnoid hemorrhage.

INTRODUCTION: To evaluate the efficacy, tolerability, and safety of nicardipine infusion in controlling the elevated blood pressure after subarachnoid hemorrhage (SAH). METHODS: Nicardipine infusion was initiated if the individual pre-specified systolic blood pressure (SBP) level goal, mandated by the admitting neurosurgeon, was not met. Systolic and diastolic BPs were measured on admission, hourly during the infusion and 12 h before and after the infusion. RESULTS: Twenty-eight patients with SAH required 50 nicardipine infusions in order to achieve a mean SBP goal of 152 mmHg. The 3,112 extracted BP measurements showed that mean infusion SBP was significantly lower than admission and pre-infusion SBP (mean 146.5 vs. 177.1 and 155.6 mmHg, P < 0.001, respectively) and significantly higher than post-infusion SBP (146.5 vs. 142.6 mmHg, P = 0.002). Five infusions were stopped prematurely, because of hypotension (n = 3), emergent surgery (n = 1), and failure to reach the SBP goal (n = 1). Rebleeding was not observed in any patient. Nicardipine achieved SBP control in 59.9% of hourly infusion measurements, with a trend for higher proportion of success with higher SBP goals. CONCLUSION: In this study, nicardipine infusion was a safe and moderately effective treatment for BP control in patients with SAH. Although SBP during nicardipine infusion was higher than the pre-specified goal in a significant percentage of hourly observations, this may be due to the drug administration protocol and other factors such as analgesia and sedation.

Radiologic appearance of chronic parapharyngeal Teflon granuloma.

Although Teflon has been used for almost 5 decades to provide tissue augmentation in various surgical indications, including head and neck surgery, its use has significantly declined in the last 2 decades, primarily because of its implication in granuloma formation. Teflon granulomas have been shown to cause false positives on positron emission tomography imaging and have been reported to have a characteristic magnetic resonance imaging (MRI) appearance. We report a patient with a large chronic Teflon granuloma of the parapharyngeal space that caused significant bony erosion of the atlas vertebra. The lesion's MRI signal characteristics were indistinguishable from those of surrounding tissues, while it showed characteristic hyperdensity on computed tomography due to the presence of fluorine atoms within Teflon. As MRI may supersede or replace computed tomography for a number of indications, and as Teflon has been used in large numbers of patients whose records may not always be available, knowledge of these findings has clinical relevance.

Spinal epidural arteriovenous fistula with late onset perimedullary venous hypertension after lumbar surgery: case report and discussion of the pathophysiology.

STUDY DESIGN: Case report and literature review. OBJECTIVE: Spinal epidural arteriovenous fistulas with secondary reflux into the perimedullary veins are rare. We report a patient who presented with delayed progressive congestive myelopathy after lumbar surgery. The pathophysiology and the anatomic basis for the responsible arteriovenous fistula are discussed. SUMMARY OF BACKGROUND DATA: Delayed neurological deterioration after spinal surgery is uncommon. Epidural fistulae uncommonly may become symptomatic from an epidural hematoma, mass effect from distended veins, and rarely from a spinal dural arteriovenous fistula. We report on a patient with delayed progressive congestive myelopathy after lumbar surgery, and discuss the pathophysiology and the anatomical basis for the causative fistula. METHODS: A 68-year-old man presented with progressive lower extremity weakness and sensory decrease, and loss of sphincter control 2 years after unilateral lumbar laminectomy and fusion for a disc herniation. MRI showed diffuse new cord edema and intradural perimedullary dilated vessels. Spinal angiography revealed an epidural arteriovenous fistula at the site of the previous laminectomy, with intradural perimedullary venous drainage. The fistula was successfully treated surgically and the patient experienced rapid and gradual neurologic improvement, being able to walk without a cane within 6 weeks of repair. RESULTS: There are few causes of delayed neurologic deterioration after lumbar spinal surgery. Epidural fistulas are uncommon and rarely symptomatic, and when they are, it is usually from an epidural hematoma or mass effect from distended epidural veins. Epidural may rarely result in spinal dural arteriovenous fistulas, the most common spontaneous spinal arteriovenous condition, causing a congestive myelopathy characterized by lower extremity spasticity, sensory changes, and loss of sphincter control. CONCLUSION: Delayed neurologic deterioration after spinal surgery is uncommon. Epidural arteriovenous fistulas with secondary intradural drainage, which are rare, should be considered.

Cerebroretinal microangiopathy with calcifications and cysts: demonstration of radiological progression.

Cerebroretinal microangiopathy with calcifications and cysts is a rare entity with progressive calcifications, enhancing subcortical cysts, and leukoencephalopathy. We present the oldest reported woman with this disorder, with 10 years of imaging documenting progression. This reveals (1) more profound white matter involvement than that previously reported and (2) growth and shrinkage of cysts over time, which may suggest a more complex pathogenesis than that previously theorized.