CASE REPORTS
Parahippocampal Corpora Amylacea: Case Report Taylor J. Abel, BS Department of Neurological Surgery, University of Washington, Seattle, Washington.
Adam O. Hebb, MD Department of Neurological Surgery, University of Washington, Seattle, Washington
C. Dirk Keene, MD, PhD Department of Anatomic Pathology, University of Washington, Seattle, Washington
Donald E. Born, MD, PhD Department of Anatomic Pathology, University of Washington, Seattle, Washington
Daniel L. Silbergeld, MD Department of Neurological Surgery, University of Washington, Seattle, Washington Reprint requests: Daniel L. Silbergeld, MD, Department of Neurological Surgery, University of Washington Medical Center, 1959 NE Pacific, Box 356470, Seattle, WA 98195. E-mail:
[email protected] Received, January 7, 2009. Accepted, November 30, 2009. Copyright © 2010 by the Congress of Neurological Surgeons
OBJECTIVE: Corpora amylacea (CA) normally accumulate within perivascular, subpial, and subependymal astrocytic processes. CA are associated with a number of conditions including normal aging, hippocampal sclerosis associated with temporal lobe epilepsy, multiple sclerosis, Lafora-type progressive myoclonic epilepsy, and adult polyglucosan body disease. Reports of massive localized accumulation of CA in the brain outside of these conditions are rare. CLINICAL PRESENTATION: A 49-year-old woman, with a long-standing history of migraine headaches, presented to her primary care provider for increased headache duration. Brain magnetic resonance imaging (MRI) revealed a left parahippocampal lesion, suggestive of low-grade glioma. INTERVENTION: Given the MRI suggestive of left parahippocampal glioma, left-sided frontotemporal craniotomy was performed for resection of the lesion. Specimens obtained during the operation revealed focal high-density accumulation of CA with no evidence of neoplasm, ischemia, or hypoxic injury. CONCLUSION: This case illustrates the possibility that localized high-density CA accumulation can present as an intrinsic lesion on brain MRI. CA should be included in the differential diagnosis for patients presenting with brain MRI suggestive of nonenhancing space-occupying lesions. KEY WORDS: Corpora amylacea, Glioma, Polyglucosan bodies Neurosurgery 66:E1206-E1207, 2010
DOI: 10.1227/01.NEU.0000369196.94664.4E
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orpora amylacea (CA) are spherical aggregations of glucose polymers (polyglucosans) that normally accumulate in astrocytic processes during aging in the subpial, perivascular, and subependymal regions of the brain.1 Lafora2 described CA in 1911 as intranuclear inclusions associated with myoclonus and dementia. Although CA have long been recognized as a byproduct of normal aging, they have also been associated with chronic hypoxia and cellular stress.2 Additionally, several reports have documented increased corpora amylacea in resected temporal lobes from patients with hippocampal sclerosis.3-7 Other neurological conditions known to be associated with CA are multiple sclerosis,8 Alzheimer’s disease,9 Lennox-Gastaut syndrome,10 Lafora-type progressive myoclonus epilepsy,11 and adult polyglucosan body disease (APBD).12,13 Although CA are known to accumulate in various conditions, ABBREVIATIONS: APBD, adult polyglucosan body disease; CA, corpora amylacea; TLE, temporal lobe epilepsy
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their precise role in the pathophysiology of neurological disease is not understood. Here we present the case of a 49-year-old woman with a long-standing history of migraine headaches who was referred for increased headache duration and imaging suggestive of a left parahippocampal low-grade glioma. A craniotomy was performed for resection of the lesion, and histopathological examination revealed mild expansion of the parahippocampal gyrus and large numbers of CA. This case represents a unique instance of CA mimicking low-grade glioma on brain magnetic resonance imaging (MRI).
CASE REPORT This 49-year-old, left-handed, woman with a long-standing medical history of migraine headaches presented to her primary care physician for evaluation of increased headache intensity and duration. The patient indicated that her headaches typically start just before her menstrual cycle, have the quality of squeezing or throbbing, last 1 to 3 days, do not involve any particular
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region of her head, and are alleviated by lying down in a cool dark room. After experiencing a headache of increased intensity that lasted 7 days, as opposed to 3, and did not coincide with her menstrual cycle, the patient went to her primary care physician for evaluation. Aside from migraine headaches, the patient’s medical history was negative, specifically for seizures or any other neurological problems. Brain MRI ordered by the patient’s primary care physician revealed an expansile, mildly infiltrating, and nonenhancing lesion in the left parahippocampal gyrus, with small cystic-appearing areas in the center of the lesion (Figure 1). The radiological characteristics of the lesions were most suggestive of a low-grade infiltrating astrocytoma, but were also thought to possibly represent a ganglioglioma. Because of these neuroimaging findings, the patient was referred to our center for further workup and evaluation. On further interview at our center, the patient reported a 10month history of changes in her cognition, including problems with reading comprehension, misplacing items, and subtle difficulty in performing normal tasks at work. Despite these symptoms, formal cognitive function tests of intelligence, language, memory, attention, visuospatial skills, and executive function did not reveal any indication of cognitive deficit. Because of the lesion’s location within the temporal lobe, preoperative intracarotid sodium amobarbital (Wada) testing was performed in the standard fashion,14 which revealed that language was associated with left hemisphere only and memory performance was intact after injection to either hemisphere. One month after referral to our center, the patient was taken to the operating room for a left frontotemporal craniotomy for resection of the lesion with awake cortical stimulation mapping. Language was identified on the posterior aspect of the superior temporal gyrus. Resection was therefore performed via an anterior temporal lobectomy beginning 3 cm anterior to the most anterior identified language site. Resection included the uncus, parahippocampus, and hippocampus. Frozen sections examined intraoperatively revealed hypercellularity and CA but no definite
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neoplasm. Ultrasonography and MRI Stealth were used to guide the resection and confirmed gross total resection of the lesion. The patient tolerated the procedure well and was discharged from the hospital without complication on postoperative day 3. Postoperative brain MRI confirmed gross total resection of the lesion. The patient was seen in clinic 1 week after surgery for routine postoperative follow-up, but has not been seen since that time approximately 2 years ago. Pathological Examination Portions of the anterior, inferior, and mediotemporal lobe including the hippocampus were submitted to pathology for histopathological analysis. Gross examination of pathological specimens revealed cerebral parenchyma including cortex and white matter. Paraffin sections were costained with Luxol fast blue to highlight myelin and periodic acid–Schiff to highlight CA and were counterstained with hematoxylin to visualize cell nuclei. Examination of the anterior and mediotemporal lobe revealed diffuse and focally dense white matter CA resulting in expansion of white matter tracts. This CA deposition was highly concentrated in the mediotemporal lobe and parahippocampal white matter (Figure 2A,B) and was less prominent in sections of the anterior (Figure 2C) and inferior temporal lobe. CA were not seen in cortical gray matter (Figure 2D) or in the hippocampus proper, including CA pyramidal fields, dentate gyrus, and hilus. Meaningful quantitation of CA requires unbiased stereological sampling that was not feasible given the nature of the specimen. However, there was a clear and abrupt transition from high-density CA to adjacent parenchyma with only rare CA. Cortical tissues were found to have increased numbers of reactive astrocytes, but no definite neuron loss was observed. No evidence of cortical ischemia or hypoxic injury was present. There was mild white matter hypercellularity caused by prominent reactive gliosis within the lesion (Figure 2E) compared with immediately adjacent parenchyma (Figure 2E inset), but no evidence of increased cell proliferation by Ki-67 immunohistochemistry. High-power microscopic examination of periodic acid–Schiff-stained CA revealed characteristic laminated architecture (Figure 2F). The final diagnosis was extensive deposition of CA without any evidence of neoplasm.
DISCUSSION
FIGURE 1. A, T2-weighted coronal brain magnetic resonance image demon-
strating nonenhancing left hippocampal lesion. The lesion appears to be mass occupying and is most consistent with a low to intermediate grade glioma. B, T1-weighted sagittal brain magnetic resonance image showing a lateral view of the lesion.
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CA CA, also known as polyglucosan bodies, are composed of branched aggregations of glucose polymers. Virchow first discovered CA in the central nervous system in the 19th century.15 Later, in 1911, Lafora2 associated CA with myoclonus and dementia. CA have now been described in several conditions including multiple sclerosis,8 Alzheimer’s disease,9 Lennox-Gastaut syndrome,10 Lafora-type progressive myoclonus epilepsy,11 APBD,12 and hippocampal sclerosis of temporal lobe epilepsy (TLE).16 Here we demonstrate high-density accumulation of CA in the parahippocampal white matter that mimicked the clinical and radiological features of mediotemporal glioma.
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teins 28, 60, 70, and 7222; and heme oxygenase-123 in CA, linking CA to the response to cell stress. Components of both the classic and terminal complement pathways have also been reported in CA removed from brains of patient’s with Alzheimer’s disease,17 Pick’s disease,17 and multiple sclerosis,24 suggesting a role for CA in neuroinflammation and neurodegeneration. Im munoreactivity to astrocytic23,25 and oligodendroglial26 moleC D cules suggests a glial influence on CA formation. Given their biochemical composition, CA in the nervous system are thought to originate from neurons, astrocytes, and oligodendrocytes.17 Massive accumulation of CA are often associated with aging and tend to occur in the ependymal lining of the ventricles, beneath the pia mater on the outer surfaces of the brain, E F and covering the cortex in the outer part of layer I.17 Although CA deposit is a normal agerelated finding, the etiology of CA formation is not known. It has been suggested that massive accumulation of CA could result from an acquired defect in glycogen metabolism,18 but others have contested that this is unlikely due to the universality of CA accumulation.17 FIGURE 2. A, Luxol fast blue (LFB)/periodic acid–Schiff (PAS)/hematoxylin-stained (H-stained) section of parahipBecause CA are associated pocampal white matter. Corpora amylacea (CA) stain pink with PAS, and myelinated axons stain blue with LFB. The with aging in normal individuwhite matter tracts are expanded by CA (arrows). B, LFB/PAS/H-stained sections of mediotemporal cortical white matals, it has been suggested that ter with dense CA. C, LFB/PAS/H-stained sections of anterior temporal cortical white matter showing paucity of CA. CA play a role in the cellular D, LFB/PAS/H-stained sections of temporal cortex showing lack of cortical CA. E, glial fibrillary acidic protein immunoaging and cellular responses to histochemical–stained sections demonstrating prominent astrogliosis within the lesion compared with reduced gliosis in adjaoxidative stress.27,28 In this concent parenchyma (inset). F, PAS-stained section demonstrating laminated architecture of CA. Scale bars = 50 µm. text, CA may function to entrap and sequester toxic products of cellular metabolism during the aging process.17 Whether CA actuCA are composed of a mixture of both short and long polysacally function in this context or are simply a byproduct of normal charides, which stain strongly with the McManus-Hotchkiss periaging is not known. odic acid–Schiff reagent and Best’s carmine.17 Centrifugationpurified CA have been reported to yield 87.9% hexose, 4.7% proPathological CA Deposition tein, and 2.5% phosphate.18 Immunohistochemical reactivity of 19 CA to anti-tau antibodies and the extracytoplasmic domain of The mechanism of high-density CA accumulation in the parahipamyloid precursor protein20 have suggested a possible neuronal pocampal region of our patient is not known. She presented with source. Biochemical analysis revealed ubiquitin21; heat-shock proincreased headache duration and intensity; history and physical
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examination failed to reveal abnormalities suggestive of any underlying or associated neurological condition. Neuropsychological evaluation of our patient also did not reveal any findings suggestive of associated cognitive abnormalities. Although CA are a common finding in individuals with hippocampal sclerosis of TLE,6 our patient did not experience seizures and the localization of CA in our patient was different from that seen in hippocampal sclerosis. Histopathological analysis of tissue resected from our patient revealed CA accumulation in the parahippocampal white matter with reactive astrocytes. CA accumulation is known to occur in the subpial border of the hippocampus with aging, but the age and pattern of CA deposition in our patient are not consistent with this mechanism. Additionally, CA accumulation is thought to coincide with neuron loss in hippocampal sclerosis,16 but the hippocampal CA pyramidal fields were intact without evidence of neuron loss. Abnormal accumulation of CA has several known etiologies. Two autosomal recessive conditions known to cause accumulation of CA are Anderson’s disease (type IV glycogenosis) and adult APBD. These 2 conditions are thought to result from the absence or dysfunction of glycogen branching enzymes.17 Additionally, CA deposition has also been associated with progressive myoclonic epilepsy, in which there is a strong hereditary background.29 A variety of other neurological conditions have been commonly associated with CA including multiple sclerosis,8 Alzheimer’s disease,9 Lennox-Gastaut syndrome,10 and amyotrophic lateral sclerosis. 3 0 Accumulations of CA have been associated with spinocerebellar syndrome,31 pallidonigroluysial atrophy,32 and anoxic brain damage.33 Massive focal accumulations of CA have been reported in temporal lobes of individuals with TLE.16 It is possible that the composition of CA varies in each of these conditions, but there is insufficient evidence to suggest alterations by immunocytological techniques.17 CA accumulation in our patient occurred in the parahippocampal white matter. Hippocampal CA accumulation is well described in TLE.16 Abnormal amounts of CA deposition have been reported in hippocampal tissues resected from TLE patients.16 Radhakrishnan et al16 described 373 patients who underwent surgery for medically refractory mediotemporal lobe epilepsy with hippocampal sclerosis, and 129 (34.5%) were found to have CA on histopathological examination of the hippocampus. An earlier case series, completed by Kawamura et al,34 documented 34 patients with medically refractory TLE, in which 20 had substantial CA accumulation. The mechanism of CA accumulation in these patients is unknown. Chung and Horoupian4 observed a correlation between neuronal loss in hippocampal sclerosis and the amount of CA deposition, suggesting that CA accumulation may be a byproduct of neuronal loss. Van Paesschen et al35 reported similar findings. CA accumulation, however, has not always been observed to correlate with neuronal loss. For example, Erdamar et al7 reported increased CA deposition in CA1 and CA3, which did not correlate with areas of greater neuronal loss. In contrast to the idea that CA accumulation is a byproduct of neuronal loss, some have argued that CA deposition in TLE may be the result of increased demand on glucose metabolic pathways leading to neurodegeneration and sub-
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sequent CA formation.11 Our patient exhibited a different pattern of temporal lobe CA accumulation from seen in TLE patients. First, our patient did not exhibit any neuronal cell loss in the hippocampus, which is a typical finding in the pathology of TLE patients with CA accumulation. Second, CA accumulation in our patient occurred in white matter surrounding the hippocampus, as opposed to CA1/CA3 neuronal cell bodies in TLE patients. Pathological accumulation of CA in the white matter is a characteristic of APBG, but not hippocampal sclerosis. It is possible, although unlikely, that the findings in our patient represent an atypical presentation of APBD. CA accumulation in ABPD typically occurs in white matter tracts similar to our patient and has also been associated with focal signal intensity increases on MRI,13 although APBD also tends to be associated with diffuse brain and spinal atrophy.36 APBD is a genetic condition, and patients typically present between the fifth and seventh decades of life with progressive sensorimotor neuropathy, upper motor neuron symptoms, neurogenic bladder, and symptoms of increased cognitive impairment. Our patient did not report experiencing any of these symptoms typical of APBD. Given the lack of similarity between the clinical findings in our patient and patients with APBD, it does not seem likely that our patient has APBD. Focal CA accumulation in our patient was associated with nonenhancing elevated signal on T2-weighted MRI. Studies examining the clinical and neuroimaging characteristics of CA in hippocampal sclerosis have reported mixed results for an association between CA deposition and MRI signal alterations. One case series that examined 46 patients with hippocampal sclerosis and found no differences in the MRI characteristics of CA hippocampal sclerosis vs non-CA hippocampal sclerosis.37 Similarly, another report evaluating MRI characteristics of CA vs non-CA hippocampal sclerosis could not distinguish between either by T1- or T2weighted or proton-density MRI sequences.16 Others have shown signal intensity increases on fluid-attenuated inversion recovery images associated with CA, which seemed to be dependent on the degree of CA deposition.34 In APBD, which results in more diffuse CA deposition, MRI generally reveals nonspecific white matter changes and concomitant central nervous system atrophy.36,38 Taken together, these reports suggest that CA can result in MRI signal alterations such as those seen in our patient. The pattern of CA deposition in our patient seems to be distinct from that of APBD and hippocampal sclerosis and demonstrates that CA deposition may mimic brain neoplasia on T2-weighted MRI. Ultimately, the mechanism of CA accumulation in the parahippocampal region of our patient is unknown. Focal massive accumulations of CA have been reported in the circumstances reviewed here, but our patient does not fit any of these scenarios well. If the lesion had been identified as massive CA deposition preoperatively, serial imaging and clinical evaluation may have been warranted to establish whether the lesion was progressing. It is possible that the focal CA deposition in our patient represents an undescribed neurodegenerative condition, which may necessitate further follow-up and evaluation. This is a possibility, but there is no way to tell how this lesion has progressed over time because
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we have imaging only at a single point in time. This lesion may have been caused by any number of pathogenic mechanisms, including either congenital or neurodegenerative mechanisms. As of the time of this report, our patient has not followed up at our center and is presumably doing well after her resection. Unfortunately, it is not known whether she continues to experience the headaches that led her to have her initial brain MRI. Additionally, her current cognitive status is unknown.
CONCLUSION We report a unique case of massive local parahippocampal CA accumulation presenting as an intrinsic mass lesion on brain MRI. This case illustrates that, although unlikely, CA should be included in the differential diagnosis for patients presenting with brain MRI suggestive of nonenhancing, space-occupying lesions. Disclosure Adam O. Hebb, MD, is funded by the Epilepsy Foundation through the generous support of Abbott Laboratories. The authors have no personal financial or institutional interest in any of the drugs, materials, or devices described in this article.
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Acknowledgments We thank Elizabeth Stroup, PhD, for neuropsychological evaluation and Shahin Hakimian, MD, for intraoperative electrocorticography.
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