Adult neuronal ceroid lipofuscinosis with clinical findings consistent with a butterfly glioma

Hammersen, Stefanie; Brock, M.; Cervós-Navarro, J.

doi:10.3171/jns.1998.88.2.0314

Cited by 13 publications

(6 citation statements)

References 22 publications

(24 reference statements)

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“…Studies on bGBM have combined this subset of patients into the same cohort of patients with non-bGBM even though they only account for 3–15 % of patients [1, 27, 38, 42, 43]. The majority of studies limited to only patients with bGBM are small case series [10–17] aimed to report rare presenting symptoms including catatonia [11], muscle atrophy [13], neuropsychological deficits [14], and leptomeningeal metastases [16]. To date, there have been few previous non-case report-based studies limited to bGBM patients (Table 1) [7, 9, 18, 20, 41].…”

Section: Discussionmentioning

confidence: 99%

The butterfly effect on glioblastoma: is volumetric extent of resection more effective than biopsy for these tumors?

et al. 2014

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A subset of patients with glioblastoma (GBM) have butterfly GBM (bGBM) that involve both cerebral hemispheres by crossing the corpus callosum. The prognoses, as well as the effectiveness of surgery and adjuvant therapy, are unclear because studies are few and limited. The goals of this study were to: (1) determine if bGBM have worse outcomes than patients with non-bGBM, (2) determine if surgery is more effective than biopsy, and (3) identify factors independently associated with improved outcomes for these patients. Adult patients who underwent surgery for a newly diagnosed primary GBM at an academic tertiary-care institution between 2007 and 2012 were retrospectively reviewed and tumors were volumetrically measured. Of the 336 patients with newly diagnosed GBM who were operated on, 48 (14 %) presented with bGBM, where 29 (60 %) and 19 (40 %) underwent surgical resection and biopsy, respectively. In multivariate analysis, a bGBM was independently associated with poorer survival [HR (95 % CI) 1.848 (1.250–2.685), p < 0.003]. In matched- pair analysis, patients who underwent surgical resection had improved median survival than biopsy patients (7.0 vs. 3.5 months, p = 0.03). In multivariate analysis, increasing percent resection [HR (95 % CI) 0.987 (0.977–0.997), p = 0.01], radiation [HR (95 % CI) 0.431 (0.225–0.812), p = 0.009], and temozolomide [HR (95 % CI) 0.413 (0.212–0. 784), p = 0.007] were each independently associated with prolonged survival among patients with bGBM. This present study shows that while patients with bGBM have poorer prognoses compared to non-bGBM, these patients can also benefit from aggressive treatments including debulking surgery, maximal safe surgical resection, temozolomide chemotherapy, and radiation therapy.

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Section: Discussionmentioning

confidence: 99%

The butterfly effect on glioblastoma: is volumetric extent of resection more effective than biopsy for these tumors?

et al. 2014

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“…Olgumuzun steroid tedavisine hızlı cevabı ve geniş spinal kord tutulumu nedeniyle gliom dışlanmıştır. Lenfoma, metastaz, toksoplazmozis, granülositik sarkom ve nöronal seroid lipofusinoz hastalıklarında da ''butterfly'' lezyonlar rapor edilmiştir (20,21,22,23).…”

Section: Discussionunclassified

Supratentorial MRG Bulguları Serebral Adrenolökodistrofiye Benzeyen ADEM Olgusu

Beyazal

Ünal

Yılmaz

et al. 2014

npa

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A 9-year-old male admitted for syncope also had the complains of pain and numbness in his legs and frequent falling down. There was a history of upper respiratory tract infection 10 days before. On neurologic examination, paraparesia and fall a sleep were identified. On magnetic resonance imaging, the symetric signal increases were seen in biparieto-occipital white matter intented to corpus callosum at T2-weighted sequences and cytotoxic edema was seen at diffusion-weighted images. Heterogeneous contrast enhancement was seen on these areas. In addition, at the C7-Th5 vertebrae levels, spinal cord had diffuse increased signal intensity and contrast enhancement. Acute disseminated encephalomyelitis was thought based on clinical and radiological findings. Steroid therapy was started. Significant improvement was shown after treatment. On 2-year follow-up, there was no recurrence. In conclusion, it must be kept in mind that acute disseminated encephalomyelitis can rarely present with biparieto-occipital involvement which extends to corpus callosum and can mimic adrenoleukodystrophy. For the differential diagnosis butterfly glioma, tumefactive demyelinating lesions or multiple sclerosis should be considered.

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“…Some studies have reported brain atrophy, low signal intensity on T2-weighted MR images of the putamen (14), high signal intensity on T2-weighted MR images of the cortex (15), high signal intensity on T2-weighted images of the cortex and basal ganglia (16) and periventricular-predominant leukoencephalopathy (3). Other studies have demonstrated the markedly localized accumulation of neuronal lipofuscins mimicking mass lesions (17), suggesting the presence of a heterogeneous pathological condition. The relationships between genetic mutations and MRI findings in patients with adult neuronal ceroid lipofuscinosis remain unclear ; however, periventricular-predominant leukoencephalopathy can be considered to be a finding suggestive of adult neuronal ceroid lipofuscinosis.…”

Section: Discussionmentioning

confidence: 99%

Neuronal Ceroid Lipofuscinosis with Early-onset Dementia and Periventricular Leukoencephalopathy in which a Skin Biopsy was Diagnostically Useful

Ueda¹,

Narushima²,

Ishida³

et al. 2013

Intern. Med.

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Neuronal ceroid lipofuscinosis (NCL) is a rare disease with onset typically during childhood; however, that developing during adulthood can lead to early-onset dementia. We report a 54-year-old man whose onset coincided with speech impairment, amnesia and dyscalculia. On brain MRI, marked diffuse leukoencephalopathy with periventricular predominance was observed. On a skin biopsy, characteristic fingerprint images were noted, and the patient was diagnosed with NCL. The differential diagnosis of cognitive impairment with leukoencephalopathy is wide ranging; however, when marked symmetrical periventricular-predominant leukoencephalopathy is prevalent and no peripheral neuropathy or gait disorders are evident, a diagnosis of NCL should be suspected and a skin biopsy should be performed.

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Adult neuronal ceroid lipofuscinosis with clinical findings consistent with a butterfly glioma

Cited by 13 publications

References 22 publications

The butterfly effect on glioblastoma: is volumetric extent of resection more effective than biopsy for these tumors?

The butterfly effect on glioblastoma: is volumetric extent of resection more effective than biopsy for these tumors?

Supratentorial MRG Bulguları Serebral Adrenolökodistrofiye Benzeyen ADEM Olgusu

Neuronal Ceroid Lipofuscinosis with Early-onset Dementia and Periventricular Leukoencephalopathy in which a Skin Biopsy was Diagnostically Useful

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