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Case Report

A clinically isolated syndrome: butterfly glioma

mimic

2
1
3
Ramshekhar Menon , Bejoy Thomas , Hariharan Venkat Easwer , Samvedam Sandhyamani , Amita Nair ,
4
4
Muralidharan Nair 1
1 Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum 695011, Kerala, India.
2 Department of Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology,
Trivandrum 695011, Kerala, India.
3 Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum 695011, Kerala, India.
4 Department of Pathology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum 695011, Kerala, India.
ABSTRA CT
The report explores a unique and treatable “butterfly”‑glioma mimic and the neuroimaging characteristics that help to diagnose
this entity. A 35‑year‑old patient presented with subacute‑onset, progressive frontal lobe dysfunction followed by features of raised
intracranial pressure. Neuroimaging features were consistent with a “butterfly” lesion that favored the possibility of a gliomatosis
cerebri with significant edema and marked corpus callosum and fornix thickening. Contrast‑enhanced and perfusion images
revealed a confluent tumefactive lesion with a characteristic “broken‑ring” pattern of enhancement, mass‑effect and low perfusion;
features favoring an alternative inflammatory pathology. This was peculiar as calloso‑forniceal involvement of this nature has not
been previously reported in inflammatory demyelinating mass lesions. This was confirmed as a tumefactive demyelination on
histopathology. Following treatment, on clinical and imaging follow‑up, significant resolution was evident suggesting a monophasic
illness. This case highlights the stringent clinico‑radiological‑pathological approach required in the evaluation and management of
butterfly lesions despite the striking imaging appearances. Tumefactive demyelination in this patient represents a clinically isolated
syndromic presentation of an inflammatory pathology that can resemble gliomatosis cerebri. These “butterfly”‑glioma mimics are
scarcely reported in the literature, are eminently treatable with variable prognosis and prone for relapse.

Key words: Butterfly lesions, glioma, tumefactive demyelination

INTRODUCTION prior to the index evaluation. The patient was observed
by relatives to be apathetic, occasionally agitated
Confluent “butterfly” lesions involving the corpus and argumentative and over 15 days developed
callosum often portend a poor prognosis in patients social dysinhibition, urinary and fecal incontinence.
presenting with acute-subacute encephalopathy, with Excessive day time somnolence was also noted. The
or without focal signs and raised intracranial pressure. relatives also noted progressive inattention and memory
Gliomatosis cerebri is often the primary pathological impairment for a week prior to presentation. There was
substrate considered in this situation. This report no history of any fever, headache, vomiting, loss of
highlights an important mimic of “butterfly”-glioma. consciousness, seizures, hallucinations, delusions,
focal limb weakness, imbalance nor any history
CASE REPORT suggestive of cranial nerve involvement.

A 35-year-old well-educated previously healthy Clinical examination revealed a conscious oriented
male with no medical co-morbidities presented with patient with normal general physical examination
complaints of behavioral disturbances for 1 month findings. Mini Mental Status Examination score was
26/30 with impaired attention. There was a distinct
Access this article online lack of insight and impaired abstract thinking and
Quick Response Code: judgment, with normal performance on tests of
Website: executive function. The rest of the cognitive evaluation
www.nnjournal.net
was normal. There was no evidence of papilledema,
DOI: ataxia or extrapyramidal signs. The plantar responses
10.4103/2347-8659.154435 were bilaterally extensor consistent with a bipyramidal
dysfunction.

Corresponding Author: Dr. Ramshekhar Menon, Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences
and Technology, Medical College Campus, Trivandrum 695011, Kerala, India. E‑mail: rsnmenon@sctimst.ac.in

174 Neuroimmunol Neuroinflammation | Volume 2 | Issue 3 | July 15, 2015 Neuroimmunol Neuroinflammation | Volume 2 | Issue 3 | July 15, 2015 175