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low‑grade gliomas, oligodendrogliomas, and ependymomas.[2] The exact cause of occurrence of multiplicity is unknown. These lesions may be the result of neoplastic transformation of a field, rendering a wide area of the brain susceptible to neoplastic growth. Multiple embryonic residues scattered at different sites, and the role of cells with blastomere potential, have also been implicated.[3] A solitary glioblastoma has an overall survival of 11–18 months while the overall survival is just 4–7 months in the case of multiple glioblastomas. The disease process may remain quiescent for some time, manifesting later with a rapid growth, as seen in the third case. It is believed that migration of glial tumor cells to new sites and changing from traveling mode to growing mode is the basis for multiplicity.[4] In clinical presentation, these tumors have no localizing signs and are usually more aggressive due to the multiplicity.[1] Our second case presented with an ultra‑short clinical history of 5 days, raising the suspicion of an inflammatory pathology. Radiologically, these lesions pose a diagnostic dilemma especially in the absence of typical clinical findings.[5] These lesions may masquerade a metastasis or a brain abscess. Management of these lesions remains controversial. One school of thought proposes aggressive surgery for all lesions citing the possibility of a better outcome related to the administration of adjuvant radiotherapy after tumor debulking; the other school, however, advocates surgery only for the larger lesion and proposes establishement of a histological diagnosis by stereotactic biopsy for the remaining lesions. Surgery for a single lesion in multiple glioblastomas may cause further clinical deterioration due to an ipsilateral brain shift, which may prove fatal. This management decision is in contrast to the management of multiple low‑grade gliomas where surgery for multiple lesions may be justified and may give optimum results.[6]
Ankur Kapoor, Sandeep Mohindra, Navneet Singla, Harsimrat Bir Singh Sodhi, Debjyoti Chatterjee1, Sunil K. Gupta Departments of Neurosurgery and 1Histopathology, PGIMER, Chandigarh, India E‑mail: [email protected]
References 1.
2.
3. 4.
452
di Russo P, Perrini P, Pasqualetti F, Meola A, Vannozzi R. Management and outcome of high‑grade multicentric gliomas: A contemporary single‑institution series and review of the literature. Acta Neurochir (Wien) 2013;155:2245‑51. Zamponi N, Rychlicki F, Ducati A, Regnicolo L, Salvolini U, Ricciuti RA. Multicentric glioma with unusual clinical presentation. Childs Nerv Syst 2001;17:101‑5. Batzdorf U, Malamud N. The problem of multicentric gliomas. J Neurosurg 1963;20:122‑36. Fuchsmann C, Traverse‑Glehen A, Durbec M, Dubreuil C, Tringali S. Glioblastoma multiforme mimicking a frontal abscess after surgery for a large vestibular schwannoma. Eur Ann Otorhinolaryngol Head Neck Dis 2010;127:46‑8.
5.
6.
Kitanaka C, Shitara N, Nakagomi T, Nakamura H, Genka S, Nakagawa K, et al. Postradiation astrocytoma. Report of two cases. J Neurosurg 1989;70:469‑74. Borovich B, Mayer M, Gellei B, Peyser E, Yahel M. Multifocal glioma of the brain. Case report. J Neurosurg 1976;45:229‑32. Access this article online
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www.neurologyindia.com DOI: 10.4103/0028-3886.158267 PMID: xxxxx
Diffusion restriction in fulminant subacute sclerosing panencephalitis: Report of an unusual finding Sir, S u b a c u t e S c l e ro s i n g Pa n e n c e p h a l i t i s ( SS P E ) i s a fatal progressive encephalitis caused by abnormal persistence of the measles virus infection in the central nervous system.[1] SSPE usually occurs in young children and adolescents; however, it is also known to affect adults and pregnant women. The clinical picture is characterized by behavioural abnormalities, cognitive decline, myoclonic epilepsy and seizures.[2] Diagnosis of SSPE is dependent upon a combination of clinical features, characteristic EEG abnormalities and elevated antimeasles antibody titre in the serum and the CSF.[3] MRI findings in SSPE have been described in previous studies.[3‑7] We report a patient of fulminant SSPE who showed atypical MRI findings on serial diffusion weighted sequences. A 15-year old girl presented with a 5 month history of slowly progressive cognitive decline characterized by behavioural changes, slowing of daily activities and deterioration of handwriting. She also had intermittent, involuntary jerky movements of head and bilateral upper limbs for 4 months. Initially, she was evaluated at another centre with a cranial MRI which showed hyperintensities on T2 FLAIR sequences involving the cortex and the subcortical white matter in the right temporal and the left parieto‑occipital lobes without any diffusion restriction or contrast enhancement. However, no specific treatment was initiated at that time. One month later, the girl developed 4 episodes of seizures and was re‑admitted.
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Repeat MRI showed new T2W hyperintense lesions in the right parieto‑occipital lobes without any significant diffusion restriction or contrast enhancement. The lesions seen in the previous scan had become less conspicuous by this time. Suspecting a demyelinating pathology, the patient was treated with intravenous methylprednisolone followed by oral steroids to which she showed partial improvement. However, over the next 2‑3 weeks, there was a symptomatic relapse with increasing myoclonic jerks and apathy. She was admitted at our centre this time, about 5 months into the illness, with paucity of left upper and lower limb movements which progressed to complete paralysis over the next 3 days. There was no significant past medical history including any history of measles in the childhood. The vaccination status for measles was also uncertain. Clinical examination revealed apathy, a MMSE score of 20, mild dysarthria, left UMN facial palsy with left hemiplegia (MRC grade I). The laboratory findings revealed elevated measles antibody titres both in the serum (>250 IU/ml) and in the CSF (1:625). Her vasculitic profile and serological markers for autoimmune encephalitis were negative. Her EEG showed characteristic long interval periodic complexes [Figure 1]. A repeat MRI of the brain showed new FLAIR hyperintensities, albeit with patchy restricted diffusion, in the right frontal lobe. Hyperintensities were also seen in bilateral posteromedial thalami [Figure 2]. The clinical presentation, elevated measles antibody titres, EEG and MRI findings were consistent with probable SSPE as per Dyken’s criteria. Meanwhie, the patient’s sensorium continued to decline with development of marked dysautonomia requiring ventilatory and hemodynamic support. Repeat MRI showed multifocal cortical swelling and FLAIR hyperintensities involving bilateral frontal, parietal and temporal lobes. Extensive confluent hyperintensities were
a
also noted involving bilateral centrum semiovale, corona radiata, external capsule, posterior limb of the internal capsule, corpus callosum, hypothalami and the subcortical white matter of bilateral frontal and occipital lobes. All the lesions showed restricted diffusion with very low ADC values except for the right frontal lobe lesion which showed facilitated diffusion [Figure 3]. Clinically, the patient continued to deteriorate and eventually succumbed to her illness. The various neurological complications of measles infection include post measles encephalitis, measles inclusion body encephalitis, SSPE and transverse myelitis.[5] SSPE is a rare neurodegenerative disease characterized by a chronic persistent progressive encephalitis. The patients generally have a history of measles infection prior to the age of 2 years followed by a latent period of about 6‑8 years before SSPE sets in. SSPE has got a variable natural history ranging from an acute onset fulminant illness resulting in death within a few weeks to 6 months, to a more insidious onset, slowly progressive disorder with multiple remission and relapses. Our patient had an atypical presentation with a fulminant clinical course leading to death within 6 months of the disease onset. Such a natural history has been noted in about 10% of the cases.[3] Even though our patient had no clinically documented attack of measles in the past, the occurrence of SSPE can be explained by a possible subclinical measles infection before the age of 1 year which is rather common in our country.[3,8] In the early stages of SSPE, MRI shows asymmetric T2/FLAIR hyperintensities involving the cortex and the subcortical white matter bilaterally with predominant parieto‑occipital lobe involvement. The lesions may show resolution with time and
b
Figure 1: Electroencephalography (EEG) findings of the patient. (a) Sixteen channel EEG recording (with sensitivity of 7 µV/mm and paper speed of 10 s/ page) shows the classical generalized long-interval periodic complexes (Rademecker’s complex) composed of delta and admixed spike and wave activity at an interburst interval of 3.5 s, seen throughout the record. The interburst interval shows attenuated background with superimposed theta-delta slowing asymmetrically expressed, more over the right hemisphere. (b) Same EEG at 30 s/page shows the periodicity more clearly
Neurology India / May 2015 / Volume 63 / Issue 3
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a
b
c
d
Figure 2: Magnetic resonance imaging at 5 months after the onset of symptoms. Axial fluid-attenuated inversion recovery images (a and b) show hyperintensities involving the cortex and subcortical white matter of right frontal lobe and bilateral thalami. Diffusion weighted trace (c) and apparent diffusion coefficient map (d) shows restricted diffusion involving both gray and white matter
on follow‑up imaging, new lesions may appear with gradually progressive cortical volume loss. In the advanced stages, there is more widespread involvement of the deep periventricular white matter. Rarely, lesions have also been described involving the corpus callosum, brainstem, thalamus, cerebellum and even the spinal cord.[3] Similar imaging findings were also noted in our case with migratory cortical‑subcortical white matter lesions in the early stages and involvement of deep white matter, corpus callosum, thalami and brain stem in the later stages. Studies have shown a poor correlation between the clinical status and the cranial imaging appearance in SSPE.[5] Few studies till date have described the role of diffusion weighted imaging in SSPE. [6,7,9] Majority of them have demonstrated increased ADC values in the involved cerebral 454
parenchyma. Alkan et al.[7] correlated the clinical stage of the disease with the diffusion weighted MRI findings. They found that the ADC values progressively increased with the advancing clinical stage of the disease. The histopathological correlates of increased diffusion include demyelination, neuronal loss and parenchymal necrosis. Diffusion restriction is a widely documented finding in acute measles encephalitis and suggests the cytotoxic edema in these cases.[6] However, diffusion restriction has not been previously reported in lesions of SSPE. Hideaki et al.[9] studied serial DWI findings in a case of SSPE. They demonstrated that reduction in the ADC values in the frontal lobe white matter correlated with clinical progression of disease. However, no ADC changes were noted in the gray or white matter lesions
Neurology India / May 2015 / Volume 63 / Issue 3
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a
b
c
d
e
f
Figure 3: Follow-up imaging. Axial fluid-attenuated inversion recovery (a) images show confluent hyperintensities involving bilateral fronto-parietal lobes. Coronal T2-weighted image (b) shows involvement of the left temporal lobe, bilateral basal ganglia and external capsule. Diffusion weighted (DW) trace (c) and apparent diffusion coefficient (ADC) map (d) shows restricted diffusion in bilateral internal capsules, subcortical white matter of right frontal and bilateral occipital lobes. DW trace and ADC map (e and f) at higher level show confluent areas of restricted diffusion in bilateral centrum semiovale. Areas of increased ADC are noted in the right frontal lobe
in their case. Oguz et al.[8] reported DWI findings in two cases of rapidly progressive SSPE. In one of their cases, there was increased diffusion in all the lesions with subtle restricted diffusion at their margins. In the other case, symmetric restricted diffusion was noted in bilateral middle cerebellar peduncles which the authors attributed to the pontocerebellar degeneration secondary to the pontine lesions. Our case was peculiar in that the restriction in diffusion was extensive and involved bilateral cerebral hemispheric white matter, internal capsule, thalami and midbrain. This pattern of restricted diffusion is probably due to cytotoxic or intramyelinic edema secondary to the necrotizing leucoencephalopathy. Though SSPE is classically described as a chronic encephalitis, the fulminant form can have acute necrotizing encephalitis like presentation. Mahadevan et al.[10] reported a case of fulminant SSPE with histopathological findings of necrotizing leukoencephalitis showing diffuse demyelination and foci of cavitation. Further, the right frontal lobe lesion which showed diffusion restriction in the initial scan was showing increased ADC on follow‑up imaging. The reason for the occurrence of transient cytotoxic edema with subsequent resolution into vasogenic edema remains unclear. We speculate that active demyelination
is followed by astrogliosis and neuronal loss which may be responsible for the increased ADC. Further studies evaluating the serial DW images in cases having a fulminant SSPE with histopathological correlation are required for a better understanding of the progression of imaging findings. To conclude, we have reported a case of fulminant SSPE with atypical MRI features of extensive diffusion restriction.
Dhaval Shah, K. Srinivasan, Tejas Sakale1, S. Sajith1, C. Kesavadas Departments of Imaging Sciences and Interventional Radiology and 1Neurology, Sree Chitra Thirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India E-mail: [email protected]
References 1. 2.
3. 4.
Graves MC. Subacute Sclerosing panencephalitis. Symposium on neurovirology. Neurol Clin 1984; 2:267‑280. Jabbour JT, Garcia JH, Lemmi H, Ragaland J, Duenas DA, Sever JL. Subacute Sclerosing panencephalitis. A multidisciplinary study of eight cases. JAMA 1969;207:2248-54. Gutierrez J, Issacson RS, Koppel BS. Subacute sclerosing panencephalitis: An update. Dev Med Child Neurol 2010;52(10):901‑7. Sener RN. Subacute sclerosing panencephalitis findings at MR imaging, diffusion MR imaging and proton MR spectroscopy. AJNR Am J
Neurology India / May 2015 / Volume 63 / Issue 3
455
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Letters to Editor
Neuroradiol 2004;25:892-4. Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J 2002;78:63-70. 6. Oguz KK, Celebi A, Anlar B. MR imaging, diffusion‑weighted imaging and MR spectroscopy findings in acute rapidly progressive subacute sclerosing panencephalitis. Brain Dev 2007; 29 (5):306‑11. 7. Alkan A, Korkmaz L, Sigirci A, Kutlu R, Yakinci C, Erdem G, et al. Subacute sclerosing panencephalitis: Relationship between clinical stage and diffusion‑weighted imaging findings. J Magn Reson Imaging 2006; 23(3):267‑72. 8. Manayani DJ, Abraham M, Gnanamuthu C, Solomon T, Alexander M, Sridharan G. SSPE‑the continuing challenge: A study based on serological evidence from a tertiary care centre in India. Indian J Med Microbiol 2002;20(1):16‑8. 9. Kanemura H, Aihara M. Serial diffusion‑weighted imaging in subacute sclerosing panencephalitis. Pediatr Neurol 2008;38(6):430‑4. 10. Mahadevan A, Vaidya SR, Wairagkar NS, Khedekar D, Kovoor JM, Santosh V, et al. Case of fulminant‑SSPE associated with measles genotype D7 from India: An autopsy study. Neuropathology 2008;28(6):621‑6. 5.
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Bilateral isolated basal ganglia bleed: An atypical presentation of Japanese encephalitis Sir, Japanese encephalitis (JE), a mosquito‑borne flaviviral encephalitis, remains a major health problem in the Indian subcontinent.[1] Though no age is immune, children between 5 and 15 years of age happen to bear the brunt of the disease. The typical cranial imaging abnormalities in JE involve bilateral thalami, basal ganglia, substantia nigra, brainstem, cerebellum and cerebral cortical and white matter.[2,3] Although haemorrhagic lesions involving bilateral thalami have been described previously, such lesions are usually accompanied by similar lesions elsewhere in the brain. Bilateral isolated basal ganglia bleed as the sole imaging abnormality in JE has not been reported before. Here, we report the case of a child with the diagnosis of JE wherein the cranial imaging revealed bilateral isolated basal ganglia bleed. 456
A 5‑year‑old boy born out of a non‑consanguineous parentage and having a normal perinatal and developmental history presented with a history of fever of 5 days duration and 4 episodes of generalised tonic‑clonic seizures in 1 day. There was no regaining of consciousness in between the episodes of seizures suggesting the presence of status epilepticus. The fever was associated with headache and vomiting although no history of loose stools, cold or cough could be elicited. A diagnosis of febrile status epilepticus was made at this point. On examination, the child was febrile (100.2° F). His pulse rate was 130/min, and he had a blood pressure of 98/72 mmHg and a respiratory rate of 28/min. He was stuporous with no eye opening and minimal limb movements to painful stimulus. His fundus examination was normal. His pupils were equal but sluggishly reacting to light. The doll’s eye response was preserved. The motor examination showed an increased muscle tone in all 4 limbs with brisk deep tendon reflexes and an extensor plantar response bilaterally. There was terminal neck stiffness with a positive Kernig’s sign. Complete hemogram showed leucocytosis (16,000 cells/cumm) with a normal platelet count. Renal and hepatic functions including serum electrolytes were normal. Computed tomography (CT) of the brain showed bleed in the right basal ganglia. Magnetic resonance imaging (MRI) showed blooming in bilateral basal ganglia on gradient echo (GRE) sequence suggestive of bleed [Figure 1]. Cerebrospinal fluid (CSF) examination showed mildly raised protein (62 mg/dl), normal glucose with mild lymphocytic pleocytosis (28 cells per cumm) and positive IgM anti‑JE antibodies. CSF polymerase chain reaction (PCR) analysis for cytomegalovirus, herpes simplex virus and Mycobacterium tuberculosis were negative. Serological testing for human immunodeficiency virus was negative. The child was started on antiepileptics and put on mechanical ventilator. The seizures were eventually controlled on two antiepileptic drugs. He was later discharged in an akinetic‑mute state. Prognosis in patients with JE is often poor with occurrence of mortality in about one third of the patients; and, half of the patients who survive are often left with severe
a
b
c
Figure 1: CT brain showing bleed in the right basal ganglia (a); MRI brain GRE sequence shows blooming in bilateral basal ganglia on gradient echo imaging (b and c; white arrow)
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Letters to Editor
low‑grade gliomas, oligodendrogliomas, and ependymomas.[2] The exact cause of occurrence of multiplicity is unknown. These lesions may be the result of neoplastic transformation of a field, rendering a wide area of the brain susceptible to neoplastic growth. Multiple embryonic residues scattered at different sites, and the role of cells with blastomere potential, have also been implicated.[3] A solitary glioblastoma has an overall survival of 11–18 months while the overall survival is just 4–7 months in the case of multiple glioblastomas. The disease process may remain quiescent for some time, manifesting later with a rapid growth, as seen in the third case. It is believed that migration of glial tumor cells to new sites and changing from traveling mode to growing mode is the basis for multiplicity.[4] In clinical presentation, these tumors have no localizing signs and are usually more aggressive due to the multiplicity.[1] Our second case presented with an ultra‑short clinical history of 5 days, raising the suspicion of an inflammatory pathology. Radiologically, these lesions pose a diagnostic dilemma especially in the absence of typical clinical findings.[5] These lesions may masquerade a metastasis or a brain abscess. Management of these lesions remains controversial. One school of thought proposes aggressive surgery for all lesions citing the possibility of a better outcome related to the administration of adjuvant radiotherapy after tumor debulking; the other school, however, advocates surgery only for the larger lesion and proposes establishement of a histological diagnosis by stereotactic biopsy for the remaining lesions. Surgery for a single lesion in multiple glioblastomas may cause further clinical deterioration due to an ipsilateral brain shift, which may prove fatal. This management decision is in contrast to the management of multiple low‑grade gliomas where surgery for multiple lesions may be justified and may give optimum results.[6]
Ankur Kapoor, Sandeep Mohindra, Navneet Singla, Harsimrat Bir Singh Sodhi, Debjyoti Chatterjee1, Sunil K. Gupta Departments of Neurosurgery and 1Histopathology, PGIMER, Chandigarh, India E‑mail: [email protected]
References 1.
2.
3. 4.
452
di Russo P, Perrini P, Pasqualetti F, Meola A, Vannozzi R. Management and outcome of high‑grade multicentric gliomas: A contemporary single‑institution series and review of the literature. Acta Neurochir (Wien) 2013;155:2245‑51. Zamponi N, Rychlicki F, Ducati A, Regnicolo L, Salvolini U, Ricciuti RA. Multicentric glioma with unusual clinical presentation. Childs Nerv Syst 2001;17:101‑5. Batzdorf U, Malamud N. The problem of multicentric gliomas. J Neurosurg 1963;20:122‑36. Fuchsmann C, Traverse‑Glehen A, Durbec M, Dubreuil C, Tringali S. Glioblastoma multiforme mimicking a frontal abscess after surgery for a large vestibular schwannoma. Eur Ann Otorhinolaryngol Head Neck Dis 2010;127:46‑8.
5.
6.
Kitanaka C, Shitara N, Nakagomi T, Nakamura H, Genka S, Nakagawa K, et al. Postradiation astrocytoma. Report of two cases. J Neurosurg 1989;70:469‑74. Borovich B, Mayer M, Gellei B, Peyser E, Yahel M. Multifocal glioma of the brain. Case report. J Neurosurg 1976;45:229‑32. Access this article online
Website:
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www.neurologyindia.com DOI: 10.4103/0028-3886.158267 PMID: xxxxx
Diffusion restriction in fulminant subacute sclerosing panencephalitis: Report of an unusual finding Sir, S u b a c u t e S c l e ro s i n g Pa n e n c e p h a l i t i s ( SS P E ) i s a fatal progressive encephalitis caused by abnormal persistence of the measles virus infection in the central nervous system.[1] SSPE usually occurs in young children and adolescents; however, it is also known to affect adults and pregnant women. The clinical picture is characterized by behavioural abnormalities, cognitive decline, myoclonic epilepsy and seizures.[2] Diagnosis of SSPE is dependent upon a combination of clinical features, characteristic EEG abnormalities and elevated antimeasles antibody titre in the serum and the CSF.[3] MRI findings in SSPE have been described in previous studies.[3‑7] We report a patient of fulminant SSPE who showed atypical MRI findings on serial diffusion weighted sequences. A 15-year old girl presented with a 5 month history of slowly progressive cognitive decline characterized by behavioural changes, slowing of daily activities and deterioration of handwriting. She also had intermittent, involuntary jerky movements of head and bilateral upper limbs for 4 months. Initially, she was evaluated at another centre with a cranial MRI which showed hyperintensities on T2 FLAIR sequences involving the cortex and the subcortical white matter in the right temporal and the left parieto‑occipital lobes without any diffusion restriction or contrast enhancement. However, no specific treatment was initiated at that time. One month later, the girl developed 4 episodes of seizures and was re‑admitted.
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Repeat MRI showed new T2W hyperintense lesions in the right parieto‑occipital lobes without any significant diffusion restriction or contrast enhancement. The lesions seen in the previous scan had become less conspicuous by this time. Suspecting a demyelinating pathology, the patient was treated with intravenous methylprednisolone followed by oral steroids to which she showed partial improvement. However, over the next 2‑3 weeks, there was a symptomatic relapse with increasing myoclonic jerks and apathy. She was admitted at our centre this time, about 5 months into the illness, with paucity of left upper and lower limb movements which progressed to complete paralysis over the next 3 days. There was no significant past medical history including any history of measles in the childhood. The vaccination status for measles was also uncertain. Clinical examination revealed apathy, a MMSE score of 20, mild dysarthria, left UMN facial palsy with left hemiplegia (MRC grade I). The laboratory findings revealed elevated measles antibody titres both in the serum (>250 IU/ml) and in the CSF (1:625). Her vasculitic profile and serological markers for autoimmune encephalitis were negative. Her EEG showed characteristic long interval periodic complexes [Figure 1]. A repeat MRI of the brain showed new FLAIR hyperintensities, albeit with patchy restricted diffusion, in the right frontal lobe. Hyperintensities were also seen in bilateral posteromedial thalami [Figure 2]. The clinical presentation, elevated measles antibody titres, EEG and MRI findings were consistent with probable SSPE as per Dyken’s criteria. Meanwhie, the patient’s sensorium continued to decline with development of marked dysautonomia requiring ventilatory and hemodynamic support. Repeat MRI showed multifocal cortical swelling and FLAIR hyperintensities involving bilateral frontal, parietal and temporal lobes. Extensive confluent hyperintensities were
a
also noted involving bilateral centrum semiovale, corona radiata, external capsule, posterior limb of the internal capsule, corpus callosum, hypothalami and the subcortical white matter of bilateral frontal and occipital lobes. All the lesions showed restricted diffusion with very low ADC values except for the right frontal lobe lesion which showed facilitated diffusion [Figure 3]. Clinically, the patient continued to deteriorate and eventually succumbed to her illness. The various neurological complications of measles infection include post measles encephalitis, measles inclusion body encephalitis, SSPE and transverse myelitis.[5] SSPE is a rare neurodegenerative disease characterized by a chronic persistent progressive encephalitis. The patients generally have a history of measles infection prior to the age of 2 years followed by a latent period of about 6‑8 years before SSPE sets in. SSPE has got a variable natural history ranging from an acute onset fulminant illness resulting in death within a few weeks to 6 months, to a more insidious onset, slowly progressive disorder with multiple remission and relapses. Our patient had an atypical presentation with a fulminant clinical course leading to death within 6 months of the disease onset. Such a natural history has been noted in about 10% of the cases.[3] Even though our patient had no clinically documented attack of measles in the past, the occurrence of SSPE can be explained by a possible subclinical measles infection before the age of 1 year which is rather common in our country.[3,8] In the early stages of SSPE, MRI shows asymmetric T2/FLAIR hyperintensities involving the cortex and the subcortical white matter bilaterally with predominant parieto‑occipital lobe involvement. The lesions may show resolution with time and
b
Figure 1: Electroencephalography (EEG) findings of the patient. (a) Sixteen channel EEG recording (with sensitivity of 7 µV/mm and paper speed of 10 s/ page) shows the classical generalized long-interval periodic complexes (Rademecker’s complex) composed of delta and admixed spike and wave activity at an interburst interval of 3.5 s, seen throughout the record. The interburst interval shows attenuated background with superimposed theta-delta slowing asymmetrically expressed, more over the right hemisphere. (b) Same EEG at 30 s/page shows the periodicity more clearly
Neurology India / May 2015 / Volume 63 / Issue 3
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a
b
c
d
Figure 2: Magnetic resonance imaging at 5 months after the onset of symptoms. Axial fluid-attenuated inversion recovery images (a and b) show hyperintensities involving the cortex and subcortical white matter of right frontal lobe and bilateral thalami. Diffusion weighted trace (c) and apparent diffusion coefficient map (d) shows restricted diffusion involving both gray and white matter
on follow‑up imaging, new lesions may appear with gradually progressive cortical volume loss. In the advanced stages, there is more widespread involvement of the deep periventricular white matter. Rarely, lesions have also been described involving the corpus callosum, brainstem, thalamus, cerebellum and even the spinal cord.[3] Similar imaging findings were also noted in our case with migratory cortical‑subcortical white matter lesions in the early stages and involvement of deep white matter, corpus callosum, thalami and brain stem in the later stages. Studies have shown a poor correlation between the clinical status and the cranial imaging appearance in SSPE.[5] Few studies till date have described the role of diffusion weighted imaging in SSPE. [6,7,9] Majority of them have demonstrated increased ADC values in the involved cerebral 454
parenchyma. Alkan et al.[7] correlated the clinical stage of the disease with the diffusion weighted MRI findings. They found that the ADC values progressively increased with the advancing clinical stage of the disease. The histopathological correlates of increased diffusion include demyelination, neuronal loss and parenchymal necrosis. Diffusion restriction is a widely documented finding in acute measles encephalitis and suggests the cytotoxic edema in these cases.[6] However, diffusion restriction has not been previously reported in lesions of SSPE. Hideaki et al.[9] studied serial DWI findings in a case of SSPE. They demonstrated that reduction in the ADC values in the frontal lobe white matter correlated with clinical progression of disease. However, no ADC changes were noted in the gray or white matter lesions
Neurology India / May 2015 / Volume 63 / Issue 3
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Letters to Editor
a
b
c
d
e
f
Figure 3: Follow-up imaging. Axial fluid-attenuated inversion recovery (a) images show confluent hyperintensities involving bilateral fronto-parietal lobes. Coronal T2-weighted image (b) shows involvement of the left temporal lobe, bilateral basal ganglia and external capsule. Diffusion weighted (DW) trace (c) and apparent diffusion coefficient (ADC) map (d) shows restricted diffusion in bilateral internal capsules, subcortical white matter of right frontal and bilateral occipital lobes. DW trace and ADC map (e and f) at higher level show confluent areas of restricted diffusion in bilateral centrum semiovale. Areas of increased ADC are noted in the right frontal lobe
in their case. Oguz et al.[8] reported DWI findings in two cases of rapidly progressive SSPE. In one of their cases, there was increased diffusion in all the lesions with subtle restricted diffusion at their margins. In the other case, symmetric restricted diffusion was noted in bilateral middle cerebellar peduncles which the authors attributed to the pontocerebellar degeneration secondary to the pontine lesions. Our case was peculiar in that the restriction in diffusion was extensive and involved bilateral cerebral hemispheric white matter, internal capsule, thalami and midbrain. This pattern of restricted diffusion is probably due to cytotoxic or intramyelinic edema secondary to the necrotizing leucoencephalopathy. Though SSPE is classically described as a chronic encephalitis, the fulminant form can have acute necrotizing encephalitis like presentation. Mahadevan et al.[10] reported a case of fulminant SSPE with histopathological findings of necrotizing leukoencephalitis showing diffuse demyelination and foci of cavitation. Further, the right frontal lobe lesion which showed diffusion restriction in the initial scan was showing increased ADC on follow‑up imaging. The reason for the occurrence of transient cytotoxic edema with subsequent resolution into vasogenic edema remains unclear. We speculate that active demyelination
is followed by astrogliosis and neuronal loss which may be responsible for the increased ADC. Further studies evaluating the serial DW images in cases having a fulminant SSPE with histopathological correlation are required for a better understanding of the progression of imaging findings. To conclude, we have reported a case of fulminant SSPE with atypical MRI features of extensive diffusion restriction.
Dhaval Shah, K. Srinivasan, Tejas Sakale1, S. Sajith1, C. Kesavadas Departments of Imaging Sciences and Interventional Radiology and 1Neurology, Sree Chitra Thirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India E-mail: [email protected]
References 1. 2.
3. 4.
Graves MC. Subacute Sclerosing panencephalitis. Symposium on neurovirology. Neurol Clin 1984; 2:267‑280. Jabbour JT, Garcia JH, Lemmi H, Ragaland J, Duenas DA, Sever JL. Subacute Sclerosing panencephalitis. A multidisciplinary study of eight cases. JAMA 1969;207:2248-54. Gutierrez J, Issacson RS, Koppel BS. Subacute sclerosing panencephalitis: An update. Dev Med Child Neurol 2010;52(10):901‑7. Sener RN. Subacute sclerosing panencephalitis findings at MR imaging, diffusion MR imaging and proton MR spectroscopy. AJNR Am J
Neurology India / May 2015 / Volume 63 / Issue 3
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Neuroradiol 2004;25:892-4. Garg RK. Subacute sclerosing panencephalitis. Postgrad Med J 2002;78:63-70. 6. Oguz KK, Celebi A, Anlar B. MR imaging, diffusion‑weighted imaging and MR spectroscopy findings in acute rapidly progressive subacute sclerosing panencephalitis. Brain Dev 2007; 29 (5):306‑11. 7. Alkan A, Korkmaz L, Sigirci A, Kutlu R, Yakinci C, Erdem G, et al. Subacute sclerosing panencephalitis: Relationship between clinical stage and diffusion‑weighted imaging findings. J Magn Reson Imaging 2006; 23(3):267‑72. 8. Manayani DJ, Abraham M, Gnanamuthu C, Solomon T, Alexander M, Sridharan G. SSPE‑the continuing challenge: A study based on serological evidence from a tertiary care centre in India. Indian J Med Microbiol 2002;20(1):16‑8. 9. Kanemura H, Aihara M. Serial diffusion‑weighted imaging in subacute sclerosing panencephalitis. Pediatr Neurol 2008;38(6):430‑4. 10. Mahadevan A, Vaidya SR, Wairagkar NS, Khedekar D, Kovoor JM, Santosh V, et al. Case of fulminant‑SSPE associated with measles genotype D7 from India: An autopsy study. Neuropathology 2008;28(6):621‑6. 5.
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www.neurologyindia.com DOI: 10.4103/0028-3886.158268 PMID: xxxxx
Bilateral isolated basal ganglia bleed: An atypical presentation of Japanese encephalitis Sir, Japanese encephalitis (JE), a mosquito‑borne flaviviral encephalitis, remains a major health problem in the Indian subcontinent.[1] Though no age is immune, children between 5 and 15 years of age happen to bear the brunt of the disease. The typical cranial imaging abnormalities in JE involve bilateral thalami, basal ganglia, substantia nigra, brainstem, cerebellum and cerebral cortical and white matter.[2,3] Although haemorrhagic lesions involving bilateral thalami have been described previously, such lesions are usually accompanied by similar lesions elsewhere in the brain. Bilateral isolated basal ganglia bleed as the sole imaging abnormality in JE has not been reported before. Here, we report the case of a child with the diagnosis of JE wherein the cranial imaging revealed bilateral isolated basal ganglia bleed. 456
A 5‑year‑old boy born out of a non‑consanguineous parentage and having a normal perinatal and developmental history presented with a history of fever of 5 days duration and 4 episodes of generalised tonic‑clonic seizures in 1 day. There was no regaining of consciousness in between the episodes of seizures suggesting the presence of status epilepticus. The fever was associated with headache and vomiting although no history of loose stools, cold or cough could be elicited. A diagnosis of febrile status epilepticus was made at this point. On examination, the child was febrile (100.2° F). His pulse rate was 130/min, and he had a blood pressure of 98/72 mmHg and a respiratory rate of 28/min. He was stuporous with no eye opening and minimal limb movements to painful stimulus. His fundus examination was normal. His pupils were equal but sluggishly reacting to light. The doll’s eye response was preserved. The motor examination showed an increased muscle tone in all 4 limbs with brisk deep tendon reflexes and an extensor plantar response bilaterally. There was terminal neck stiffness with a positive Kernig’s sign. Complete hemogram showed leucocytosis (16,000 cells/cumm) with a normal platelet count. Renal and hepatic functions including serum electrolytes were normal. Computed tomography (CT) of the brain showed bleed in the right basal ganglia. Magnetic resonance imaging (MRI) showed blooming in bilateral basal ganglia on gradient echo (GRE) sequence suggestive of bleed [Figure 1]. Cerebrospinal fluid (CSF) examination showed mildly raised protein (62 mg/dl), normal glucose with mild lymphocytic pleocytosis (28 cells per cumm) and positive IgM anti‑JE antibodies. CSF polymerase chain reaction (PCR) analysis for cytomegalovirus, herpes simplex virus and Mycobacterium tuberculosis were negative. Serological testing for human immunodeficiency virus was negative. The child was started on antiepileptics and put on mechanical ventilator. The seizures were eventually controlled on two antiepileptic drugs. He was later discharged in an akinetic‑mute state. Prognosis in patients with JE is often poor with occurrence of mortality in about one third of the patients; and, half of the patients who survive are often left with severe
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Figure 1: CT brain showing bleed in the right basal ganglia (a); MRI brain GRE sequence shows blooming in bilateral basal ganglia on gradient echo imaging (b and c; white arrow)
Neurology India / May 2015 / Volume 63 / Issue 3
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Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
