Letters to the Editor
Author’s response on “Neuropsychiatric profiles in patients with Alzheimer’s disease and vascular dementia” Sir, We sincerely thank the reader(s) for his/her/their interest in our article entitled “Neuropsychiatric profiles in patients with Alzheimer’s disease and vascular dementia” published in Ann Indian Acad Neurol (2015;18:120-21).[1] As written in the article, we have used the mini-mental status examination (MMSE) for initial screening of our patients. The usual protocol we follow at our clinic is to apply the MMSE at the beginning and get the score. It gives us an estimate about the domains involved in a given patient. The qualitative assessment of MMSE (i.e., how a patient is performing while applying MMSE) tells more about the impairment the patient is having than the crude score itself. Similar to CDR (Clinical Dementia Rating) score, MMSE also used to determine the stage of dementia. Therefore, in our study, MMSE was not meant to select or reject patients but it was part of the whole assessment. We applied this initial test before embarking upon the full assessment. The second concern of the reader(s) was why we did not use the Bengali version of MMSE in our subjects. We did not use a vernacular version because the available Hindi and Bengali versions of MMSE have limitations. The Hindi adaptation of the MMSE was developed by Ganguli et al. while developing a screening tool for rural illiterate Indian elderly subjects.[2] Thus, it was modified to enable an illiterate subject to complete the test without difficulty. The Bengali version of MMSE was an adaptation of the Hindi version.[3] While these are useful for illiterate subjects, both the batteries are too easy for an urban educated subject. The ‘ceiling effect’ is observed while generating the cut-off scores for educated subjects.[2,3] This means the 10th percentile score of normative data for educated subject touches the highest possible score. Thus, early impairment is missed if these instruments are used for educated subjects. Hence, both Hindi and Bengali versions of MMSE are not useful for testing an educated subject. Conversely, MMSE is an accepted and widely used global cognitive screening scale used across the world. It is comprehensive, easy to apply, and useful for comparison. We therefore used MMSE for testing educated subjects in our clinic.
Tushar Kanti Bandyopadhyay, Atanu Biswas, Arijit Roy, Deb Sankar Guin, Goutam Gangopadhyay, Sujit Sarkhel1, Malay Kumar Ghoshal2, Asit Kumar Senapati Department of Neurology, Bangur Institute of Neurosciences, Kolkata, 1Department of Psychiatry, Institute of PostGraduate Medical Education and Research, Kolkata, 2 Department of Psychiatry, Calcutta Medical College, Kolkata, West Bengal, India For correspondence: Dr. Atanu Biswas, Department of Neurology, Bangur
Institute of Neurosciences, 52/1A, S.N. Pandit Street, Kolkata - 700 025, West Bengal, India. E-mail: [email protected]
References 1. 2.
3.
Raina SK, Neuropsychiatric profiles in patients with Alzheimer’s disease and vascular dementia. Ann Indian Acad Neurol 2015;18:120-21. Ganguli M, Ratcliff G, Chandra V, Sharma S, Gilby J, Pandav R, et al. A Hindi version of the MMSE: The development of a cognitive screening instrument for a largely illiterate rural elderly population in India. Int J Geriatr Psychiatry 1995;10:367-77. Das SK, Banerjee TK, Mukherjee CS, Bose P, Biswas A, Hazra A, et al. An urban community based study of cognitive functions among non-demented elderly population in India. Neurol Asia 2006;11:37-48. Access this article online Quick Response Code:
Website: www.annalsofian.org
DOI: 10.4103/0972-2327.156565
Refractory status epilepticus from NMDA receptor encephalitis successfully treated with an adjunctive ketogenic diet Sir, N-methyl D-aspartate receptor encephalitis (NMDARE) is characterized by the presence of NMDAR antibodies in the Annals of Indian Academy of Neurology, April-June 2015, Vol 18, Issue 2
CSF, and it can progress to psychosis and refractory status epilepticus (RSE). We report a case of the use of a ketogenic diet (KD) as an adjunct in RSE from NMDARE.
Letters to the Editor
A healthy 21-year-old female presented to the ED with symptoms that progressed from depression, headaches, and nausea to word-finding difficulties, psychosis, and seizures. She developed SE and was intubated. A comprehensive evaluation was unremarkable, and she was transferred to our institution on hospital day 11 where an electro-encephalogram (EEG) revealed RSE. The NMDAR antibody was detected in her CSF. A thorough investigation was negative for any tumor. Despite corticosteroids, plasmapheresis, IV Ig in addition to 5 anticonvulsant medications and propofol, continuous EEG showed RSE. Ketosis was achieved after a KD (ratio of fat: Combined carbohydrate and protein of 4:1) was started (day 21). Her continuous EEG showed a significant decrease in her seizures within 2 weeks (day 35). She was weaned off mechanical ventilation and discharged to a long-term care facility on 3 anticonvulsant medications, mycophenolate mofetil, and on a KD. Most patients with NMDARE develop a multistage illness that progresses to psychosis, memory deficits, RSE, and into a state of unresponsiveness with catatonic features.[1] Management of NMDARE should initially focus on immunotherapy and the detection and removal of the tumor.[2,3] Most patients receive corticosteroids, PLEX, or IV Ig as first-line immunotherapy. Additional treatment with second-line cyclophosphamide or rituximab is sometimes needed. Our patient continued to deteriorate despite these therapies. A KD, by reducing the firing of central neurons by opening K ATP channels[4], is a very effective adjunct to antiepileptics in patients with refractory epilepsy. Side effects of KD[5] are usually mild, predictable, and preventable, and rarely lead to diet discontinuation. Important ones include constipation, acidosis, dyslipidemia, kidney stones, and bone fractures. To our knowledge, this is the first case report of initiating a KD as an adjunct in RSE from NMDARE.
257
Syed Amer, Parth Shah1, Vishnu Kommineni1 Department of Neurology, 1Medicine, Mayo Clinic, Phoenix, Arizona, USA For correspondence: Dr. Syed Amer, Mayo Clinic, 5777 East Mayo Boulevard,
Phoenix, Arizona - 85054, USA E-mail: [email protected]
References 1. 2. 3. 4. 5.
Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011;10:63-74. Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, et al. Anti-NMDA-receptor encephalitis: Case series and analysis of the effects of antibodies. Lancet Neurol 2008;7:1091-8. Niehusmann P, Dalmau J, Rudlowski C, Vincent A, Elger CE, Rossi JE, et al. Diagnostic value of N-methyl-D-aspartate receptor antibodies in women with new-onset epilepsy. Arch Neurol 2009;66:458-64. Ma W, Berg J, Yellen G. Ketogenic diet metabolites reduce firing in central neurons by opening K (ATP) channels. J Neurosci 2007;27:3618-25. Kang HC, Chung DE, Kim DW, Kim HD. Early-and late-onset complications of the ketogenic diet for intractable epilepsy. Epilepsia 2004;45:1116-23. Access this article online Quick Response Code:
Website: www.annalsofian.org
DOI: 10.4103/0972-2327.150620
Early detection of adult onset Susac’s syndrome in a South Indian female Sir, Since the first description of Susac’s Syndrome (SS) in 1979, about 304 cases of SS have been reported,[1] mainly in Western countries. To the best of our knowledge, it is a case of adult onset of SS diagnosed at early stage reported first time from south India and it is the second case reported from India. A 26-year-old woman presented with headache of one year. It was insidious onset, gradually progressive in nature.. She had visual disturbances for 2 months and decreased left-sided hearing since 1 month. Blurring of vision was mainly involving the left eye in nasal fields. She felt continuous tinnitus in left ear with decreased hearing. She had no features of increased intracranial pressure (ICP). Her electroencephalogram (EEG) was within normal limit. Her blood investigation for vasculitis
work up was negative. Magnetic resonance imaging (MRI) brain showed T1W hypointense lesions in body of corpus callosum [Figure 1a]. There was no contrast enhancement on post-contrast sagittal T1-weighted (T1W) image [Figure 1d]. Color fundus photography of her right eye [Figure 2a] shows multiple greyish white puffy patches, secondary to retinal artery branch occlusions. Late phase of fluorescein angiogram of right eye revealed blocked choroidal fluorescence in all the three corresponding areas [Figure 2b]. Right eye inferior temporal quadrant late phase angiography, shows [Figure 2c] retinal artery branch occlusion and the left eye posterior pole late phase [Figure 2d] showed blocked choroidal fluorescence due to retinal thickening. Pure tone audiogram (PTA) showed mild sensory neural deafness. Visually evoked potential (VEP) study result is suggestive of demyelinating type of visual pathway defect on the left side. The combination of patient’s Annals of Indian Academy of Neurology, April-June 2015, Vol 18, Issue 2
Copyright of Annals of Indian Academy of Neurology 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.
Author’s response on “Neuropsychiatric profiles in patients with Alzheimer’s disease and vascular dementia” Sir, We sincerely thank the reader(s) for his/her/their interest in our article entitled “Neuropsychiatric profiles in patients with Alzheimer’s disease and vascular dementia” published in Ann Indian Acad Neurol (2015;18:120-21).[1] As written in the article, we have used the mini-mental status examination (MMSE) for initial screening of our patients. The usual protocol we follow at our clinic is to apply the MMSE at the beginning and get the score. It gives us an estimate about the domains involved in a given patient. The qualitative assessment of MMSE (i.e., how a patient is performing while applying MMSE) tells more about the impairment the patient is having than the crude score itself. Similar to CDR (Clinical Dementia Rating) score, MMSE also used to determine the stage of dementia. Therefore, in our study, MMSE was not meant to select or reject patients but it was part of the whole assessment. We applied this initial test before embarking upon the full assessment. The second concern of the reader(s) was why we did not use the Bengali version of MMSE in our subjects. We did not use a vernacular version because the available Hindi and Bengali versions of MMSE have limitations. The Hindi adaptation of the MMSE was developed by Ganguli et al. while developing a screening tool for rural illiterate Indian elderly subjects.[2] Thus, it was modified to enable an illiterate subject to complete the test without difficulty. The Bengali version of MMSE was an adaptation of the Hindi version.[3] While these are useful for illiterate subjects, both the batteries are too easy for an urban educated subject. The ‘ceiling effect’ is observed while generating the cut-off scores for educated subjects.[2,3] This means the 10th percentile score of normative data for educated subject touches the highest possible score. Thus, early impairment is missed if these instruments are used for educated subjects. Hence, both Hindi and Bengali versions of MMSE are not useful for testing an educated subject. Conversely, MMSE is an accepted and widely used global cognitive screening scale used across the world. It is comprehensive, easy to apply, and useful for comparison. We therefore used MMSE for testing educated subjects in our clinic.
Tushar Kanti Bandyopadhyay, Atanu Biswas, Arijit Roy, Deb Sankar Guin, Goutam Gangopadhyay, Sujit Sarkhel1, Malay Kumar Ghoshal2, Asit Kumar Senapati Department of Neurology, Bangur Institute of Neurosciences, Kolkata, 1Department of Psychiatry, Institute of PostGraduate Medical Education and Research, Kolkata, 2 Department of Psychiatry, Calcutta Medical College, Kolkata, West Bengal, India For correspondence: Dr. Atanu Biswas, Department of Neurology, Bangur
Institute of Neurosciences, 52/1A, S.N. Pandit Street, Kolkata - 700 025, West Bengal, India. E-mail: [email protected]
References 1. 2.
3.
Raina SK, Neuropsychiatric profiles in patients with Alzheimer’s disease and vascular dementia. Ann Indian Acad Neurol 2015;18:120-21. Ganguli M, Ratcliff G, Chandra V, Sharma S, Gilby J, Pandav R, et al. A Hindi version of the MMSE: The development of a cognitive screening instrument for a largely illiterate rural elderly population in India. Int J Geriatr Psychiatry 1995;10:367-77. Das SK, Banerjee TK, Mukherjee CS, Bose P, Biswas A, Hazra A, et al. An urban community based study of cognitive functions among non-demented elderly population in India. Neurol Asia 2006;11:37-48. Access this article online Quick Response Code:
Website: www.annalsofian.org
DOI: 10.4103/0972-2327.156565
Refractory status epilepticus from NMDA receptor encephalitis successfully treated with an adjunctive ketogenic diet Sir, N-methyl D-aspartate receptor encephalitis (NMDARE) is characterized by the presence of NMDAR antibodies in the Annals of Indian Academy of Neurology, April-June 2015, Vol 18, Issue 2
CSF, and it can progress to psychosis and refractory status epilepticus (RSE). We report a case of the use of a ketogenic diet (KD) as an adjunct in RSE from NMDARE.
Letters to the Editor
A healthy 21-year-old female presented to the ED with symptoms that progressed from depression, headaches, and nausea to word-finding difficulties, psychosis, and seizures. She developed SE and was intubated. A comprehensive evaluation was unremarkable, and she was transferred to our institution on hospital day 11 where an electro-encephalogram (EEG) revealed RSE. The NMDAR antibody was detected in her CSF. A thorough investigation was negative for any tumor. Despite corticosteroids, plasmapheresis, IV Ig in addition to 5 anticonvulsant medications and propofol, continuous EEG showed RSE. Ketosis was achieved after a KD (ratio of fat: Combined carbohydrate and protein of 4:1) was started (day 21). Her continuous EEG showed a significant decrease in her seizures within 2 weeks (day 35). She was weaned off mechanical ventilation and discharged to a long-term care facility on 3 anticonvulsant medications, mycophenolate mofetil, and on a KD. Most patients with NMDARE develop a multistage illness that progresses to psychosis, memory deficits, RSE, and into a state of unresponsiveness with catatonic features.[1] Management of NMDARE should initially focus on immunotherapy and the detection and removal of the tumor.[2,3] Most patients receive corticosteroids, PLEX, or IV Ig as first-line immunotherapy. Additional treatment with second-line cyclophosphamide or rituximab is sometimes needed. Our patient continued to deteriorate despite these therapies. A KD, by reducing the firing of central neurons by opening K ATP channels[4], is a very effective adjunct to antiepileptics in patients with refractory epilepsy. Side effects of KD[5] are usually mild, predictable, and preventable, and rarely lead to diet discontinuation. Important ones include constipation, acidosis, dyslipidemia, kidney stones, and bone fractures. To our knowledge, this is the first case report of initiating a KD as an adjunct in RSE from NMDARE.
257
Syed Amer, Parth Shah1, Vishnu Kommineni1 Department of Neurology, 1Medicine, Mayo Clinic, Phoenix, Arizona, USA For correspondence: Dr. Syed Amer, Mayo Clinic, 5777 East Mayo Boulevard,
Phoenix, Arizona - 85054, USA E-mail: [email protected]
References 1. 2. 3. 4. 5.
Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R. Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 2011;10:63-74. Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, et al. Anti-NMDA-receptor encephalitis: Case series and analysis of the effects of antibodies. Lancet Neurol 2008;7:1091-8. Niehusmann P, Dalmau J, Rudlowski C, Vincent A, Elger CE, Rossi JE, et al. Diagnostic value of N-methyl-D-aspartate receptor antibodies in women with new-onset epilepsy. Arch Neurol 2009;66:458-64. Ma W, Berg J, Yellen G. Ketogenic diet metabolites reduce firing in central neurons by opening K (ATP) channels. J Neurosci 2007;27:3618-25. Kang HC, Chung DE, Kim DW, Kim HD. Early-and late-onset complications of the ketogenic diet for intractable epilepsy. Epilepsia 2004;45:1116-23. Access this article online Quick Response Code:
Website: www.annalsofian.org
DOI: 10.4103/0972-2327.150620
Early detection of adult onset Susac’s syndrome in a South Indian female Sir, Since the first description of Susac’s Syndrome (SS) in 1979, about 304 cases of SS have been reported,[1] mainly in Western countries. To the best of our knowledge, it is a case of adult onset of SS diagnosed at early stage reported first time from south India and it is the second case reported from India. A 26-year-old woman presented with headache of one year. It was insidious onset, gradually progressive in nature.. She had visual disturbances for 2 months and decreased left-sided hearing since 1 month. Blurring of vision was mainly involving the left eye in nasal fields. She felt continuous tinnitus in left ear with decreased hearing. She had no features of increased intracranial pressure (ICP). Her electroencephalogram (EEG) was within normal limit. Her blood investigation for vasculitis
work up was negative. Magnetic resonance imaging (MRI) brain showed T1W hypointense lesions in body of corpus callosum [Figure 1a]. There was no contrast enhancement on post-contrast sagittal T1-weighted (T1W) image [Figure 1d]. Color fundus photography of her right eye [Figure 2a] shows multiple greyish white puffy patches, secondary to retinal artery branch occlusions. Late phase of fluorescein angiogram of right eye revealed blocked choroidal fluorescence in all the three corresponding areas [Figure 2b]. Right eye inferior temporal quadrant late phase angiography, shows [Figure 2c] retinal artery branch occlusion and the left eye posterior pole late phase [Figure 2d] showed blocked choroidal fluorescence due to retinal thickening. Pure tone audiogram (PTA) showed mild sensory neural deafness. Visually evoked potential (VEP) study result is suggestive of demyelinating type of visual pathway defect on the left side. The combination of patient’s Annals of Indian Academy of Neurology, April-June 2015, Vol 18, Issue 2
Copyright of Annals of Indian Academy of Neurology 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.
