Year : 2022 | Volume
: 13 | Issue : 3 | Page : 154--160
Clinical profile of primary central nervous system demyelinating disorders: A tertiary care hospital-based study in Guwahati
Manisha Manisha, Anirban Mahanta, Munindra Goswami, Marami Das
Department of Neurology, Gauhati Medical College, Guwahati, Assam, India
Dr. Anirban Mahanta
Department of Neurology, Gauhati Medical College, Guwahati - 781 032, Assam
Background: The primary central nervous system (CNS) demyelinating disorders are witnessing significant advancement in terms of treatment options and the diagnostics. However, a resource poor country like ours has to rely more on our clinical findings. Aims and Objectives: To study the spectrum of different clinical manifestations in patients of various primary CNS demyelinating disorders in the hospital setting; categorizing them to the most possible extent into its various types viz. multiple sclerosis(MS), neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis(ADEM), idiopathic optic neuritis(ON). Metarials and Methods: An observational study was conducted for 2 years including old and newly diagnosed cases in whom detailed clinical assessment was done for every attack including the previous and subsequent ones. Statistical analysis was done using SPSS version 21. Results: Of the 47 patients included, 26 (55.3%) were cases of NMOSD, 12 (25.5%) of MS, 4 (8.5%) of ADEM and 5 (10.6%) were isolated cases of ON. 30 were female and 17 were male (ratio 1:0.6). The total mean age for primary CNS demyelinating disorders at presentation was 27.09 ± 13.44 years. Maximum patients fell in the age range of 11–20 years. Among the clinical manifestations, motor abnormalities (97.6%), sensory abnormalities (69%), bladder dysfunction (59.5%), visual manifestations (54.8%) (Unilateral in all MS patients, both bilateral and unilateral in NMOSD) were the most common presentations. In MS, the most common manifestations were motor dysfunction followed by sensory symptoms, optic neuropathy (mostly unilateral), and bladder dysfunction. In NMOSD, the most common clinical manifestations were motor dysfunction, followed by bladder dysfunction, optic neuropathy and then sensory abnormalities. In ADEM, most common manifestations were motor abnormalities, acute encephalopathy, headache, optic neuropathy. Among idiopathic ON, most patients presented with painful diminution of vision (80%). Mean number of attacks was 2.53. Conclusion: A wide spectrum of clinical presentations in various disorders of primary CNS demyelination was found with maximum patients presenting with motor dysfunction in various forms.
|How to cite this article:|
Manisha M, Mahanta A, Goswami M, Das M. Clinical profile of primary central nervous system demyelinating disorders: A tertiary care hospital-based study in Guwahati.Indian J Med Spec 2022;13:154-160
|How to cite this URL:|
Manisha M, Mahanta A, Goswami M, Das M. Clinical profile of primary central nervous system demyelinating disorders: A tertiary care hospital-based study in Guwahati. Indian J Med Spec [serial online] 2022 [cited 2023 Feb 1 ];13:154-160
Available from: http://www.ijms.in/text.asp?2022/13/3/154/350917
Disorders that cause demyelination of nervous system can affect the peripheral nervous system as well as central nervous system (CNS). It can be primary (no known cause) or secondary (with a well-established cause such as autoimmune, infectious, toxic and metabolic, and vascular). Primary demyelinating disorders of the CNS mainly include multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD). Some literatures also include Idiopathic optic neuritis (ON), acute disseminated encephalomyelitis (ADEM), and acute hemorrhagic leukoencephalitis under this category., The past few decades have witnessed a growing interest among neurologists worldwide about primary CNS demyelinating disorders mostly because of availability of better treatment options. Although many advancement has been made in the field of diagnostics such as magnetic resonance imaging (MRI), cerebrospinal fluid OCB, anti-AQP4 antibody, anti-MOG antibody, in a resource-deficient country like ours, we have to rely more on our clinical findings before sending the patient for expensive radiological and laboratory evaluation. Also record keeping and long-term follow-up of patients have serious limitations, hindering data collection and analysis. Hence, there is a dire need to understand the spectrum of distribution of various clinical manifestations amongst the primary CNS demyelinating disorders.
There is a paucity of large-scale studies in our country that comprehensively include all categories of primary demyelinating disorders of CNS, although there are regional studies taking individual diagnosis into consideration. Contemplating this fact, our study was conducted. Our objective was to study the spectrum of different clinical manifestations in patients of various primary CNS demyelinating disorders in the hospital setting; categorizing them to the most possible extent into various types of primary CNS demyelinating disorders namely MS, NMOSD, ADEM, idiopathic ON.
MS is approximately threefold more common in women than men. The age of onset is typically between 20 and 40 years. Relapsing or bout onset MS accounts for about 90% of MS.
It is now recognized that NMOSD is distinct from MS. Individuals of Asian and African origin are disproportionately affected. Among white populations, MS is far more common than NMO. ON is a common inflammatory disease of the optic nerve.
ADEM usually has a monophasic course and is most frequently associated with an antecedent infection (post-infectious encephalomyelitis); approximately 5% of ADEM cases follow immunization (post-vaccine encephalomyelitis). ADEM is far more common in children than adults.
Materials and Methods
Approval was taken from the Institutional Ethical Committee, Gauhati Medical College and Hospital for this observational study which was carried on for 2 years.
New cases presenting with features suggestive of CNS demyelinating disorders after ruling out secondary causes such as infection, other systemic inflammatory/autoimmune disorder, toxin, nutritional causes, hypoxia, and ischemiaAlready diagnosed cases of primary demyelinating disorders presenting with new onset of neurological deficitBoth gendersUp to 60 years of age.
Cases presenting with features of demyelinating disorder of CNS due to secondary causesDiagnosed cases of primary CNS demyelinating disorder not presenting with new-onset neurological deficitMore than 60 years of age.
Method of data collection
Patients were chosen from the Department of Neurology, Medicine, and Pediatrics wards after taking informed consent. A detailed history was taken from each patient about the current illness, previous attacks wherever present, and on subsequent attacks on follow-up. Thorough clinical examination was done on each patient; the clinical findings as well as investigational findings were also noted on the proforma. For diagnosis, appropriate diagnostic criteria were applied namely the 2017 McDonald criteria for diagnosis of MS; the International consensus diagnostic criteria for NMOSD (2015); diagnostic criteria for ADEM by the Paediatric MS study group (2012).
Chi-square test was used to evaluate association between categorical variables. Data were checked for normality using Kolmogorov–Smirnov, and Shapiro–Wilk test. Independent t-test and ANOVA is used depending on fulfillment of normality assumption for continuous variables. For non normal data, Mann–Whitney U test and Krushkal–Wallis test were used.
All data were analyzed using SPSS version 21 (IBM Corp. Released 2012. IBM SPSS Statistics for Windows, Version 21.0. Armonk, NY: IBM Corp.). A P < 0.05 is considered as statistically significant at 5% level of significance.
Results and Observations
Total number of patients with primary CNS demyelinating disorders included in the study was 47. Among them 26 were cases of NMOSD, 12 of MS, 4 of ADEM, and 5 were isolated cases of ON.
Thirty were females and 17 were males. Overall female: male ratio was approximately 1:0.6. On studying the sex distribution in individual diagnostic category, it is shown that female: male ratio is 1:0.3 in ADEM, 1:1.4 in MS, approximately and 1:0.25 in NMOSD, 1:4 in ON. This gender-wise distribution pattern among individual diagnostic category of primary central demyelinating diseases is statistically significant with P = 0.017.
The maximum number of patients fell in the age range of 11–20 years, i.e., 13 cases (27.7%), minimum number of patients fell in age range of 51–60 years, i.e., 2 cases (4.3%). The total mean age for primary CNS demyelinating disorders at presentation was 27.09 ± 13.44 years.
The mean ages at presentation for patients with ADEM, MS, NMOSD, and ON were 12.5 ± 4.8 years, 29 ± 10.6 years, 26.42 ± 11.96, and 37.6 ± 22.15 years, respectively. The P value here was 0.039 which shows statistically significant difference.
The headache was present in 2 (50%) in ADEM; 2 (7.7%) in NMOSD, while it was absent in MS. The P value (0.011) shows statistical significance.
Acute cognitive impairment at presentation was seen in 2 (50%) cases of ADEM. It wasn't found in any case of MS and NMOSD. The P value was 0.001, i.e., statistical significance exists.
The number of cases who presented with visual abnormalities were 1 (25%) among ADEM; 7 (57.7%) among MS; 15 (54.8%) among NMOSD. The P value was 0.453, i.e., no statistical significant difference exists.
Unilateral visual symptoms were found in 7 (58.3%) cases of MS, 5 (19.2%) cases of NMOSD, 3 (60%) cases of ON. Bilateral visual symptoms were present in 1 (25%) case of ADEM; 10 (38.5%) cases of NMOSD, 2 (40%) cases of ON. The P value (0.026) denotes statistical significance.
The weakness/motor symptoms at presentation were there in 4 (100%) cases of ADEM; 12 (100%) cases of MS, 25 (96.2%) cases of NMOSD. No statistically significant differences were found for weakness/motor abnormalities (P = 0.73) among our case distribution. However, hemiparesis was the finding in 2 (50%) cases of ADEM, 5 (41.7%) of MS, and not seen among NMOSD and the P value calculated (P < 0.001) shows there is statistically significant difference. Quadriparesis was found in 1 (25%) of case amongst ADEM, 5 (41.7%) of MS, 18 (69.2%) of NMOSD, but the P value calculated (P = 0.017) shows there is statistically significant difference. Paraparesis was a presenting complaint in 4 (33.3%) of MS, 10 (38.5%) of NMOSD, but the P value calculated (P = 0.185) shows that there is no statistically significant difference. Monoparesis was the finding in 1 (25%) case of ADEM, 2 (16.7%) of MS, 1 (3.8%) of NMOSD, the P value calculated (P = 0.306) shows no statistically significant difference.
The speech was impaired in 1 (25%) case of ADEM, 1 (8.3%) of MS, 1 (3.8%) of NMOSD, the P value calculated (P = 0.305) shows no statistically significant difference.
Dysphagia or impairment of swallowing or nasal regurgitation were 1 (25%) among ADEM, 2 (7.7%) among NMOSD. The P value is 0.24, i.e., there is no statistically significant difference.
The number of cases presented with back pain were 5 (19.2%) among NMOSD, with no case complained of back pain in ADEM or MS. The P value is 0.174 i.e., no statistical significance.
The number of cases who had bladder/bowel dysfunction at presentation were 4 (33.3%) in MS; 21 (80.8%) in NMOSD. There is a statistically significant difference amongst the presentation (P = 0.001).
The gait ataxia was present in 1 (25%) case of ADEM; 3 (25%) cases of MS. No cases found out with gait ataxia amongst NMOSD. The P value (P = 0.028) shows there is a statistical significant difference for this condition.
The number of cases with sensory symptoms at presentation were 1 (25%) among ADEM; 10 (83.3%) among MS; 18 (69.2%) among NMOSD. The P value is 0.092 which denotes no statistical significance.
The pruritus/itching as presenting complaint was present in 2 cases of NMOSD. The P value is 0.524 which is statistically insignificant.
Nausea/vomiting is present in 2 (50%) cases of ADEM; 8 (30.8%) in NMOSD, while it is absent in MS; The P value is 0.051 which do not show any statistically significant difference. Hiccups at presentation in 4 (15.4%) cases of NMOSD; no cases of MS/ADEM complained of hiccups. The P value is 0.257 which shows no statistical significance.
So the most common clinical manifestation was weakness/motor symptoms present in all 3 groups i.e., 100% cases of MS, ADEM; and 96.2% cases of NMOSD. The clinical manifestation next in line in ADEM was headache, nausea/vomiting, acute cognitive decline-all 50%. In MS next most common clinical manifestation was sensory symptoms (83.3%); and in NMOSD it was the bowel/bladder dysfunction (80.8%).
Out of 5 isolated cases of ON, 4 cases were painful. Recurrence was found in 2 cases. 2 cases had bilateral presentation and rest 3 had unilateral presentation.
History of fever few weeks prior to the onset of neurological manifestation was present in 2 (50%) patients of ADEM and 1 (3.8%) case of NMOSD (P = 0.003 which was significant).
Based on clinical presentation, doing thorough clinical examination, biochemical; electrophysiological; radiological support, all the findings among the cases of ADEM, MS, NMOSD have been placed under various subcategories which are as follows:
The ON was present in 2 ADEM cases (50%) out of 4; 8 (66.7%) cases of MS of 12; 19 (73.1%) cases of NMOSD out of 26. The P value is 0.365 which doesn't show statistical significance of above comparison.
The acute tranverse myelitis (ATM) was present in 1 (25%) case of ADEM; 2 (16.7%) cases of MS/longitudinally extensive transverse myelitis (LETM), 25 (96.2%) cases of NMOSD. The P value is < 0.001 (statistically significant).
The number of cases presenting as acute partial transverse myelitis (APTM) were 8 (66.7%) in MS; none in ADEM, NMOSD. The P < 0.001 which means there is statistically significant difference.
The features of cerebellar dysfunction were present in 1 (25%) case of ADEM; 3 (25%) cases of MS; none in NMOSD. The P value is 0.028 which means there is statistically significant difference.
The acute encephalopathy is present in 2 (50%) cases of ADEM. No encephalopathy found in MS or NMOSD cases.
The features of brainstem syndrome at presentation were found in 7 (16.7%) cases of NMOSD and were absent in ADEM/MS. The P value is 0.075 which doesn't show statistical significance.
The features of cranial neuropathy (other than the optic nerve) were present in 3 (25%) cases of MS and weren't found in ADEM and NMOSD. The P value is 0.018 which shows statistical significance.
Amongst all CNS demyelinating diseases commonest subcategory at presentation was of ON (34 [72.3%], followed by LETM/ATM [n = 28 (59.6%)]. APTM [8 (17%)], Brainstem syndrome [7 (14.9%)] was also a common presenting complaint but in lesser frequency.
Distribution of attacks or relapses among acute disseminated encephalomyelitis, multiple sclerosis, neuromyelitis optica spectrum disorder, idiopathic optic neuritis
The mean number of attacks in each category i.e., ADEM, MS, NMOSD, ON were respectively 1.25, 2.83, 2.81, 1.4. The overall mean attack in our study was 2.53. P value is 0.039 which means the difference of mean attacks in all 4 diagnostic categories is statistically significant.
In this study with duration of about 2 years, total 47 patients were included who had primary CNS demyelinating disorders. Of these maximum number of cases were of NMOSD (26 [55.3%]), followed by MS (12 [25.5%]), idiopathic ON (5 [10.6%]) and ADEM (4 [8.5%]) respectively. The study by Nayak et al. conducted in northwest India also found more number of NMSOD cases as compared to other primary CNS demyelinating diseases. But Gorthi et al. found maximum number of patients belonging to the diagnostic category of MS followed by NMOSD, then ON and then ADEM.
The female: male ratio separately in ADEM, MS, NMOSD, Idiopathic ON respectively were 1:0.3, 1:1.4, 1:0.25, 1:4 (P = 0.017). The overall female preponderance in our study is a usual and consistent finding for the primary demyelinting disorders of CNS. However, this trend wasn't followed in our study in the MS group. Previous Indian studies done by Mathew et al., Chopra et al., Gourie-Devi and Nagaraja showed higher male: female ratio.,,, This skewing was also found in cases of idiopathic ON. Some studies from Nepal have also found male preponderance in ON., The possible factors for this deviation may be cultural and societal belief (reflected in under reporting of female patients), geography, race or ethnicity. Further study is needed to establish the cause.
The maximum number of patients fell in the range of 11–20 years at the time of presentation. The mean age at presentation was 27.09 ± 13.4 years. The mean ages at presentation individually disease-wise are 12.5 ± 4.8 years, 29 ± 10.6 years, 26.42 ± 11.9 years, 37.6 ± 22.1 years respectively in ADEM, MS, NMOSD, idiopathic ON (P = 0.039). The mean age in our study in case of MS and NMOSD are consistent with Indian scenario. Studies too by Barhate et al., Mukherjee et al. had similar mean age., Mean age is higher as compared to other Indian studies in case of idiopathic ON., The mismatch may be due to inclusion of smaller number of patients in our study.
The most common clinical manifestations among the three categories of diagnosis is motor weakness, found in 4 (100%), 12 (100%), 25 (96.2%) cases of ADEM, MS, NMOSD respectively which can be summed up to 97.6% of total patients (42) (except idiopathic/isolated ON). Nayak et al. and Gorthi et al. also found motor manifestations as the most common clinical manifestations. Pratibha et al. found pyramidal manifestations in 80.7% cases of ADEM. The patients in their study were more than ours which can explain the difference. A study conducted by Jayakrishnan et al. found motor manifestations in 71% ADEM patients.
Jena et al. found pyramidal dysfunction (86.6%) as most common manifestation of MS. Syal et al. also found pyramidal manifestation in 87.7%.
Monoparesis (8.5%) was present in 50% patients of ADEM, 8.3% of MS, 3.8% of NMOSD (P = 0.306). The P value here suggested insignificance of this data. The one case of monoparesis of right and left lower limbs alternately in NMOSD was found in a female patient while taking history of previous attacks. We couldn't find any relevant study based on monoparesis in NMOSD to compare our findings, so we attributed it to be a manifestation of cerebral involvement. Hemiparesis (14.9%) was present in 50% of ADEM and 41.7% of MS (P < 0.001). Paraparesis (29.8%) was present in 33.3% of MS and 38.5% cases of NMOSD (P = 0.185). Quadriparesis (51.1%) (Most common) was present in 25% of ADEM, 41.7% of MS, 69.2% of NMOSD (0.017). A study conducted by Jayakrishnan and Krishnakumar found motor manifestations in 71% ADEM patients of which, paraparesis was found in 29% and quadriparesis was found in 43%. Prabhas et al. found motor weakness in 52.7% of which paraparesis was 25% and hemiparesis and quadriparesis were 13.8% each. Ahmed et al. found hemiparesis, paraparesis, monoparesis in 9.5%, 4.8%, 9.5% of ADEM cases repectively.
Jena et al. found pyramidal dysfunction (86.6%) as most common manifestation of MS. They also found 18%, 9%, 42%, 3% cases of monoparesis, hemiparesis, paraparesis and quadriparesis respectively (unlike this study where paraparesis is most common, we found hemiparesis and quadriparesis as the most common motor manifestation in MS).
The subcategory of Myelitis was found in 36 out of 42 patients of which 8 cases were APTM and rest 28 were case of complete myelitis out of which 25 case were LETM and 3 were ATM.
APTM was found in 66.7% cases of MS which is a consistent finding in cases of MS. ATM were found in 16.7% cases of MS. The pyramidal manifestation in MS is more common in Asian population as compared to Caucasians where sensory manifestations are more common and it can be observed from several Indian studies.
Several Indian studies quote myelitis as common manifestation in NMOSD., In our study, LETM was found in 96.2% cases of NMOSD (MRI-confirmed). Nayak et al. found LETM in 44% cases. Nasrin et al. found LETM in 30 out of 36 cases. So our study finding correlated with theirs.
The next common manifestations were the sensory symptoms (in 69% patients out of 42 patients) in the form of numbness, tingling, burning paraesthesias. Nayak et al. also found sensory manifestations as next most common clinical manifestation in his study. These were present in 80.8% cases of MS and 69.2% cases of NMOSD. Gangopadhya et al. got sensory symptoms in 75.5% of his MS cases which is approximating to the value we got in our study. Jena et al. found sensory manifestations in about 68% patient of MS.
Itching below neck was bothersome to 7.7% patients of NMOSD. Neuropathic pruritus in neuromyelitis optica may reflect the typical central cord lesions affecting dorsal horn neurons, the dorsal horn being rich in mediators of pruritus, or lesions surrounding periaqueductal grey matter (both the patients had lesions of brainstem in MRI). Neuropathic pruritus has been reported in Indian studies by Bansal et al. (12.4%), Manjunath et al. (26.3%)., Pruritus has been found to herald weakness or relapse in NMOSD. In our patients too, itching below neck preceded the onset of quadriparesis in two female patients of NMOSD.
Upper motor neuron pattern of bladder dysfunction was presenting feature in 33.3% cases of MS, 80.8% cases of NMOSD, but not at all in ADEM. It constituted 59.5% of the 42 cases. Nayak et al. found bladder dysfunction in 49.4% cases. Jena et al. got bladder manifestations in 66.2%.a value correlating with our study.
Out of total 47 patients of which 5 were idiopathic/isolated ON, clinically visual manifestations were present only in 54.8% of the total 42 patient including MS, NMOSD, ADEM. Nayak et al. found optic nerve involvement in 59.3% cases matching with our study. It was present in our study in 50% cases of ADEM (ON-ADEM), 66.7% of MS (ON-MS), 73.1% cases of NMOSD (ON-NMOSD) (P = 0.453). Clinically, 25% of ADEM presented with complaint of visual diminution, confirmed by clinical examination of optic nerves which had bilateral involvement. History of visual diminution was given by 58.3% of MS, all with unilateral presentation. However, only 41.7% cases had abnormal findings while examining the optic nerves. Syal et al. found optic nerve involvement in 57% of MS cases. ON was manifested in 58.6% of study done by Jena et al. Gangopadhya et al. found optic nerve involvement in 71% cases. Our study parameters in this regard is correlating with other Indian studies.
Diminution of vision was complained in 57.7% cases of NMOSD (38.5% had bilateral with higher likelihood of the diagnosis of NMOSD and 19.2% had unilateral presentation) and 53.8% confirmed optic nerve abnormality on clinical examination. Sharma et al. found optic nerve impairment in 48% cases.
The comparison of laterality of visual symptoms in all three disease category-MS (unilateral), NMOSD (mostly bilateral), ADEM (bilateral) was statistically significant P = 0.026.
The rest cases of the ON who didn't have clinical manifestations, showed optic nerve involvement on VEP or MRI.
Brainstem syndrome consisting of intractable or recurrent vomiting and hiccup was present in 26.9% cases of NMOSD and not at all in cases of ADEM and MS. Sharma et al. had 24.9% cases of NMOSD presenting with the brainstem syndrome (P = 0.075), matching our study. Nayak et al. reported it in 16% of patients.
Gait ataxia along with other cerebellar manifestations was present in 25% cases each of ADEM and MS. Maramattom et al. found gait ataxia in 25% cases of ADEM. Jena et al. found cerebellar involvement in 36.9% cases. It has been found that cerebellar manifestations are less common in Asian population (30%–58%) as compared to Caucasian population (about 80%). This fact is represented in our study too. Nayak et al. reported 21% patients with cerebellar dysfunction matching with our study. Study with more sample size is needed for proper estimation.
Acute encephalopathy was manifested in 50% cases of ADEM who presented with acute cognitive impairment, headache and vomiting (significant P value). Maramattom et al. found altered sensorium in 29% patients. One of the patient in encephalopathy was a young adult. Studies outside India have shown that encephalopathy in adult in case of ADEM is found in 20%–56% cases.,
Cranial neuropathy involving cranial nerves other than the optic nerve were present in 25% cases of MS (significant P value) which presented as diplopia, dysphagia, dysarthria, difficulty in chewing, tongue atrophy and deviation, facial deviation indicating the involvement of oculomotor, abducens, facial, glossopharyngeal, vagus, trigeminal, hypoglossal, facial nerves. Cranial neuropathies with involvement other than that of optic nerve is rare, but a study in Japan showed cranial neuropathies in 43% of study population including oculomotor, trochlear, trigeminal, abducens, facial nerves. Although dysphagia, dysarthria (7.7%, 3.8% respectively) were present in NMOSD, they can be attributed to brainstem syndrome.
Back pain could be appreciated by 19.2% cases of NMOSD which was mostly diffuse, dull aching, nonlocalising (funicular). No statistical significance was found. No appropriate Indian studies could be found for comparison. However, a Japanese study found trunk and limb pain in 83% of NMOSD patients against 41% of MS. Central neuropathic pain may be more frequent in NMO than in MS due to the distinct distributions of spinal cord lesions in the two diseases.
Two patients of ADEM had history of fever a few weeks before onset of neurological manifestations which is a usual finding. One patient of NMOSD (anti-MOGantibody positive) also had past history of fever which is also a usual finding.
Among 5 cases of idiopathic ON (who didn't fit in to any of the other category viz. ADEM, MS, NMOSD), 4 (80%) cases presented with painful diminution of vision. This is also consistent with other Asian studies (e.g. Japan) but there are considerably more number of cases in west (ONTT trial). Three cases (60%) had unilateral involvement, while 2 (40%) had bilateral involvement. History of recurrence with past history of visual diminution was present in 2 (40%) patients which correlates with the standard studies., Fundoscopy suggested papillitis in 3 (60%) patients which was comparable to study done by Saxena et al. who found them in 53.5% cases.
Although ADEM is primarily a monophasic illness, rarely it may be multiphasic. In our study, mean number of attacks is 1.25 ± 0.5 as we got one case of multiphasic ADEM who presented twice with ADEM within a span of 3 years. The mean of attacks in MS is 2.83 ± 1.59, in NMOSD is 2.81 ± 1.88, in idiopathic ON it is 1.4 ± 0.55. So, the mean number of attacks was highest in MS, followed by NMOSD; a finding similar to what Nayak et al. found. Overall mean attack was 2.53.
It has been observed that in NMOSD, anti-AQP4 seropositivity is associated with higher relapses a finding supported by other studies.,
We conclude that our study showed a wide spectrum of clinical presentations in various disorders of primary CNS demyelination with maximum patients presenting with motor dysfunction in various forms and MS had highest number of attacks. Major drawback of the study was the inclusion of less number of patients in our study the reason for which could be the intercurrent COVID19 pandemic, initial self remitting nature of illness and poor financial status of the patients. All of these could have led to less number of patients seeking medical attention. Lack of in-hospital facilities of certain laboratory investigations also acted as a hindrance in our study.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
|1||Love S. Demyelinating diseases. Clin Pathol 2006;59:1151-9.|
|2||Berkowitz AL. Chapter 21: Demyelinating diseases of the central. In: Moyer A, Brown RY, editors. Clinical Neurology and Neuroanatomy: A Localization-Based Approach. United States of America: McGraw-Hill Education; 2017.|
|3||Pandit L. Insights into the changing perspectives of multiple sclerosis in India. Autoimmune Dis 2011;2011:937586.|
|4||Cree BA. Multiple sclerosis. In: Larry JP, Dunlop UR, editors. Harrison's Principles of Internal Medicine. 20th ed. United States of America: McGraw-Hill: 2018. p. 3188-202.|
|5||Nayak SD, Sharma CM, Kumawat BL, Khandelwal D, Yadav R, Sahu S, et al. Clinical spectrum of idiopathic inflammatory demyelinating disorders in a tertiary care centre in North–West India. Indian J Public Heal Res Dev 2019;10:338-44.|
|6||Pandit L. Demyelinating disorders in India – An update. In: G.S. wander, editor. Medicine -Update-2016. India: Jaypee Brothers. 2022. p. 1111-6.|
|7||Bhatia R, Bali P, Chowdhary R. Epidemiology and genetic aspects of multiple sclerosis in India. Ann Indian Acad Neurol 2015;18:6.|
|8||Chopra JS, Radhakrishnan K, Sawhney BB, Pal SR, Banerjee AK. Multiple sclerosis in North-West India. Acta Neurol Scand 1980;62:312-21.|
|9||Gourie-Devi M, Nagaraja D. Multiple sclerosis in South India. In: Multiple Sclerosis East and West. Japan: S. Karger AG; 2015. p. 135-47. Available from: https://www.karger.com/Article/FullText/408036. [Last accessed on 2021 Feb 03].|
|10||Das H, Gautam M, Lavaju P. An overview of idiopathic optic neuritis in eastern Nepal. Nepal J Ophthalmol 2010;2:10-5.|
|11||Godar MS, Sharma AK, Thapa M, Sitaula S, Gurung S. Demographic pattern and clinical characteristics of optic neuritis in a tertiary eye care centre. Nepal J Ophthalmol 2017;9:51-5.|
|12||Barhate KS, Ganeshan M, Singhal BS. A clinical and radiological profile of neuromyelitis optica and spectrum disorders in an Indian cohort. Ann Indian Acad Neurol 2014;17:77-81.|
|13||Mukherjee S, Guha G, Roy M, Ghosh S, Saha SP. A study on patients with neuromyelitis optica spectrum disorder from Eastern India. Neurol Psychiatry Brain Res 2020;35:22-8.|
|14||Pandit L, Rao R, Shetty R, Amin H, Bhat S, Misri Z, et al. Optic neuritis: Experience from a south Indian demyelinating disease registry. Neurol India 2012;60:470.|
|15||Saxena R, Phuljhele S, Menon V, Gadaginamath S, Sinha A, Sharma P. Clinical profile and short-term outcomes of optic neuritis patients in India. Indian J Ophthalmol 2014;62:265-7.|
|16||Singhi PD, Ray M, Singhi S, Kumar Khandelwal N. Acute disseminated encephalomyelitis in North Indian children: Clinical profile and follow-up. J Child Neurol 2006;21:851-7.|
|17||Jena SS, Alexander M, Aaron S, Mathew V, Thomas MM, Patil AK, et al. Natural history of multiple sclerosis from the Indian perspective: Experience from a tertiary care hospital. Neurol India 2015;63:866-73.|
|18||Syal P, Prabhakar S, Thussu A, Sehgal S, Khandelwal N. Clinical profile of multiple sclerosis in north-west India. Neurol India 1999;47:12-7.|
|19||Jayakrishnan MP, Krishnakumar P. Clinical profile of acute disseminated encephalomyelitis in children. J Pediatr Neurosci 2010;5:111-4.|
|20||Pandit L. Transverse myelitis spectrum disorders. Neurol India 2009;57:126.|
|21||Asgari N, Skejoe HP, Lillevang ST, Steenstrup T, Stenager E, Kyvik KO. Modifications of longitudinally extensive transverse myelitis and brainstem lesions in the course of neuromyelitis optica (NMO): A population-based, descriptive study. BMC Neurol 2013;13:33.|
|22||Gangopadhyay G, Das SK, Sarda P, Saha SP, Gangopadhyay P, Roy TN, et al. Clinical profile of multiple sclerosis in Bengal. Neurol India 1999;47:18-21.|
|23||Bansal PK, Nawal CL, Singh A, Chejara R, Marker S, Mangal P. Neuropathic pruritus: An early indicator of neuromyelitis optica spectrum disorders. Int J Res Med Sci 2018;6:2867.|
|24||Netravathi M, Saini J, Mahadevan A, Hari-Krishna B, Yadav R, Pal PK, et al. Is pruritus an indicator of aquaporin-positive neuromyelitis optica? Mult Scler 2017;23:810-7.|
|25||Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol 2007;6:805-15.|
|26||Sharma C, Kumawat B, Rana K, Panchal M, Khandelwal D, Parekh J. Neuromyelitis optica spectrum disorders: An experience from tertiary care hospital of North-West India. Indian J Med Spec 2017;8:192-6.|
|27||Schwarz S, Mohr A, Knauth M, Wildemann B, Storch-Hagenlocher B. Acute disseminated encephalomyelitis: A follow-up study of 40 adult patients. Neurology 2001;56:1313-8.|
|28||Koelman DL, Chahin S, Mar SS, Venkatesan A, Hoganson GM, Yeshokumar AK, et al. Acute disseminated encephalomyelitis in 228 patients: A retrospective, multicenter US study. Neurology 2016;86:2085-93.|
|29||Nakashima I, Fujihara K, Okita N, Takase S, Itoyama Y. Clinical and MRI study of brain stem and cerebellar involvement in Japanese patients with multiple sclerosis. J Neurol Neurosurg Psychiatry 1999;67:153-7.|
|30||Kanamori Y, Nakashima I, Takai Y, Nishiyama S, Kuroda H, Takahashi T, et al. Pain in neuromyelitis optica and its effect on quality of life: A cross-sectional study. Neurology 2011;77:652-8.|
|31||Lana-Peixoto MA, Talim N. Neuromyelitis optica spectrum disorder and anti-MOG syndromes. Biomedicines 2019;7:42.|
|32||Wakakura M, Minei-Higa R, Oono S, Matsui Y, Tabuchi A, Kani K, et al. Baseline features of idiopathic optic neuritis as determined by a multicenter treatment trial in Japan. Optic Neuritis Treatment Trial Multicenter Cooperative Research Group (ONMRG). Jpn J Ophthalmol 1999;43:127-32.|
|33||Gal RL. Visual function more than 10 years after optic neuritis: Experience of the optic neuritis treatment trial. Am J Ophthalmol 2004;137:77-83.|
|34||Du Y, Li JJ, Zhang YJ, Li K, He JF. Risk factors for idiopathic optic neuritis recurrence. PLoS One 2014;9:e108580.|
|35||Weinshenker BG, Wingerchuk DM, Vukusic S, Linbo L, Pittock SJ, Lucchinetti CF, et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol 2006;59:566-9.|
|36||Fujihara K. Treatment of neuromyelitis optica. Japanese J Clin Immunol 2012;35:129-35.|