The disease presentation of Moyamoya angiopathy in Eastern India

Das, Shambaditya; Dubey, Souvik; Acharya, Mrinal; Ghosh, Ritwik; Chatterjee, Subhankar; Hazra, Avijit; Lahiri, Durjoy; Segupta, Samya; Chatterjee, Subham; Das, Goutam; Sarkar, Nilanjan; Ray, Biman Kanti; Kraemer, Markus

doi:10.1016/j.jstrokecerebrovasdis.2020.104957

Cited by 22 publications

(34 citation statements)

References 47 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Radiologically, in MMA brain atrophy is present in upto 63% of the patients and has a direct relation with the diagnostic latency. [ 1 ] Our patient did not have brain atrophy. DWI showed restriction in the splenium of corpus callosum along with hypointensity in ADC (Apparent Diffusion Coefficient) map, suggestive of infarction, (“Boomerang Sign”) [ Figure 1 ][ 5 ] in addition to the described infarcts.…”

mentioning

confidence: 74%

See 1 more Smart Citation

A Case of Moyamoya Syndrome with Isolated Protein S Deficiency Unmasked by Pregnancy

Chakraborty

Dubey

Sarkar

et al. 2021

Annals of Indian Academy of Neurology

Self Cite

View full text Add to dashboard Cite

mentioning

confidence: 74%

“…The mean delay in having onset of first neurological symptom to the final diagnosis in Eastern India is about 14.7 ± 41.7 months. [ 1 ] However our patient, previously asymptomatic had a stormy course over 1-2 weeks. Moreover, ischemic stroke as a presentation in MMA is less common than intracranial hemorrhage in Asian adults.…”

mentioning

confidence: 78%

A Case of Moyamoya Syndrome with Isolated Protein S Deficiency Unmasked by Pregnancy

Chakraborty

Dubey

Sarkar

et al. 2021

Annals of Indian Academy of Neurology

Self Cite

View full text Add to dashboard Cite

“…7 In the present case caudate atrophy might actually represent the severe end of the spectrum of DS due to prolonged neglected hyperglycemia. 24,25 The macrovascular angiopathy (such as involvement of lenticulostriate artery in Moyamoya vasculopathy 26 ) and Huntington's disease, and other neurodegenerative diseases (like neuroacanthocytosis, frontotemporal lobar degeneration, 27 and in some deposition disorders 28 ), where caudate atrophy is a predominant feature, 25 were ruled out by history, examination and relevant investigations, including genetic-testing wherever deemed appropriate.…”

Section: Discussionmentioning

confidence: 99%

Recurrent Facial Focal Seizures With Chronic Striatopathy and Caudate Atrophy—A Double Whammy in an Elderly Woman With Diabetes Mellitus

Chatterjee

Ghosh

Ojha

et al. 2021

The Neurohospitalist

Self Cite

View full text Add to dashboard Cite

Seizures and involuntary movements are relatively rare, but well-known neurological complications of non-ketotic hyperglycemia. While hemichorea-hemiballism secondary to diabetic striatopathy is increasingly being reported, unilateral caudate atrophy resulting from chronic vascular insufficiency/insult in a backdrop of poorly controlled diabetes mellitus is sparsely described in literature. We herein report a 75-year-old woman with poorly controlled diabetes mellitus who presented with concurrent epilepsia partialis continua involving left side of her face and hemichorea on the right side in the context of non-ketotic hyperglycemia. Neuroimaging revealed a space-occupying lesion suggestive of low-grade glioma in the right superior frontal cortex and left-sided caudate atrophy as well. Possibly, space-occupying lesion in motor cortex acted as an inciting factor for seizures and non-ketotic hyperglycemia further lowered the seizures threshold. On the other hand, atrophied left caudate had led to persistent choreiform movements secondary to chronic uncontrolled hyperglycemia. The simultaneous presence of acute and chronic neurological complications of diabetes mellitus makes this case unique. It also highlights the need for strict control of blood glucose and utility of appropriate neuroimaging to rapidly diagnose and prevent further complications.

show abstract

“…Possibly due to these genetic differences, the frequency and epidemiological features of MMA between East Asian countries like Korea and Japan, vary from those in the Western Hemisphere. Besides, differences have been observed in prevalence and MMA phenotype between countries within Asia (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21). Due to these regional variations, extrapolation of knowledge from Japanese MMA to the Indian population may be unwise (22).…”

Section: Introductionmentioning

confidence: 99%

“…Due to these regional variations, extrapolation of knowledge from Japanese MMA to the Indian population may be unwise (22). However, only limited data exist pertaining to MMA in the Indian population, with no nation-wide epidemiological study till date (14,(23)(24)(25)(26)(27)(28)(29)(30)(31). Nation-wide studies on MMA epidemiology are available for only a few countries like Japan, Korea, Taiwan, China, and the USA (4, 9-11, 17, 20, 21).…”

Section: Introductionmentioning

confidence: 99%

Epidemiology of Moyamoya Angiopathy in Eastern India

et al. 2022

Self Cite

View full text Add to dashboard Cite

IntroductionMoyamoya angiopathy (MMA) is a chronic, progressive intracranial vasculopathy with variation in prevalence and clinical manifestations across different populations. This study was aimed to estimate the frequency of MMA as an etiology of stroke and its epidemiological features in the largest cohort of MMA patients in India.MethodA single-centered cross-sectional observational study over a period of 5 years (2016–2021) was undertaken among consecutive stroke and transient ischemic attack (TIA) patients to look for the presence of MMA angiographically. Each patient with angiographically proven MMA was further evaluated for demographic, clinical, and radiological characteristics.ResultsAmong 10,250 consecutive stroke and TIA patients (ischemic = 78%, hemorrhagic = 22%), frequency of MMA was 1.56% (n = 160); 15.3% among children. Female preponderance (Male:Female = 1:1.4) was noted among 160 MMA patients, with bimodal age distribution, first peak at 3–8 years, and a shorter second peak at 41–47 years. Childhood-onset MMA was seen in 75 (46.9%) with commonest initial neurological symptom of fixed-motor-weakness (44.0%), followed by TIA (26.7%); while 85 (53.1%) had adult-onset MMA with fixed-motor-weakness (50.6%) followed by headache (24.7%) as the predominant initial neurological symptom; seizure significantly higher in children (p < 0.001) and headache in adults (p = 0.012). Transient and fixed neurological manifestations constituted 87.5 and 69.4% respectively, of symptoms throughout the disease course. Cerebral infarction (45.0%) and TIA (21.9%) were the commonest types of MMA. On brain imaging, infarction was noted in 80.6%, hemorrhage in 11.3%, significantly higher among adults (p < 0.001). Cortical infarct and Gyral pattern were commoner in children (p = 0.004), subcortical infarcts in adults (p = 0.018). Frequent Suzuki staging observed was stage 4 (31.3%), followed by stage 3 (30.0%). Involvement of posterior circulation was detected in 55.6%, brain atrophy at the time of diagnosis was seen in 65.0%.ConclusionMMA is an important etiological consideration in patients with stroke, especially in children. It can present with a myriad of transient neurological symptoms, frequently overlooked, leading to delayed diagnosis, and contributing to socio-economic burden. Indian MMA showed aberrations in its gender predisposition, age distribution, frequency of familial cases, disease manifestation, and type of stroke, in comparison to its Japanese and Caucasian counterparts pointing to the inter- and intra-continent differences of MMA phenotype. Future development of the Indian MMA national registry is of essence.

show abstract

The disease presentation of Moyamoya angiopathy in Eastern India

Cited by 22 publications

References 47 publications

A Case of Moyamoya Syndrome with Isolated Protein S Deficiency Unmasked by Pregnancy

A Case of Moyamoya Syndrome with Isolated Protein S Deficiency Unmasked by Pregnancy

Recurrent Facial Focal Seizures With Chronic Striatopathy and Caudate Atrophy—A Double Whammy in an Elderly Woman With Diabetes Mellitus

Epidemiology of Moyamoya Angiopathy in Eastern India

Contact Info

Product

Resources

About