Infantile Tremor Syndrome Followed by West Syndrome: Effect or Continuation of Spectrum?

2020 ◽  
Vol 66 (5) ◽  
pp. 556-560
Author(s):  
Chandrika Azad ◽  
Jasmine Singh ◽  
Vishal Guglani
Keyword(s):  
Vitamin B12 ◽  
Indian Subcontinent ◽  
Time Lag ◽  
Case Series ◽  
Vitamin B12 Deficiency ◽  
Deficiency Syndrome ◽  
Epileptic Encephalopathy ◽  
West Syndrome ◽  
Normal Limits ◽  
B12 Deficiency

Abstract Background Infantile tremor syndrome (ITS) is a nutritional deficiency syndrome, frequently reported from the Indian subcontinent caused by vitamin B12 deficiency. The West syndrome (WS), on the other hand, is a type of epileptic encephalopathy with variable etiology. Case Series We present a series of five children who presented with symptoms consistent with ITS and received standard intramuscular vitamin B12 therapy to which good response was observed. All these children were readmitted with WS with a time lag varying from 2 to 12 months. Magnetic resonance imaging brain and metabolic screen were within normal limits. Three out of five patients responded well to adrenocorticotropic hormone (ACTH), remaining two required additional drugs. We hypothesize that WS could have developed after ITS due to developmental desynchronization. Conclusion In the Indian scenario, it is particularly important to be aware of appearance of WS after ITS, because of high prevalence of ITS and devastating nature of WS.

Neuropsychological Profiles of Children Following Vitamin B12 Deficiency During Infancy: A Case Series

2016 ◽  
Vol 17 (3) ◽  
pp. 242-253
Author(s):  
M. Greenham ◽  
V. Anderson ◽  
J. Campbell ◽  
P. Monagle ◽  
M.H. Beauchamp
Keyword(s):  
Vitamin B12 ◽  
Case Series ◽  
Vitamin B12 Deficiency ◽  
Brain Maturation ◽  
Attention And Memory ◽  
Long Term Outcomes ◽  
Retrospective Case ◽  
School Aged Children ◽  
B12 Deficiency

Previous studies investigating long-term outcomes in children following vitamin B12 deficiency during infancy have been limited to IQ or clinical observation. This paper seeks to describe comprehensive neuropsychological profiles in a case series of school-aged children who were treated for infantile vitamin B12 deficiency. This was a retrospective case series of seven children who were treated for vitamin B12 deficiency during infancy and aged 5 to 16 years at the time of testing. While most children had age-expected intellectual performance, the distribution of the sample was skewed to the lower end of the normal range. Furthermore, children were found to have impairments in a number of neuropsychological domains, most common were attention and memory, followed by executive function. These results suggest that while neurological symptoms quickly resolve following treatment, these effects on early brain development may disrupt brain maturation and have the potential to impact on later development.

scholarly journals West Syndrome in an Infant With Vitamin B12 Deficiency Born to Autoantibodies Positive Mother

2019 ◽  
Vol 7 ◽  
Author(s):  
Pin Fee Chong ◽  
Masaru Matsukura ◽  
Kaoru Fukui ◽  
Yoriko Watanabe ◽  
Naomichi Matsumoto ◽  
...  
Keyword(s):  
Vitamin B12 ◽  
Vitamin B12 Deficiency ◽  
West Syndrome ◽  
Positive Mother ◽  
B12 Deficiency

Vitamin B12 deficiency in infants secondary to maternal deficiency: A case series of seven infants

2017 ◽  
Vol 21 ◽  
pp. e3 ◽  
Author(s):  
A. Fadilah ◽  
R. Musson ◽  
M.T. Ong ◽  
A.V. Desurkar ◽  
S.R. Mordekar
Keyword(s):  
Vitamin B12 ◽  
Case Series ◽  
Vitamin B12 Deficiency ◽  
B12 Deficiency

Vitamin B12 Deficiency and West Syndrome: An Uncommon but Preventable Cause of Neurological Disorder. Report on Three Cases, One of Them with Late Onset during Vitamin B12 Treatment

2021 ◽  
Author(s):  
Piero Pavone ◽  
Federica Sullo ◽  
Raffaele Falsaperla ◽  
Filippo Greco ◽  
Agustina Crespo ◽  
...  
Keyword(s):  
Vitamin B12 ◽  
Late Onset ◽  
Vitamin B12 Deficiency ◽  
West Syndrome ◽  
Water Soluble ◽  
Vegan Diet ◽  
Inborn Errors ◽  
Water Soluble Vitamin ◽  
B12 Deficiency ◽  
Somatic Diseases

AbstractVitamin B12 is a water-soluble vitamin that plays a fundamental role as an essential cofactor for two enzymes responsible for the production of succinyl-CoA and methionine. Vitamin B12 deficiency can occur in infants and may be related to the breastfeeding mother's adherence to a vegan diet or somatic diseases in the mother. It should be differentiated from inborn errors of vitamin B12 metabolism. Herein, we report the cases of three infants with West syndrome; all three were breastfed by mothers who followed a strict vegan diet. In one of the three infants, West syndrome developed during treatment with vitamin B12 and normalization of the vitamin B12 level. Early treatment and replacement therapy are worthwhile to prevent serious neurological problems and to improve the patient's clinical course.

scholarly journals KNUCKLE HYPERPIGMENTATION AS A PRELIMINARY MARKER OF VITAMIN B 12 DEFICIENCY: A CASE SERIES

2021 ◽  
pp. 36-38
Author(s):  
Manali Patil ◽  
S. N. Agrawal ◽  
V. V. Saoji
Keyword(s):  
Vitamin B12 ◽  
Megaloblastic Anemia ◽  
Case Series ◽  
Vitamin B12 Deficiency ◽  
Neurological Deficits ◽  
Vitamin B ◽  
Important Clue ◽  
B12 Deficiency ◽  
Systemic Manifestations ◽  
Vitamin B 12

Vitamin B12 deficiency is common in vegetarian population in India and can present with variable Hematological (megaloblastic anemia), Neuropsychiatric, Mucocutaneous (glossitis, angular stomatitis), Skin (pallor, hyperpigmentation) and Hair changes (dry, brittle, thin, lustreless, prematurely grey). Knuckle hyperpigmentation has been described in vitamin B12 deficiency but usually these patients are dermatologically asymptomatic but they have systemic manifestations like megaloblastic anemia, pancytopenia or neurological deficits. The foreground of this study is highlighting the importance of knuckle hyperpigmentation as an early cutaneous sign of vitamin B12 deficiency which points an important clue towards the aetiology of Megaloblastic anemia.

scholarly journals West syndrome due to vitamin B12 deficiency

2015 ◽  
Vol 50 (4) ◽  
pp. 251-253 ◽  
Author(s):  
Hepsen Mine Serin ◽  
Aslihan Orucoglu Kara ◽  
Baran Oguz
Keyword(s):  
Vitamin B12 ◽  
Vitamin B12 Deficiency ◽  
West Syndrome ◽  
B12 Deficiency

scholarly journals Subacute combined degeneration of the spinal cord in functional vitamin B12 Deficiency states

2021 ◽  
Vol 20 (4) ◽  
pp. 21-23
Author(s):  
Christina Englezou ◽  
◽  
Di Liang ◽  
Keyword(s):  
Spinal Cord ◽  
Vitamin B12 ◽  
Cognitive Deficits ◽  
Metabolic Pathways ◽  
Case Series ◽  
Vitamin B12 Deficiency ◽  
Neurological Manifestations ◽  
Subacute Combined Degeneration ◽  
Ability To Act ◽  
B12 Deficiency

Vitamin B12 is an essential enzymatic cofactor in multiple cellular metabolic pathways. Deficiency states can arise as a result of both depletion of absolute systemic levels and pathological processes that block its ability to act as an enzymatic cofactor. The latter is also known as functional vitamin B12 deficiency. This can cause a variety of systemic, haematological, and neurological manifestations, some of which may be irreversible if not promptly treated. Neurological syndromes include subacute combined degeneration of the cord (SCDC), peripheral, optic, and autonomic neuropathies, and neuropsychiatric or cognitive deficits. This review presents a case series of vitamin B12 deficiencies leading to SCDC, and we include the clinical features, significant investigations, treatments, and prognoses.

scholarly journals Case Series on Vitamin B12 Deficiency...Though Rare but a Treatable Disease

2018 ◽  
Vol 1 (1) ◽  
Keyword(s):  
Vitamin B12 ◽  
Developmental Delay ◽  
Megaloblastic Anemia ◽  
Case Series ◽  
Vitamin B12 Deficiency ◽  
Breastfed Infants ◽  
B12 Deficiency ◽  
Breast Fed

Megaloblastic anemia due to vitamin B12 deficiency is an uncommon problem in childhood that is most frequently associated with decreased ingestion or impaired absorption or utilization of B12 [1,2]. Nutritional B12 deficiency in childhood is rare. Most cases are due to maternal insufficiency, resulting from deficient stores and intake generally among exclusively breastfed infants [3]. B12 deficiency in children often presents with nonspecific manifestations [4,5]. We present two cases of vitamin B12 deficiency in breast fed infants presenting as pancytopenia and gross developmental delay respectively

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