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LETTER TO EDITOR
Year : 2014  |  Volume : 23  |  Issue : 1  |  Page : 71-73  Table of Contents     

Cerebral hemi atrophy as a consequence of cerebral insult in infancy: Is it difficult to diagnose?


1 Department of Psychiatry, Padmashree Dr. Dnyandeo Yashwantrao Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India
2 Consultant in Psychiatry, Padmashree Dr. Dnyandeo Yashwantrao Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India
3 Associate Professor Radiodiagnosis, Padmashree Dr. Dnyandeo Yashwantrao Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India

Date of Web Publication18-Nov-2014

Correspondence Address:
Daniel Saldanha
Flat No 1102, N Block, Grevillea,Magarpattacity, Pune - 411 013 Maharashtra
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-6748.144978

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How to cite this article:
Saldanha D, Chaudhari B, Mehta SK, Javadekar AN, Kharat A. Cerebral hemi atrophy as a consequence of cerebral insult in infancy: Is it difficult to diagnose?. Ind Psychiatry J 2014;23:71-3

How to cite this URL:
Saldanha D, Chaudhari B, Mehta SK, Javadekar AN, Kharat A. Cerebral hemi atrophy as a consequence of cerebral insult in infancy: Is it difficult to diagnose?. Ind Psychiatry J [serial online] 2014 [cited 2019 Sep 16];23:71-3. Available from: http://www.industrialpsychiatry.org/text.asp?2014/23/1/71/144978

Sir,

There are a variety of causes that lead to cerebral hemi atrophy with resultant complications, ranging from poor physical growth, hormonal abnormalities, mental retardation, seizures, and hemiplegia. [1],[2],[3],[4] Dyke, Davidoff, and Masson published a series of 9 cases in 1933 presenting with hemi paresis, seizures, facial asymmetry, and mental retardation and attributed to cerebral asymmetry on plain skull radiographs' and pneumato-encephalographs. [5] They showed thickening of calvarium, dilatation of frontal and ethnoid sinuses, and elevation of greater wing of sphenoid and petrous ridge. This condition is known as Dyke-Davidoff-Masson Syndrome (DDMS). Subsequent to this publication, several reports followed explaining the different types of cerebral atrophy and the distinction between atrophy and hyperplasia. Some case reports in children as well as in adults too were reported. [6],[7],[8] Brain insult from a variety of causes from foetal stage to early infancy can result in cerebral hemi atrophy. The causes are either congenital, i.e. idiopathic (Primary) and intrauterine vascular injury or acquired, i.e. perinatal intracranial hemorrhage, infection (Encephalitis), trauma, vascular abnormalities (Sturge-Weber's Syndrome), ischemia, hypoxia, etc., When the cerebral volume reduces in early stages, it may lead to changes in the skull, which is apparent in skull radiographs. Magnetic resonance imaging (MRI) reveals changes in the brain parenchyma with thinning of grey matter, reduced volume of white matter, enlarged lateral ventricle, reduced size of cerebral peduncles (ipsilateral), and reduced size of cerebellar hemisphere. We report a case of recurring seizures in a young 22-year-old female who presented to psychiatry outpatient in a tertiary care hospital and was diagnosed with cerebral asymmetry that could be traced to a cerebral insult in infancy.

A 22-year-old female was brought to the psychiatric OPD by her mother with the complaint of behavioral problems, recurring seizures, and weakness of right upper and lower limbs. Detailed history revealed that the patient was born at full term through vaginal delivery without any trauma/birth asphyxia. Birth weight recorded was 2300 g. At 3 months of age, the child suffered high-grade fever, had convulsions, and was admitted to the hospital for a month. She was suspected to be a case of encephalitis. Although the child recovered from fever and febrile convulsions, the milestones were delayed and speech was affected. Later, an IQ assessment showed moderate mental retardation (IQ 30-49). She had to be cared for her daily needs and had to be constantly attended to. As she grew up, seizures' returned at regular intervals and she was put on anticonvulsants by the neurophysician. She started showing behavioral changes in 2004 (at 14 years of age) in the form of irritability, aggressiveness, and violence while on anticonvulsants Tab Sodium Valproate 300 mg BD and Tab. Phenytoin sodium 100 mg three times a day. She was put on low doses of atypical antipsychotic tab Risperidone 1 mg once a day and small dose of benzodiazepine (Clonazepam 0.25 mg/day). She remained symptom free. Frequency of seizures reduced considerably to once in 3-4 months. General physical examination was normal. Her gait was hemi paretic type with motor power grade III in right upper and lower limbs.

All hematological and hormonal (T3, T4, TSH, FSH, LH) investigations revealed no abnormality. MRI brain done on 12/10/2012 revealed thickened skull vault with thickened diploic spaces. Mild prominence of the sylvian fissure sulcal spaces on the left side indicated hemi atrophy of the brain. Mild prominence of left lateral ventricular system was noted. Midline structures were maintained. Cerebellum and brain stem appeared normal [Figure 1].
Figure 1: MRI brain plain. Arrows show the thickened vault and decreased brain volume

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From the foregoing, it is apparent that the patient suffered a febrile illness, which was labeled as encephalitis at a very vulnerable age of 3 months and affected the growth of the brain. The extent of the brain damage was revealed only through the MRI brain [Figure 2] and [Figure 3] when she presented with behavioral problems. It showed the reason for right-sided weakness and hemi paretic gait, seizure disorder, mental retardation, and difficulty in speech due to atrophy and reduced volume of brain with thin grey matter and reduced volume of white matter. Since her IQ fell in the range of moderately mentally challenged group, not much thought was given to investigate the real reason for this state. The MRI findings of frontal lobe hemi atrophy together with seizures and mentally challenged state resembled the condition described by Dyke, Davidoff, and Masson's series of 9 cases in 1933. The appearance in these cases showed thickening of calvarium, dilatation of frontal, and ethnoid sinuses, and elevation of greater wing of sphenoid and petrous ridge. The MRI findings in the above case almost conform to this condition. Solomon [4] and Parker [5] while reporting on the case studies explained the mechanism behind thickening of the skull vault. It is a fact that the brain grows to half of its adult size by the first year and reaches 75% of the adult size by three years. Any insult during these years will result in stunted growth of the brain and the skull vault grows inwards resulting in thickening of the vault, enlargement of frontal sinuses, and increased width of diploic spaces and greater wing of sphenoid and petrous ridge on the affected side. This is possible when the brain damage is sustained in the first few months of infancy, as in this case. This shows that DDMS although not a very common condition, it can present in varying degree of severity. What is important is to document a proper clinical history, a detailed clinical examination and investigations (CT, MRI, and Hormonal) to confirm the diagnosis of DDMS. Cerebral insult very early in infancy due to febrile illness (Encephalitis) seems likely to be the cause in this case.
Figure 2: MRI brain plain. Arrows show hemiatrophy and reduction in the volume of brain parenchyma and thinning of grey matter

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Figure 3: MRI brain plain. Arrows show prominence of ventricular system. Mild prominences of sylvian fissure and sulcal spaces indicate hemi atrophy

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   References Top

1.Alpers BJ, Dear RB. Hemiatrophy of the brain. J Nerv Ment Dis 1939;89:653-651.  Back to cited text no. 1
    
2.Zilkha A. CT of cerebral hemiatrophy. AJR Am J Roentgenol 1980;135:259-62.  Back to cited text no. 2
    
3.Hageman G, Gooskens RH, Willemse J. A cerebral cause of arthrogryposis: Unilateral cerebral hypoplasia. Clin Neurol Neurosurg 1985;87:119-22.  Back to cited text no. 3
    
4.Solomon GE, Hilal SK, Gold AP, Carter S. Natural history of acute hemiplegia of childhood. Brain 1970;93:107-20.  Back to cited text no. 4
    
5.Dyke CG, Davidoff LM, Masson LB. Cerebral hemi atrophy with homolateral hypertrophy of skull and sinus. Surg Gynecol Obstet 1933;57:588-600.  Back to cited text no. 5
    
6.Parker CE, Harris N, Mavalwala J. Dyke-Davidoff-Masson syndrome. Five case studies and deductions from dermatoglyphics. Clin Pediatr (Phila) 1972;11:288-92.  Back to cited text no. 6
    
7.Goyal, V Shah, S Rao, N Jindal. Dyke Davidoff Masson syndrome in Children. The Internet Journal of Pediatrics and Neonatology. 2009;10:101-107.  Back to cited text no. 7
    
8.Sharma S, Goyal D, Negi A, Sood RG, Jhobta A, Surya M. Dyke-Davidoff Masson syndrome. Indian J Radiol Imaging 2006;16:165-6.  Back to cited text no. 8
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    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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