The Hemispheric Imbalance: A Double Encounter with Dyke-Davidoff-Masson Syndrome

Abstract

This article aims to present and interpret two instances of Dyke-Davidoff-Masson syndrome (DDMS), with the main features of cerebral hemiatrophy and compensatory skull changes, focusing on the clinical spectrum, imaging features, and variations in timing of presentation. Two male patients with left cerebral hemisphere involvement were evaluated. The first case was an 8-year-old child presenting with intellectual disability, delayed milestones, right hemiparesis, abnormal jerky movements, and generalized seizures. The second case was a 22-year-old adult with hemiparesis and generalized seizures since birth. Both underwent detailed neurological examination and magnetic resonance imaging (MRI) on a 3T scanner, including time-of-flight (TOF) angiography to assess cerebral vasculature. MRI of the first patient demonstrated left cerebral hemiatrophy with dilatation of the ipsilateral lateral ventricle and sulcal prominence, mild falcine deviation toward the affected side, and compensatory hypertrophy of the left calvarial bones (temporal, parietal, frontal, occipital) with enlargement of the frontal sinus and mastoid air cells and elevation of the left petrous ridge. Mild attenuation of the left middle cerebral artery (MCA) segments was seen on TOF imaging. The second patient's MRI revealed left cerebral hemisphere atrophy with gliosis in the MCA territory, left ventricular dilatation, ipsilateral sulcal prominence, and a neuroglial cyst in the left insular cortex with similar bony changes as those of the previous patient. TOF angiography showed attenuation of the left MCA segments without filling defects. DDMS is associated with a certain specific set of clinical and radiological features. Early recognition through MRI and computed tomography is essential for appropriate management and improved outcomes. These cases illustrate the variability in presentation and emphasize the importance of considering DDMS in the differential diagnosis of unilateral cerebral atrophy with neurological deficits.

 

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Keywords

Dyke-Davidoff-Masson syndrome - cerebral hemisphere atrophy - calvarial hypertrophy - middle cerebral artery

Background

Dyke-Davidoff-Masson syndrome (DDMS), first described by Dyke, Davidoff, and Masson in 1933, is a rare neurological condition characterized by hemiparesis with typical radiological findings. This condition is characterized by a range of symptoms, including intellectual disability, facial asymmetry, speech and gait problems, contralateral hemiparesis/hemiplegia, epileptic seizures, and chorea.[1] Characteristic radiological changes include cerebral hemisphere atrophy, dilatation of ventricles with ipsilateral falcine shift and compensatory hypertrophy of the calvaria, enlargement of frontal sinuses, and elevation of the petrous ridge. This condition can be easily overlooked or misdiagnosed due to its uncommon occurrence.[2] [3] [4] We present two cases of DDMS at different times of presentation, which illustrate unique and defining features of this syndrome. Both cases were referred to the department of radiology for magnetic resonance imaging (MRI) evaluation of the brain, following which a diagnosis of DDMS was made using clinical details and imaging findings.

 

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Case 1

An 8-year-old male child presented with complaints of intellectual disability, delayed milestone achievement, and right hemiparesis with abnormal jerky movements from 2.5 years of age, followed by generalized tonic and clonic seizures from 4 years of age. A history of behavioral changes in the form of increased irritability and increased frequency of temper tantrums was present. There was no history of any previous infection, prolonged illness, or hospital stay. The birth history was insignificant.

Upon examination, the child was irritable and inattentive. The sensory examination revealed diminished sensation on the right side of the body. The motor function of the right upper and lower limbs was assessed at ⅘. The rest of the examination was unremarkable.

The patient was taken up for an MRI examination on a GE Discovery 3T 750W scanner, which revealed left cerebral hemisphere atrophy, resultant dilatation of ipsilateral lateral ventricle and sulcal spaces with mild deviation of the falx toward the left. Associated compensatory hypertrophy of the left temporal bone, left parietal bone, and left side of the frontal and occipital bones was also seen with enlargement of the frontal sinus and mastoid air cells on the left side with elevation of the left petrous ridge ([Fig. 1A–D]). The right cerebral hemisphere, brainstem, and bilateral cerebellar hemispheres were normal in morphology and signal intensity. Axial and coronal time-of-flight (TOF) images revealed mild attenuation of all the segments of the left middle cerebral artery (MCA) ([Fig. 1E] and [F]).

Fig. 1 Axial T2-weighted images (A, B), T1-weighted images (C), and axial fluid-attenuated inversion recovery (FLAIR) image (D) show volume loss and thinning of the gray white matter of the left frontoparietotemporal lobe (black asterisk) with enlargement of adjacent sulcal spaces and dilation of the ipsilateral lateral ventricle. In addition, calvaria thickening is seen over the right frontoparietotemporal convexity (white arrow), and dilation with mucosal hypertrophy of the ipsilateral frontal sinus is also seen (yellow asterisk). In maximum intensity projection (MIP) images of time-of-flight (TOF) angiography in axial (E) and coronal planes (F), the left middle cerebral artery (MCA) and its cortical branches (green asterisk) are attenuated in caliber.

The patient has been seizure-free for 6 months and has been taking two antiepileptic medications for the past year. Furthermore, the child is receiving appropriate physical rehabilitation.

 

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Case 2

Another patient presented to the radiology department for an MRI of the brain at 22 years of age with complaints of generalized tonic-clonic seizures and hemiparesis since birth. No other significant clinical details were present. On examination, sensory functions were preserved; however, there was marked compromise in the motor function with ⅖ power in both upper and lower limbs of the right side.

The patient's MRI demonstrated a paucity of white and gray matter with gliosis in the left MCA territory, left ventricular dilatation, and prominence of ipsilateral sulcal spaces. The falx cerebri was slightly shifted toward the left. There was the formation of a neuroglial cyst in the left insular cortex. Wallerian degeneration was also seen in the form of left hemi-midbrain and pontine atrophy. Compensatory hypertrophy of the left side of the calvaria was seen with mild hypertrophy of the frontal sinus on the left side and elevation of the left petrous ridge ([Fig. 2A–D]). On TOF images, all the segments of the left MCA were attenuated, with no filling defect seen ([Fig. 2E] and [F]).

Fig. 2 Coronal T2-weighted images (A, B), fluid-attenuated inversion recovery (FLAIR) image (C), and axial T2 image (D) show volume loss and thinning of the gray white matter of the left frontoparietotemporal lobe (black asterisk) with enlargement of adjacent sulcal spaces and dilation of the ipsilateral lateral ventricle. Mild calvaria thickening is seen over the right frontoparietotemporal convexity (white arrow), and dilation of the ipsilateral frontal sinus is also seen (yellow asterisk). In maximum intensity projection (MIP) images of time-of-flight (TOF) angiography in axial planes (E and F), the left middle cerebral artery (MCA) and its cortical branches (green asterisk) are attenuated in caliber.

This patient is also on an antiepileptic drug with noncompliance to medication.

 

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Discussion

DDMS is a rare neurological condition with a distinct spectrum of symptoms and imaging features. These symptoms include intellectual disability, hemiparesis, facial asymmetry, and seizures. Most of the cases present and are diagnosed in the pediatric population; however, rare occurrences of adult presentation are also seen.[2]

Etiology

It can be broadly divided into congenital and acquired types of DDMS, depending on whether the insult occurs during gestation or at a later stage in the patient's life. The main cause of congenital DDMS remains vascular abnormality, and diagnosis is made at a median age of 14.2 years. On the other hand, acquired DDMS has a wide range of potential causes, including infections, trauma, cerebral ischemia, neoplastic etiologies, immunological disorders, or idiopathic causes. Acquired DDMS is diagnosed at a median age of 22.8 years. The severity of the insult and age of the patient determine the magnitude of the imaging findings.[1]

 

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Clinical Implications

Although there is no definitive gender predilection or hemispheric dominance, cases involving the left hemisphere and male patients appear more frequently in the literature.[3] Ünal et al, in their study published in 2004, documented male sex predominance and also the predilection of left hemisphere involvement over right.[5] The brain undergoes rapid maturation in the early stages of life. It is hypothesized that in children, particularly between the ages of 1 and 3 years, there is an increase in cerebral blood flow on the right side. This could be a possible reason for frequent left hemisphere involvement.[1] In both our cases, the patients were male and had involvement of the left hemisphere.

 

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Imaging Classifications

Computed tomography and MRI are considered the gold standards for diagnosing DDMS. The main imaging findings include cerebral hemiatrophy, ipsilateral ventricular prominence, ipsilateral falcine shift, compensatory hypertrophy of the calvaria, enlargement of frontal sinuses, and elevation of the petrous ridge.[4] [6] Congenital forms generally show cerebral volume loss, gliosis, ipsilateral ventricular enlargement, falx cerebri shift, and cerebral vascular malformations. Whereas acquired type shows dilated sulci, calvarial changes, and hyperpneumatization of paranasal sinuses. However, compensatory bone changes can usually be absent in acquired cases, according to the literature, as the brain insult possibly occurs after the calvarial maturation.[1] When the brain experiences injury of any kind before the age of 3, compensatory changes occur in the overlying skull, including inward growth of the calvarial bones, widening of the diploic spaces and paranasal sinuses, as well as elevation of the petrous ridge and orbital roof.[7]

There are three imaging patterns of congenital hemiatrophy: diffuse cortical and subcortical atrophy (pattern I), diffuse cortical atrophy with porencephalic cysts (pattern II), and old infarction with gliosis in the territory of the MCA (pattern III).[8] Aggarwal et al proposed the addition of pattern IV, which entails an old infarct with gliosis in the anterior and the MCA territories.[9] Our first case had pattern I, whereas the second patient had involvement similar to pattern III.

 

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Differential Diagnosis

Sturge–Weber syndrome (SWS) and Rasmussen encephalitis (RE) are important differential diagnoses to consider in cases of suspected DDMS. SWS can be differentiated from DDMS by the presence of characteristic facial cutaneous vascular malformations, leptomeningeal enhancement, cortical calcifications, enlarged transmedullary veins, and choroid plexus. Whereas RE is an immune-mediated chronic condition associated with infections and demonstrates progressive atrophy, typically of gray matter.[1] Early diagnosis is crucial for guiding seizure management, timely drug adjustments, initiating physiotherapy and behavioral therapy, and ensuring better long-term outcomes.[10] [11]

Our cases illustrate two distinct patterns of congenital DDMS, with one case presenting in the first decade and another showing symptoms from birth but a delayed diagnosis in adulthood. This comparison provides the similarities in the radiological findings across different times of presentation, also highlighting the increased severity of degenerative changes observed in patients presenting in adulthood. This serves as a diagnostic guide for clinicians and radiologists by providing insights into the evolution of findings over time, aiding in prognosis and multidisciplinary management planning.

 

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Conclusion

DDMS is a rare neurological condition that has a variety of clinical and imaging characteristics. These cases offer valuable insights into the diverse ways DDMS can present. Early diagnosis of this condition is crucial for effective seizure management, rehabilitation, and behavioral therapy, all of which can significantly enhance long-term neurological health and functional outcomes.

 

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Conflict of Interest

None declared.

Authors' Contributions

J.C. and R.S. drafted the article and prepared images. S.G.J. edited the draft and images, supervised the work, and finalized the manuscript.

Patients' Consent

Written informed consent was obtained from the patients to publish this case report and any accompanying images.

^* These authors' have contributed equally and share the first authorship.

• References

• 1 Rondão MBA, Hsu BRRHS, Centeno RS, de Aguiar PHP. Dyke-Davidoff-Masson syndrome: main clinical and radiological findings- systematic literature review. Seizure 2023; 110: 58-68
• 2 Diestro JDB, Dorotan MKC, Camacho AC, Perez-Gosiengfiao KT, Cabral-Lim LI. Clinical spectrum of Dyke-Davidoff-Masson syndrome in the adult: an atypical presentation and review of literature. BMJ Case Rep 2018; 2018: x
• 3 Younas A, Saim M, Maqsood H, Younus S, Hassan Raza M. Dyke-Davidoff-Masson syndrome: a case report and review of literature. Cureus 2020; 12 (12) e11919
• 4 Abdul Rashid AM, Md Noh MSF. Dyke-Davidoff-Masson syndrome: a case report. BMC Neurol 2018; 18 (01) 76
• 5 Ünal O, Tombul T, Çırak B, Anlar O, İncesu L, Kayan M. Left hemisphere and male sex dominance of cerebral hemiatrophy (Dyke-Davidoff-Masson Syndrome). Clin Imaging 2004; 28 (03) 163-165
• 6 Kumar NV, Gugapriya TS, Guru AT, Kumari SN. Dyke-Davidoff-Masson syndrome. Int J Appl Basic Med Res 2016; 6 (01) 57-59
• 7 Jageer M, Siripuram DH, Abdullah DS, Rachakonda DV. A rare disease Dyke-Davidoff-Masson syndrome-case series. 2022. Accessed April 11, 2025 at: https://www.semanticscholar.org/paper/A-RARE-DISEASE-DYKE-DAVIDOFF-MASSON-SYNDROME-CASE-Jageer-Siripuram/c579f6bf7a19159dcd1567235df121bb93b43671
• 8 Shen WC, Chen CC, Lee SK, Ho YJ, Lee KR. Magnetic resonance imaging of cerebral hemiatrophy. J Formos Med Assoc 1993; 92 (11) 995-1000
• 9 Aggarwal A, Aggarwal AK, Kapoor A, Kapoor R, Bansal A. Hemiatrophy of brain: antenatal ultrasonography and MRI/postnatal MRI diagnosis with the introduction of “shifted falx sign”. J Med Ultrason 2017; 44 (01) 147-151
• 10 Behera MR, Patnaik S, Mohanty AK. Dyke-Davidoff-Masson syndrome. J Neurosci Rural Pract 2012; 3 (03) 411-413
• 11 Palani A, Periyanayagam A, James S, Thangavel R, Sudarsan A. Dyke-Davidoff-Masson syndrome - a dainty spectrum with a diligent diagnosis. J Family Med Prim Care 2024; 13 (10) 4730-4733

Publication History

Article published online:
24 July 2025