Dr Pir Abdul Ahad Aziz Qureshi1, Dr Peer Asad Aziz Qureshi2, Dr Qurat-ul-ain Qureshi3, Dr Saifullah Brohi4Patient
20 years, female
A 20-year-old female presented with complaints of right-sided weakness, epilepsy and gait problems. On physical examination, she was mentally disabled and showed facial asymmetry and contralateral hemiparesis. On further investigation, she manifested dysdiadochokinesia on the right side.
CT scan images show atrophy of the left cerebral hemisphere with a large hypodense area almost entirely involving the left cerebral hemisphere. It is associated with effacement of cortical sulci and ex-vacuo dilatation of the left lateral and 3rd ventricles and ipsilateral shift of midline (Fig. 1). There is also enlargement of the ipsilateral frontal and ethmoid sinuses with thickening of the left frontal and parietal bones (Fig. 2 and 3). In addition, there is mild atrophy of the contralateral right cerebellar hemisphere (Fig. 4).
Based on these findings, the diagnosis of Dyke-Davidoff-Mason syndrome with old ischemic infarction and crossed cerebellar diaschisis was made.
Diaschisis refers to the phenomenon in which there is reduced or loss of function of one part of the brain due to the involvement of another remote part of the brain secondary to the disturbance in the afferent neuronal pathway resulting in alteration in the cerebral blood flow and regional neuronal metabolism. This term was first described by the Russian neuropathologist von Monakow in 1914 and was first imaged by Baron et al. in 1980 on positron emission tomography (PET) [1,2]. Crossed cerebellar diaschisis (CCD) therefore represents a decrease in the perfusion, metabolism and function of the cerebellar hemisphere occurring as a result of a lesion in the contralateral cerebral hemisphere  which is thought to result from an interruption in the cerebro-cerebellar pathways probably the cortico-pontocerebellar fibres which connect the cerebral hemisphere with contralateral cerebellum . Many causes have been described in the literature that can cause this phenomenon like cerebral infarction, encephalitis, tumours, intracerebral haemorrhage, Dyke-Davidoff-Masson syndrome, and radiation necrosis [4-7]. Radiologically, on CT scan and MRI, there is volume loss of the contralateral cerebral hemisphere and Wallerian degeneration in CCD . Moreover, on PET-CT scan, hypometabolism is seen in the involved cerebellar hemisphere, which is diagnostic of this condition.
In our case, the patient had typical findings of Dyke-Davidoff-Masson syndrome (DDMS). DDMS is characterized by hemicerebral hypoplasia/atrophy due to brain insult in fetal or early childhood. There is also compensatory hypertrophy of ipsilateral skull bones, enlargement of the frontal and ethmoid sinuses and mastoid air cells, ipsilateral falcine displacement, the elevation of the petrous ridge and capillary malformations [8,9]. The patients with DDMS usually present with facial asymmetry, seizures, intellectual disability and contralateral hemiparesis .
DDMS can be due to congenital (infantile) or acquired aetiology. The congenital causes can include infection, occlusion of the middle cerebral artery in the neonatal or gestational period, coarctation of the mid-aortic arch and unilateral cerebral arterial circulation anomalies. The acquired causes can include infection, ischemia, tumour, trauma, haemorrhage and prolonged febrile seizures . In our case, the patient has an old ischemic infarction involving the left cerebral hemisphere, resulting in DDMS and CCD.
 Carrera E, Tononi G. (2014) Diaschisis: past, present, future. Brain;137(Pt 9):2408-22. doi: 10.1093/brain/awu101. Epub 2014 May 28 (PMID: 24871646)
 Han S, Wang X, Xu K, Hu C. (2016) Crossed Cerebellar Diaschisis: Three Case Reports Imaging Using a Tri-Modality PET/CT-MR System. Medicine;95(2):e2526. doi: 10.1097/MD.0000000000002526 (PMID: 26765477)
 Miura H, Nagata K, Hirata Y, Satoh Y, Watahiki Y, Hatazawa J. (1994) Evolution of crossed cerebellar diaschisis in middle cerebral artery infarction. J Neuroimaging;4(2):91-6 (PMID: 8186536)
 Tien RD, Ashdown BC. (1992) Crossed cerebellar diaschisis and crossed cerebellar atrophy: correlation of MR findings, clinical symptoms, and supratentorial diseases in 26 patients. AJR Am J Roentgenol;158(5):1155-9 (PMID: 1566683)
 Förster A, Kerl HU, Goerlitz J, Wenz H, Groden C. (2014) Crossed cerebellar diaschisis in acute isolated thalamic infarction detected by dynamic susceptibility contrast perfusion MRI. PLoS One;9(2):e88044. doi: 10.1371/journal.pone.0088044 (PMID: 24505372)
 Jiménez-Caballero PE. (2012) Crossed cerebellar atrophy: Update. J Neurosci Rural Pract;3(3):235-6. doi: 10.4103/0976-3147.102588 (PMID: 23188966)
 Demir Y, Sürücü E, Çilingir V, Bulut MD, Tombul T. (2015) Dyke-Davidoff-Masson Syndrome With Cerebral Hypometabolism and Unique Crossed Cerebellar Diaschisis in 18F-FDG PET/CT. Clin Nucl Med;40(9):757-8. doi: 10.1097/RLU.0000000000000890 (PMID: 26164180)
 Bagazgoitia L, García-Peñas JJ, Duat-Rodríguez A, Hernández-Martín A, Torrelo A. (2010) Facial capillary malformation and Dyke-Davidoff-Masson syndrome. Pediatr Neurol;43(3):202-4. doi: 10.1016/j.pediatrneurol.2010.04.011 (PMID: 20691943)
 Ayas ZÖ, Asil K, Öcal R. (2017) The clinico-radiological spectrum of Dyke-Davidoff-Masson syndrome in adults. Neurol Sci;38(10):1823-1828. doi: 10.1007/s10072-017-3074-7. Epub 2017 Jul 21 (PMID: 28733757)
 Singh P, Saggar K, Ahluwalia A. (2010) Dyke-Davidoff-Masson syndrome: Classical imaging findings. J Pediatr Neurosci;5(2):124-5. doi: 10.4103/1817-1745.76108 (PMID: 21559157)
 Behera MR, Patnaik S, Mohanty AK. (2012) Dyke-Davidoff-Masson syndrome. J Neurosci Rural Pract;3(3):411-3. doi: 10.4103/0976-3147.102646 (PMID: 23189018)
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.