|Ahead of print
|Porencephalic cyst with ipsilateral cerebral hemiatrophy presenting with generalized tonic–clonic seizures and neuropsychiatric features: A case report with literature review
Bhogaraju Anand1, Mahesh Chandra2, Neilofer Hussain3
1 Professor, Department of Psychiatry, Malla Reddy Institute of Medical Sciences, Hyderabad, Telangana, India
2 Professor, Radiology, Malla Reddy Institute of Medical Sciences, Hyderabad, Telangana, India
3 Assistant Professor, Department of Psychiatry, Malla Reddy Institute of Medical Sciences, Hyderabad, Telangana, India
Click here for correspondence address and email
|Date of Submission||27-Nov-2021|
|Date of Acceptance||02-Jan-2022|
|Date of Web Publication||08-Apr-2022|
Porencephalic cysts in combination with cerebral hemiatrophy have been described rarely. Herewith, we describe an 18-year-old female who presented with seizures, neuropsychiatric symptoms, and mild intellectual disability without facial asymmetry or neurological signs of weakness or rigidity. Magnetic resonance imaging head and brain, neuropsychological tests, and video electroencephalography findings are presented. The combination of porencephaly with cerebral hemiatrophy and intellectual disability with neuropsychiatric symptoms is extremely rare and probably the only case. A review of available studies is presented.
Keywords: Cerebral hemiatrophy, neuropsychiatric symptoms, porencephaly, seizure
|How to cite this URL:|
Anand B, Chandra M, Hussain N. Porencephalic cyst with ipsilateral cerebral hemiatrophy presenting with generalized tonic–clonic seizures and neuropsychiatric features: A case report with literature review. Arch Ment Health [Epub ahead of print] [cited 2023 Mar 22]. Available from: https://www.amhonline.org/preprintarticle.asp?id=342751
| Introduction|| |
Porencephaly is cysts or cavities within cerebral hemisphere, with reported estimated incidence rates of 5.2/100,000 live births., Cerebral hemiatrophy syndromes are uncommon and characterized by atrophy or hypoplasia of one cerebral hemisphere. Combination of porencephalic cyst communicating with lateral ventricle on right side with ipsilateral cerebral hemiatrophy, generalized tonic/clonic seizures, intellectual disability, and neuropsychiatric syndrome has not been reported in literature.
| Case Report|| |
Patient information and clinical findings
A 18-year-old female presented with 1-week episodes (2–3 times in a day) of giddiness, blurred vision with confusion, lasting for few minutes, followed by full recovery.
From the age of 13 years, she has generalized tonic–clonic seizures with variable frequency (occasionally 3–5 episodes in a day). For the past 6 months, she was on treatment with carbamazepine and phenytoin. Moreover, treatment compliance was good. The seizures were also triggered by contact with very cold water as per her mother's narration. For the last 6 months, the patient developed behavioral problems characterized by sudden onset of unprovoked anger spells, aggression, suspiciousness about neighbors spreading rumors about her, mood swings with crying spells, and suicidal ideas. These episodes were interspersed with seizures. She has disturbed sleep and reduced appetite and deterioration in personal care. Administration of risperidone 0.5 mg BD remitted these behavioral symptoms.
No birth complications achieved developmental milestones normally. At the age of 8 months, she sustained head injury due to a fall followed by loss of consciousness lasting for 8 days followed by full recovery in a month's time. Performance at school was average. Relations with peers were good. Schooling was discontinued in 5th standard at the age of 13 years after the onset of seizures.
Consanguinity was present, and no history of epilepsy or any psychiatric illness.
General physical examination
No significant findings.
Neurological examination showed no facial or body asymmetry, normal visual acuity, normal tone of the upper and lower limbs muscles with normal reflexes, and no cerebellar signs.
Mental status examination
Psychomotor activity, speech, thought, affect, and perception were normal. Insight was present.
Orientation, attention, concentration, and memory were intact. She has a poor understanding of similarities and differences and proverb interpretation.
Neuropsychological assessment: IQ score of 52.44 (mild intellectual disability disorder) on Binet Kamat Test of Intelligence (Indian adaptation of the Stanford–Binet test).
Hematological, blood biochemistry, complete urine examination, electrocardiography, and thyroid profile did not show any abnormalities. Video of electroencephalography was also administered while subjecting the patient to hand contact with ice-cold water and seizure activity could be recorded irrespective of contact with cold water [Video appended in supplement no: 1].
Magnetic resonance imaging (MRI) revealed a porencephalic cyst in the right parieto-occipital region of the brain parenchyma communicating with the dilated occipital horn of the ipsilateral lateral ventricle and cerebral hemi hypoplasia of right cerebral hemisphere with thickening of the adjacent calvarium. Furthermore, frontal, maxillary, and sphenoidal sinuses were widened.
Discussion: The absence of facial asymmetry and hemiparesis/hemiplegia in our case differentiates it from Dyke-Davidoff-Masson syndrome More Details (DDMS). MRI findings are similar to DDMS showing, cerebral hemiatrophy with compensatory ipsilateral calvarial thickening and hyperpneumatization of the paranasal sinuses and mastoid cells. Porencepahalic cyst is an addition in our case [Figure 1] and [Figure 2]. The condition is secondary to brain insult in utero or early childhood period. Other causes could be vascular, congenital or acquired ischemic disease, trauma, or inflammation.
|Figure 1: Magnetic resonance imaging axial view T2-weighted image showing Right hemisphere porencephalic cyst communicating with dialated lateral ventricle, ipsilateral cerebral atrophy. Note also calvarial thickening on the same side|
Click here to view
|Figure 2: Magnetic resonance imaging sagittal view T1 weighted showing dilatation of frontal, maxillary, and sphenoidal air sinuses. Notice porencephalic cyst communicating with dilated lateral ventricle and cerebral atrophy|
Click here to view
Decreased prefrontal gray or white matter, hippocampal and entorhinal cortices volume, or disturbed prefrontal metabolism and blood flow are strong indications that prefrontal cortical regions are associated with poor intellectual functions and psychosis. Larger the lesion size more it disrupts cortical mater.
[Table 1] summarizes cases from literature pertinent to the discussion of cerebral hemiatrophy, DDMS, and poencephalic cysts with neuropsychiatric complications.
|Table 1: Shows Porencephaly/cerebral hemiatrophy/ Davidoff-Dyke-Masson syndrome/with neuropsychiatric presentation|
Click here to view
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
We thank Dr. Siddappa Gourav, Medical Superintendent for giving total concession for MRI.
| References|| |
Hino-Fukuyo N, Togashi N, Takahashi R, Saito J, Inui T, Endo W, et al.
Neuroepidemiology of porencephaly, schizencephaly, and hydranencephaly in Miyagi prefecture, Japan. Pediatr Neurol 2016;54:39-42.e1.
Roopesh BN. Binet Kamat Test of Intelligence: Administration, Scoring and Interpretation – An In-Depth Appraisal .Indian Journal of Mental Health 2020;7:180-201.
Shen WH. MRI of porencephalic cyst associated with cerebralhemiatrophy. Eur Radio 1993;3:337-41.
Puri BK, Hall AD, Lewis SW. Cerebral hemiatrophy and schizophrenia. Br J Psychiatry 1994;165:403-5.
Reiter E, Heim B, Scherfler C, Mueller C, Nocker M, Ndayisaba JP, et al.
Clinical heterogeneity in cerebral hemiatrophy syndromes. Mov Disord Clin Pract 2016;3:382-8.
White JH, Rust JB. Davidoff-Dyke-Masson syndrome presenting as childhood schizophrenia. J Autism Dev Disord 1979;9:37-40.
Amann B, García de la Iglesia C, McKenna P, Pomarol-Clotet E, Sanchez-Guerra M, Orth M. et al.
Treatment-refractory schizoaffective disorder in a patient with Dyke-Davidoff-Masson syndrome. CNS Spectr 2009;14:36-9.
Hegde D, Guru N, Krishna Prasad M, Raghuraj U, Rao S. Psychosis in a patient with Davidoff-Dyke-Masson syndrome. Clin Schizophr Relat Psychoses 2018;12:9-11.
Kumari P, Mohsin H, Koola MM. Dyke-Davidoff-Masson syndrome presenting with bipolar I mania with psychosis. Indian J Psychiatry 2018;60:149-51.
] [Full text]
Sordia-Ramírez J, Infante-Valenzuela A, Hernández-Galarza IJ, Costilla-Esquivel A. Neuropsychiatric symptoms in a patient with Dyke-Davidoff-Masson syndrome and systemic lupus erythematosus: A case report. J Med Case Rep 2019;13:111.
Douzenis A, Rizos EN, Papadopoulou A, Papathanasiou M, Lykouras L. Porencephaly and psychosis: A case report and review of the literature. BMC Psychiatry 2010;10:19.
Boyer L, Dassa D, Lancon C. A case of porencephalic cyst in a schizophrenic patient with history of postnatal encephalitis. Prog Neuropsychopharmacol Biol Psychiatry 2011;35:288-9.
Hussain T, Ahmed BJ, Shoib S, Shafat M, Mushtaq R, Altaf AM. Psychosis in a patient with porencephaly – A case report. J Pioneer Med Sci 2015;5:26-8.
Noyan OC, Şalçini C, Talu BS, Eryilmaz G. Porencephalic cyst and late onset brief psychotic disorder. BMJ Case Rep 2016;2016:bcr2016215098.
de Oliveira-Souza R, Monteiro M, Pacheco P, Tovar-Moll F, Mattos P, Moll J, et al.
Right hemisphere dominance for language in a woman with schizophrenia and a porencephalic cyst of the left hemisphere. Neurocase 2016;22:324-31.
Uvais NA, Sayantanava M. “I do not know them” – Capgras syndrome associated with porencephaly and agenesis of the splenium. Asian J Psychiatr 2020;54:10228954.
Department of Psychiatry, Malla Reddy Institute of Medical Sciences, Hyderabad, Telangana
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]
| Article Access Statistics|
| Viewed||745 |
| PDF Downloaded||23 |