ABSTRACT
We report the case of a 14-year-old boy with bilateral congenital perisylvian syndrome as evidenced by polymicrogyria in the left and right frontotemporal regions, pseudobulbar paresis, seizures, and spastic diplegia. This is the first description of comprehensive neuropsychological assessment in a child with this syndrome. The results reflect significant impairments in auditory processing and independent learning and retention, but relatively preserved cognitive performance on interactive tasks involving frequent feedback and redirection. The findings are interpreted in the context of the neuropathology of this syndrome, with an emphasis on pragmatic implications for rehabilitation.
Subject(s)
Abnormalities, Multiple/diagnosis , Cerebral Cortex/pathology , Cognition , Malformations of Cortical Development/pathology , Nervous System Malformations/diagnosis , Abnormalities, Multiple/pathology , Abnormalities, Multiple/physiopathology , Adolescent , Cerebral Cortex/abnormalities , Cerebral Cortex/physiopathology , Functional Laterality , Humans , Magnetic Resonance Imaging , Male , Malformations of Cortical Development/diagnosis , Nervous System Malformations/pathology , Nervous System Malformations/physiopathology , Neuropsychological Tests , Pseudobulbar Palsy/congenital , Pseudobulbar Palsy/diagnosis , SyndromeABSTRACT
Congenital bilateral perisylvian syndrome (CBPS) is rare in literature, especially in China. In this article, we report the clinical and treatment of a patient with CBPS and discuss its mechanism, clinical features and therapy. This patient was a 28-year-old man. His main clinical features were pseudobulbar palsy, cognitive deficits and intractable epilepsy. MRI shows bilateral thickening of the cortex around the sylvian fissures which were deeper than normal and polymicrogyria. The electroencephalogram demonstrated slow spike in right temporal lobe and left frontal lobe. Rhythmal 4 Hz theta waves exist in left frontal and parietal lobe. As the epilepsy was poorly controlled by antiepileptic, section of the corpus callosum was carried out. After callosotomy, there was pronounced seizure reduction and intelligence development improvement. CBPS is characterized by pseudobulbar palsy, cognitive deficits, and bilateral perisylvian abnormalities in imaging studies. If intractable epilepsy is combined, callosotomy may be effective.
Subject(s)
Epilepsy , Intellectual Disability , Paresis , Pseudobulbar Palsy , Adult , Electroencephalography , Epilepsy/congenital , Epilepsy/diagnosis , Humans , Intellectual Disability/diagnosis , Magnetic Resonance Imaging , Male , Paresis/congenital , Paresis/diagnosis , Pseudobulbar Palsy/congenital , Pseudobulbar Palsy/diagnosis , SyndromeABSTRACT
In the congenital bilateral perisylvian syndrome, pseudobulbar symptoms, cognitive deficits and cortical malformations in the perisylvian region are typical features. We report two initially magnetic resonance imaging (MRI) negative patients from our epilepsy surgery program that shared the same seizure and ictal SPECT characteristics suggesting seizure onset localized in the perisylvian region. In one patient, reevaluation revealed perisylvian cortical malformation on MRI while in the other patient MRI was normal. In these patients, subtraction ictal SPECT coregistered with MRI (SISCOM) proved useful together with clinical data in diagnosing the patients with mild forms of perisylvian syndrome.
Subject(s)
Cerebral Cortex/abnormalities , Cerebral Cortex/diagnostic imaging , Cognition Disorders/diagnostic imaging , Pseudobulbar Palsy/congenital , Pseudobulbar Palsy/diagnostic imaging , Adult , Cognition Disorders/pathology , Epilepsy/diagnostic imaging , Epilepsy/pathology , Humans , Magnetic Resonance Imaging , Male , Syndrome , Tomography, Emission-Computed, Single-PhotonSubject(s)
Brain/abnormalities , Chromosomal Proteins, Non-Histone , Epilepsy , Pseudobulbar Palsy , Repressor Proteins , Child , DNA-Binding Proteins/genetics , Diagnosis, Differential , Diagnostic Imaging , Dysarthria/etiology , Epilepsy/etiology , Female , Humans , Methyl-CpG-Binding Protein 2 , Prognosis , Pseudobulbar Palsy/congenital , SyndromeSubject(s)
Cognition Disorders/diagnosis , Dysarthria/diagnosis , Paresis/congenital , Paresis/diagnosis , Pseudobulbar Palsy/congenital , Pseudobulbar Palsy/diagnosis , Adolescent , Bulbar Palsy, Progressive/classification , Bulbar Palsy, Progressive/diagnosis , Child , Child, Preschool , Cognition Disorders/complications , Cranial Nerve Diseases/complications , Cranial Nerve Diseases/diagnosis , Diagnosis, Differential , Dysarthria/complications , Female , Humans , Magnetic Resonance Imaging , Male , Paresis/classification , Pseudobulbar Palsy/complications , Seizures/etiology , Syndrome , Terminology as TopicABSTRACT
We report a 17-year-old female patient with a variant form of congenital bilateral perisylvian syndrome (CBPS). She had pseudobulbar palsy, partial epilepsy and mild pyramidal symptoms predominantly in the left hand. Magnetic resonance imaging revealed asymmetric perisylvian and perirolandic polymicrogyric cortical dysplasia and septum pellucidum defect. The clinicoradiological findings for this patient met the criteria for CBPS. Moreover, they appeared to overlap those of congenital unilateral perisylvian syndrome. The findings in this case support the hypothesis that these two syndromes are parts of a continuous spectrum of one clinico-radiological syndrome.
