1/12. Bilateral opercular syndrome caused by perinatal difficulties. Four patients with pseudobulbar palsy, mental retardation and various degrees of speech disturbance associated with perinatal difficulties are described as having an acquired type of opercular syndrome. There were two patients with fetal bradycardia and three with subarachnoid haemorrhage and neonatal convulsion. magnetic resonance imaging revealed cortical atrophy in the bilateral opercula with some signal abnormalities in the underlying white matter in common. Single photon emission computed tomography (SPECT) also confirmed the presence of hypoperfusion in the regions. Although the opercular syndrome is a clinical entity with a multitude of underlying pathologies, perinatal difficulties could be an important cause of the acquired type. ( info) |
2/12. A variant case of congenital bilateral perisylvian syndrome with asymmetric findings on neuroimaging and septum pellucidum defect. 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. ( info) |
3/12. Foix-Chavany-Marie syndrome. Anarthria and bilateral central faciolinguopharyngeomasticatory paralysis with automatic voluntary dissociation are the clinical hallmarks of Foix-Chavany-Marie syndrome (FCMS), the cortico-subcortial type of suprabulbar palsy. We present a 64-year-old man who was admitted due to acute onset of speechlessness for days. Neurological examinations demonstrated typical features of FCMS. Cranial MRI showed a right opercular lesion and several lacunar infarcts in bilateral hemispheres. Hexamethylpropylenamine oxime (HMPAO) brain SPECT revealed hypoperfusion in bilateral opercular regions and notably on the right side. In review of previous reports, the most common etiology of FCMS is stroke in the region of either operculum. Our case may be a good example of FCMS after multiple cerebrovascular insults over bilateral opercular cortico-subcortical areas as demonstrated in MRI and SPECT studies. ( info) |
| Antiphospholipid antibody syndrome (APS) is now recognized as an important risk factor for young stroke. Recurrent stroke seems to be common in patients with APS and a first stroke, recurrent stroke and vascular dementia are feared consequences of APS. We are reporting a case of primary APS with recurrent stroke presenting as pseudobulbar palsy in an young man. ( info) |
5/12. Treatment of pseudobulbar laughter after gamma knife thalamotomy. We describe a case of pathological laughter after gamma knife thalamotomy which resolved after treatment with sertraline. It is important to identify this potentially treatable complication of surgical therapy. ( info) |
| A case of left atrial (LA) myxoma presenting as pseudobulbar palsy, due to multiple cerebral infarcts, without any cardiac manifestations, is presented. LA myxoma is rare cause of embolization to CNS causing ischemic infarcts. Due to multiple CNS infarcts patient can present with varied clinical picture and pseudobulbar palsy is not a very common presentation. It was a real diagnostic dilemma before LA myxoma was diagnosed on echocardiography. ( info) |
7/12. The first nonsense mutation in alsin results in a homogeneous phenotype of infantile-onset ascending spastic paralysis with bulbar involvement in two siblings. Eight mutations in the ALS2 gene have been described as causing autosomal-recessive juvenile-onset forms of the motor neuron diseases amyotrophic lateral sclerosis, primary lateral sclerosis and hereditary spastic paraplegia. All mutations are small deletions that are predicted to result in a frameshift and premature truncation of the alsin protein. Here we describe a ninth ALS2 mutation, in two siblings affected by infantile-onset ascending spastic paraplegia with bulbar involvement. This mutation is predicted to result in the substitution of an amino acid by a stop codon, and thus is the first nonsense mutation detected in this gene. It is probable that full-length alsin is required for the proper development and/or functioning of upper motor neurons. ( info) |
8/12. Ictal SPECT in clinical perisylvian syndrome. 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. ( info) |
9/12. Pseudobulbar crying induced by stimulation in the region of the subthalamic nucleus. We describe a case of pseudobulbar crying associated with deep brain stimulation (DBS) in the region of the subthalamic nucleus (STN). patients with pseudobulbar crying show no other evidence of subjective feelings of depression such as dysphoria, anhedonia, or vegetative signs. This may be accompanied by other symptoms of pseudobulbar palsy and has been reported to occur with ischaemic or structural lesions in both cortical and subcortical regions of the brain. Although depression has been observed to result from DBS in the region of the STN, pseudobulbar crying has not been reported. A single patient who reported the symptoms of pseudobulbar crying after placement of an STN DBS was tested in the off DBS and on DBS conditions. The patient was tested using all four DBS lead contacts and the observations and results of the examiners were recorded. The Geriatric depression Scale was used to evaluate for depression in all of the conditions. The patient exhibited pseudobulbar crying when on monopolar stimulation at all four lead contacts. The pseudobulbar crying resolved off stimulation. This case describes another type of affective change that may be associated with stimulation in the region of or within the STN. Clinicians should be aware of this potential complication, the importance of differentiating it from stimulation induced depression, and its response to a serotonin reuptake inhibitor, such as sertraline. ( info) |
10/12. Congenital bilateral perisylvian syndrome with partial epilepsy. Case report with long-term follow-up. Congenital bilateral perisylvian syndrome (CBPS) is a rare neurological disorder characterised by pseudobulbar palsy, cognitive deficits and epilepsy associated with bilateral perisylvian cortical dysplasia on neuroimaging studies. We report a long-term follow-up of a 18-years girl diagnosed with CBPS according to the typical clinical and magnetic resonance imaging (MRI) features. The patient showed faciopharyngoglossomasticatory diplegia, severe dysarthria, ataxia, spastic quadriparesis and severe mental retardation. Brain MRI evidenced bilateral perisylvian cortical dysplasia. Since early life she suffered from complex febrile seizures and epilepsy consisting of complex partial attacks with affective manifestations associated with centro-temporal EEG abnormalities. During 18 years of follow-up she was treated with phenobarbital, carbamazepine, lamotrigine, gabapentin but did not show any significant clinical improvement. Subsequently, monotherapy with phenytoin (PHT) was followed by a significant clinical improvement. At age 17, because of adverse effects, PHT was gradually substituted by topiramate (TPM). Full control of seizures was obtained at the age of 17 years with TPM. EEG abnormalities throughout the years have been reduced according to the clinical course. These findings emphasised the importance of long-term follow-up, suggesting that the prognosis for epilepsy may not be predicted based on the early response to treatment or on the presence of structural encephalic abnormalities, as reported in the literature. ( info) |