Rare case of pseudobulbar palsy: subopercular syndrome.

We present a case of a 73-year-old man who developed sudden onset dysarthria, dysphagia and bilateral facial weakness with automato-voluntary dissociation, which deteriorated rapidly to anarthria and aphonia within a few days. MRI scan of the head showed acute infarct in right internal capsule and an old infarct in the left corona radiata while the rest of the investigations were normal. Based on these findings, diagnosis was thought to be subopercular syndrome. He recovered significantly in a few weeks' time.

Pseudobulbar Affect in Survivors of Extreme Prematurity With Cerebellar Injury: Support for the Cerebellar Link in Pathologic Laughter and Crying.

Pseudobulbar affect, that is, pathologic laughter and crying is being increasingly recognized in adults and is seen in association with a number of diseases like Parkinson disease, dementia, traumatic encephalopathy, and others, but has not previously been described in children with cerebral palsy. The condition pseudobulbar affect may be due to lesions in (or degeneration of) the cerebro-ponto-cerebellar pathways. Here we report 2 children with cerebral palsy who have structural cerebellar injury because of their being born extremely premature who have pathologic crying and probably laughter.

[TRANSIENT PSEUDOBULBAR SYNDROME IN UNILATERAL FRONTAL OPERCULAR INFARCTS]. / ÁTMENETI PSEUDOBULBARIS TÜNETCSOPORT EGYOLDALI FRONTÁLIS OPERCULARIS INFARKTUSOKNÁL.

The classic anterior (frontal) opercular syndrome (Foix-Chavany-Marie sy.) is a cortical pseudobulbar palsy mainly due to bilateral lesions of anterior brain operculum. In 2000 the authors had a 70-year old female patient with acute onset of swallowing and speaking difficulty. Neurological examination established a left facial central palsy, the palsy of the tongue and the soft palate, dysarthry, difficulty in chewing with left side hemiparesis. The CT scan showed a right side (one-sided) frontal opercular ischemic lesion. This event switched their attention especially to this group of cases and subsequently the authors collected 12 patients with these symptoms. Authors discuss the patomechanism of transient pseudobulbar palsy that occurs due to unilateral opercular lesion that the diaschisis effect might explain.

Case report of transient acquired Foix-Chavany-Marie syndrome following sequential trauma.

Foix-Chavany-Marie Syndrome (FCMS) or bilateral opercular syndrome is characterised by faciopharyngoglossomasticatory diplegia with automatic voluntary dissociation and is a rare form of pseudobulbar palsy most commonly associated with occlusive cerebrovascular disease. We present a transient manifestation of the syndrome, in a patient who suffered two sequential traumatic brain injuries.

Familial and genetic associations in Worster-Drought syndrome and perisylvian disorders.

Worster-Drought syndrome (WDS) is a distinct clinical phenotype, comprising a congenital pseudobulbar palsy usually in association with a mild tetraplegia and often additional impairments. The phenotype is identical to that described in congenital bilateral perisylvian polymicrogyria syndrome (CBPS) and appears to have several different causes and a significant familial incidence. This study draws from a database of children with WDS phenotype or perisylvian polymicrogyria, held at a tertiary center. The findings suggest that genetic factors are important for a significant proportion of children and points to considerable genetic heterogeneity. There are grounds for considering WDS and perisylvian polymicrogyria as a spectrum of perisylvian malfunction.

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.

Primary antiphospholipid syndrome presenting as pseudobulbar palsy in a young male.

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.