Foix-Chavany-Marie opercular syndrome is a severe form of
pseudobulbar palsy caused by bilateral anterior opercular lesions (1,2).
Five patients suffered hypoxic ischaemic brain injury, two had left ventricular hypertrophy, one developed
pseudobulbar palsy, and the other suffered renal failure.
Dysphagia, sometimes as part of
pseudobulbar palsy, is one of the most common complications of stroke among patients.
It is an acute demyelinating condition primarily affecting central pons and commonly presenting clinically with spastic quadriparesis, dysarthria,
pseudobulbar palsy, and altered mental status.
Box From Darwin to IEED, nomenclature has evolved over time Charles Darwin (5) was among the first to acknowledge the correlation between neurologic insult and dysregulated emotional expression, writing in 1872 that "certain brain disease, such as hemiplegia, brain-wasting, and senile decay, have a special tendency to induce weeping." In 1886, Oppenheim and Siemerling (6) proposed the terms "pseudobulbar affect" (PBA) and "
pseudobulbar palsy" (a separate and distinct state that includes dyscontrol of facial muscles, resulting in dysarthria, dysphagia, and dysphonia) in their descriptions of patients with bilateral forebrain injury that appeared to mimic brainstem dysfunction.
In severe cases, there is spastic quadriparesis and
pseudobulbar palsy. This may evolve within 3-10 days into a locked-in-state (pseudo coma).
Examination showed the following striking findings: non-fatigable ophthalmoplegia and bilateral ptosis, and severe
pseudobulbar palsy with a profoundly brisk jaw (and head) jerk.
Twenty-four hours post podophyllin ingestion, the child's GCS remained 5/15; however, he had now developed abnormal neurological signs, including hypotonia, areflexia with no clonus and reduced power in all limbs, but with no evidence of a
pseudobulbar palsy. He had no signs of autonomic dysfunction; however, he did show deficits in sensation examination with loss of pain and touch.
Olszewsk, "Progressive Supranuclear Palsy, a heterogeneous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and
pseudobulbar palsy, nuchal dystonia and dementia," Archives of Neurology, vol.
The patients were having normal ICP with no pseudomeningocele formation and
pseudobulbar palsy except CSF leak, observed in 3 patients.
Most patients have a family history of this disease, and it is clinically characterized by recurrent subcortical ischemic strokes, sometimes leading to
pseudobulbar palsy and dementia.
Neurologic disorders such as Arnold-Chiari malformation, myotonic dystrophy,
pseudobulbar palsy, pharyngeal paresis, myasthenia gravis, and neurofibromatosis
