Putting pontine anatomy into clinical practice: the 16 syndrome.

The anatomical localisation of brainstem syndromes is the domain of the clinical neurologist, though MRI has made an encyclopaedic knowledge of neuroanatomy less crucial. Isolated pontine syndromes comprise ∼20% of the brainstem lacunar syndromes. Typical presentations such as pure motor hemiparesis and ataxic hemiparesis are easily recognisable but atypical syndromes, particularly when bilateral, may present with puzzling signs. We discuss a patient with an unusual acute bilateral brainstem syndrome, in whom MRI was contraindicated. We use the relevant neuroanatomy to support the likely diagnosis of bilateral caudal pontine tegmentum infarction due to occlusion of a single paramedian pontine tegmental perforating artery.

Cerebral amyloid angiopathy and motor neurone disease presenting with a progressive supranuclear palsy-like syndrome.

We describe a 68-year-old woman who presented with falls, mild limb bradykinesia, axial rigidity, and a severe supranuclear gaze palsy, which failed to benefit from levodopa. She subsequently developed severe apraxia, progressive dysarthria, dysphagia, and a frontal cognitive impairment. Pyramidal weakness with fasciculations and widespread chronic partial denervation appeared shortly before her death from bronchopneumonia, 6 months after disease onset. A severe cerebral amyloid angiopathy diffusely involving the cerebral hemispheres and cerebellum was present at autopsy as well as a second pathological condition indicative of motor neurone disease. Cerebral amyloid angiopathy may rarely present with a progressive supranuclear palsy-like phenotype.