Yenta, was the word she used to describe her brother in law. He has a lot wrong with him; the high blood pressure was discovered when he was in his thirties. Diabetes; he uses an insulin pump, and has a neuropathy in the left leg and foot, as a consequence of the illness. He underwent cardiac ablation, had a triple by pass surgery to his heart, and wears a pacemaker. The pacemaker for his heart makes an MRI of the brain impossible. He is sometimes incontinent, has difficulty rising from a chair, and has fallen and injured his right shoulder; doctors think he has a torn labrum and will need surgery. The toes of his left foot curl up in dystonic spasms he cannot control. Some time ago, he worked as an architect, and he retains the ability to draw well, though his handwriting has succumbed to illness.
They come for another opinion of what they can do for their family member. The patient has had speech and physical therapy. The specialist reads the notes from other physicians and the differential diagnosis; the list of possible diagnoses the patient may suffer from. Over the course of several months, the wife has seen her spouse decline in function. She contends he has lost a lot of drive. He was a Type- A personality and now lacks the motivation for common things. His personality has become more emotional, and he admits he cries easily. The physician listening comments to the medical student sitting next to him that it sounds as though the frontal lobes are affected, as the area on both sides of the brain, dampens emotional expression.
The specialist performs the physical examination and notes the patient’s eye movements are full. The women mention the patient usually has his eyes closed, and frequently walks into objects when using his walker. This makes some sense to the specialist, as other neurologists have noted he may suffer from Supranuclear Palsy, which usually results in the patient having difficulty looking downwards, though this is not his problem right now. Botox injections to the muscles of the foot helped relieve the uncontrollable spasms on the right. When given to the muscles around the orbit of the eyes, they have been less effective.
The specialist is concerned the patient may suffer from normal pressure hydrocephalus, which can mimic vascular, or lower body parkinsonism. He requests a CT of the brain, which will show whether the ventricles are enlarged. If so, a neurosurgeon can place a shunt in the brain, allowing the excess cerebral spinal fluid to drain out of the brain and into the body.
The doctor recommends the family have a consultation with a fellow neurologist trying to assemble a group of patients for a study on progressive supranuclear palsy. The colleague intends to do a drug study to discover whether a certain medication is useful in that population. At the very least, they will get another opinion from a movement disorder specialist, who will have the results of the CT of the brain to possibly to rule out normal pressure hydrocephalus.


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