Pisa syndrome

Definition of Pisa syndrome
Pisa syndrome: A condition in which there is sustained involuntary flexion of the body and head to one side and slight rotation of the trunk so the person appears to lean like the Leaning Tower of Pisa.

The Pisa syndrome is an adverse effect (a side-effect) of some medications. It occurs sometimes following the long-term use of narcoleptics (drugs used to treat schizophrenia) or cholinesterase inhibitors (a class of drugs used to treat alzheimer disease).

History
Pisa syndrome was discovered by Karl Axel Ekbom, a Swedish neurologist, in the early 1970s. Cases of the syndrome were first observed in three elderly female patients suffering from presenile dementia. Each of these women were undergoing treatment with the antipsychotic drug methylperone, haloperidol or a combination of the two. The use of neuroleptic drugs caused the patients to exhibit a lateral flexion along with a rotation of the trunk. As the patients walked they experienced an increase in rotation. The postural and gait disturbances symptoms is what set this apart from any other form of acute dystonia previously observed. These symptoms proved to be the making of a new dystonic reaction, which was termed pleurothotonus or Pisa syndrome.The first patient, a 59-year-old woman with no family history of neuroleptic disease, was put through two periods of treatment with methylperone. The first trial of the drug was administered in February 1971. In the beginning the patient demonstrated no symptoms of dystonia. However, within the first few days the patient began to exhibit a tilting to right upon walking. The women was then taken off the methylperone treatment and as a result progressively regressed back to her previous state of exhibiting no symptoms within the first two months. The patient started a second trial of methylperone treatment in late October 1971. After a little over a week of the drug treatment, she began to express previous symptoms that including a bending of the trunk towards the right along with a rotation. The patient also experienced a pulling away from her direction of walking and a difficulty of turning. Within a couple of days of exhibiting symptoms, the patient was then treated with orphenadrine. This treatment helped regress the expressed symptoms quicker than the first time. By the end of the week the patient was able to return to her normal state.

The second patient to undergo methylperone treatment was a 63-year-old woman with presenile dementia, which caused her to experience restlessness and paranoid hallucinations. The methylperone treatment was able to alleviate the woman’s problems induced by her dementia. It did not take long for the woman to begin to experience symptoms of Pisa syndrome and as a result she was taken off of the methylperone treatment. Like the first patient, she was able to overcome the induced symptoms of Pisa syndrome within a month. The patient was again treated with methylperone after two months from the first treatment. Soon after the patient began to lean toward the right when standing or walking. She was then administered orphenadrine, which soon stopped the patient’s tilting posture.

The final patient was a 69-year-old woman, diagnosed with presenile dementia after she expressing symptoms of memory dysfunction, depression and urinary incontinence. As a result the woman was put under a methylperone treatment, which soon caused a bending and rotation to her left. These symptoms disappeared soon after being taken off of methylperone. Unlike the other two patients, when the woman was again administered methylperone she did not exhibit any previous dystonic symptoms after two weeks of treatment. The patient was then switched to a small dosage of haloperidol along with the typical dosage of orphenadrine used on the previous patients. Instead of not having symptoms of Pisa syndrome, the woman began to experience a leaning to her left side and a particular rotation of her shoulder towards the left. Once haloperidol was eliminated from the treatment the patient no longer had these symptoms. As more cases of the syndrome came about, research discovered that the switching of drug treatments can be debated as a possible inducer of the disease. Other cases of the disease have been proven to be caused by medications other than neuroleptic drugs. These patients were observed exhibiting symptoms of Pisa syndrome as a result of having a prior neurodegenerative disease.

Symptoms

The predominant symptom of Pisa syndrome is dystonia. Dystonia is a neurological movement disorder characterized by sustained muscle contraction leading to abnormal posture, twisting, and repetitive movement. In Pisa Syndrome specifically there is commonly a tonic flexion of the trunk of the body to one side, leading to a slight lean (reminiscent of the leaning tower of pisa, hence the name "Pisa syndrome"). This is usually associated with a backward axial, rotation of the spine and indifferent to markedly abnormal posture. Patients diagnosed with Pisa Syndrome usually experience either acute dystonia or tardive dystonia, also known as tardive dyskinesia. Differential diagnosis between the two may be hard to accomplish without a complete patient history, since both types of dystonia may occur simultaneously in a patient. These symptoms generally disappear after discontinuation of the antipsychotic drug. The time of onset of symptoms may vary depending on drug being administered and the neurological characteristics of the patient in question.