Reversible Pisa syndrome associated to subdural haematoma: case-report

Abstract

Background: Pisa Syndrome or Pleurothotonus is a relatively rare truncal dystonia, characterized by tonic flexion of the trunk and head to one side with slight rotation of the body. Since frequently associated to specific drugs such as antipsychotics and cholinesterase inhibitors or to Parkinson Disease, a pathophysiological role of cholinergic-dopaminergic imbalance has been suggested. We report here the first case of Pisa Syndrome due to an extracerebral pathology as subdural haematoma.

Case presentation: A hypertensive patient was admitted to Our Department for subacute onset of tonic flexion and slight rotation of the trunk associated to progressive motor deficit in left upper limb after a mild head trauma without loss of consciousness occurred around three month before. No previous or current pharmacological interventions with antidepressant, neuroleptic or anticholinergic drugs were anamnestically retrieved. Familiar and personal history was negative for neurological disorders other than acute cerebrovascular diseases. Acutely performed cerebral MRI with DWI showed a voluminous right subdural haematoma with mild shift of median line. After surgical evacuation, both motor deficit and truncal dystonia were dramatically resolved. At one-year follow up, the patient did not develop any extrapyramidal and cognitive signs or symptoms.

Conclusions: According to many Authors, the occurrence of truncal dystonia during several pharmacologic treatments and neurodegenerative disorders (such as Alzheimer disease and parkinsonian syndromes) supported the hypothesis that a complex dysregulation of multiple neurotransmitter systems are involved. We suggest a possible role of basal ganglia compression in pathogenesis of truncal dystonia by means of thalamo-cortical trait functional disruption and loss of proprioceptive integration. A further contribution of the subcortical structure displacement that alters motor cortex connectivity to basal ganglia may be postulated.

Figure 1

Patient in standing position before intervention.cph. A slight but sustained tonic flexion of the trunk to the left side was present in standing position with ipsilateral forward rotation. Vertical axis highlights the left lateral skew of the trunk. Horizontal line reflects the forward rotary motion of the left shoulder.

Figure 2

Axial T2-weighted slice of cerebral MRI. A voluminous right frontal, temporal and parietal subdural haematoma with a diameter of 28 mm was present with ipsilateral ventricle compression and an 8 mm shift of median line. A minimal amount of haematic shedding (3 mm diameter) was also present in left frontal subdural space. Some periventricular hyperintense areas were observed as probable expression of microvascular pathology.

Figure 3

Patient in standing position after intervention. Four days after haematoma evacuation, both tonic trunk flexion and weakness in left upper limb dramatically improved until complete resolution. The patient maintained adequate standing position and did not swerve to the left during walking.