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Reversible Parkinsonism and Pisa Syndrome in Juvenile Normal Pressure Hydrocephalus

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Authors:  Sanjay Pandey MD, DM

Article first published online:   30 DEC 2014 | DOI: 10.1002/mdc3.12121


Gait disorders and parkinsonism (akinetic-rigid syndrome) have been described in the setting of idiopathic normal pressure hydrocephalus (iNPH), which is usually considered to be a disorder of the elderly and adult population.[1, 2] However, there are reports of occurrence of this disorder in pediatric patients as well, described as a juvenile variant of NPH.[3, 4]

Pisa syndrome (PS) is characterized by tonic flexion of the trunk and head to one side with slight rotation of the body and is mainly observed in patients on antipsychotic drugs. This syndrome is also described in Alzheimer's disease, Lewy body dementia, Parkinson's disease, and secondary parkinsonism, such as multisystem atrophy.[5]

I report on a 17-year-old boy having symptoms suggestive of parkinsonism and PS. His MRI of the brain was showing chronic hydrocephalus, and symptoms improved completely following ventriculo peritoneal shunt surgery.

 

Case Report

A 17-year-old boy developed progressive slowness of body movements for 2 years and leaning toward the right side for 1 year. He had increased anger for 6 months, but there was no cognitive decline or urinary incontinence. There was no family history of parkinsonism or dystonia. A trial of levodopa (100 mg)/carbidopa (25 mg; maximum dosage: 1,050 mg) was given for 4 weeks, but he did not improve. There was no history of taking neuroleptic medications or dopamine agonists.

On clinical examination, his horizontal and vertical saccadic eye movements were very slow. His vision and fundus examination was normal. On slit-lamp examination, there was no evidence of Kayser-Fleischer ring. Speech had loss of modulation, and most sentences were difficult to understand. Detailed cognitive assessment was normal. He had masked facies and decreased eye-blink frequency. All four limbs had grade 2 rigidity (UPDRS-III). Finger tapping, hand movements, and toe tapping were very slow. He had postural tremor in the right hand. On standing erect and while walking, he had “Pisa syndrome or pleurothotonous” characterized by leaning toward the right side as a result of lateral flexion and trunk rotation (see Video 1). An investigation for Wilson's disease was within normal limits (serum copper: 8 μg/dL; serum ceruloplasmin: 58 mg/dL; 24-hour urine copper: 22 μg/day).

His MRI of brain was suggestive of chronic hydrocephalus (Fig. 1A,B). A diagnosis of juvenile NPH leading to parkinsonism with PS was made. A cerebral spinal fluid (CSF) tap drainage test was done, and 30 mL of CSF were drained. The opening pressure was normal (125 mm of H2O). CSF glucose was 60 mg/dL (blood sugar: 102 mg/dL), protein was 27 mg/dL, and cell counts were nil. After CSF tap, he had significant improvement in gait, which lasted for only 3 days. Observing the temporary improvement after removal of CSF, a ventriculo peritoneal shunt surgery was done through a right occipital burr hole. During the first week after surgery, he started improving, and after 2 months, his lateral flexion and trunk rotation on the right side completely disappeared. His gait became normal (see Video 1). His speech was clear and rigidity markedly reduced. At 6-month follow-up, he had rejoined his school.

Preoperative MRI of brain (fluid attenuation inversion recovery sequence axial [A] and T2 coronal [B]) and CT scans of the head Figure 1. Preoperative MRI of brain (fluid attenuation inversion recovery sequence axial [A] and T2 coronal [B]) and CT scans of the head (C) are suggestive of chronic hydrocephalus. There is mild asymmetry in the ventricular dilatation (left > right). Patient CT scans of the head 2 days after surgery show a ventriculoperitoneal shunt in the frontal horn of the lateral ventricle (D). R, right; L, left.

 

 

 

Discussion

Parkinsonism with PS in a child with no previous history of neuroleptic exposure and its complete reversal after ventricular peritoneal surgery has never been reported on in the literature. A case report describing reversible PS in juvenile NPH is available on literature search, but shunt surgery was not done in this patient and follow-up is not available.[3] iNPH has been classified as probable, possible, and unlikely based on clinical history, brain imaging, physical findings and physiological criteria.[2] This patient fulfills all the criteria of probable iNPH except age. Parkinsonism is commonly observed in patients of NPH.[1] In a study, 71% patients of definite iNPH had parkinsonism.[6] In another study, 68% patients of adult hydrocephalus had movement disorders, including parkinsonism.[7] A published criterion for the suspected NPH includes parkinsonism refractory to medical treatment.[8] Our patient also received a trial of l-dopa/carbidopa (maximum dosage: 1,050 mg) for 1 month, but he did not show any improvement. The pathophysiology of parkinsonism secondary to hydrocephalus involves dysfunction in the cortico-striato-pallido-thalamo-cortical circuit and nigrostriatal pathway.[1, 4]Curran and Lang proposed that there may be additional disconnection between basal ganglia and the supplementary motor area.[1]Irrespective of the mechanism of the dysfunction, not every patient responds favorably to the shunting procedure.[6] There are only few reports of NPH in children and adolescents. In a study, 16 patients less than 20 years of age had NPH and the most common clinical manifestations were psychotic behavior and gait problem.[4] Eleven patients had an underlying etiological factor for NPH.

Our patient also had PS with no previous exposure to neuroleptics given that he was leaning toward the right side while walking. There was mild asymmetry in the ventricular dilatation (left > right) on MRI. Alteration in striatal postsynaptic dopaminergic function explains the pathophysiology of parkinsonism in iNPH.[9] Asymmetrical and uneven enlargement of the ventricle in our patient is likely to cause asymmetrical imbalance in the dopaminergic-cholinergic level, leading to PS in this patient.[9, 10] After shunt intervention, his symptom improved, which suggests the reversible nature of the underlying pathogenesis.

Conclusion

Parkinsonism may be a feature of NPH, which may also manifest in children and adolescents. Early postural deformity and poor l-dopa response may provide important clues. In this case report, a patient with parkinsonism and PS was found to be the result of NPH, which should be considered given that it is therapeutically rewarding.

 

 

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