Hydrocephalic parkinsonism due to Paget's disease of bone: Dramatic improvement following ventriculoperitoneal shunt and temporary levodopa/carbidopa therapy
✍ Scribed by Ken Ikeda; Masao Kinoshita; Kazuya Aoki; Atsushi Tomatsuri
- Publisher
- John Wiley and Sons
- Year
- 1997
- Tongue
- English
- Weight
- 355 KB
- Volume
- 12
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
s disease of bone has many neurological symptoms (1). Basilar impression is also associated with this disease and may result in hydrocephalus. Hydrocephalus due to Paget's disease of bone manifests in the form of dementia, ataxic gait, or urinary incontinence; these symptoms resemble those of normal pressure hydrocephalus (211). Hydrocephalic parkinsonism rarely accompanies this disease (4). Here we report the case of a patient with drug-resistant parkinsonism due to Paget's disease of bone, who underwent successful treatment with a ventriculoperitoneal (V-P) shunt and subsequent temporary levodopa/carbidopa therapy.
A 69-year-old woman showed signs of a slow gait with small steps in April 1985. She noticed bilateral hearing loss in September 1988. Her gait disturbance gradually progressed. Bradykinesia in the arms and slurred speech also appeared in 1992. On May 30, 1994, she was admitted to our department for evaluation of her gait difficulty. There was no history of alcohol abuse or toxic exposure or a family history of Parkinson's disease. The general physical examination was normal except for a short neck. Neurological examination showed right facial hemispasm, bilateral deafness, and dysarthria. She had severe bradykinesia and rigidity in the extremities, predominantly in the legs. Her gait was very slow with small steps; she manifested initial hesitation even when she was supported. Involuntary movements were absent. Muscle stretch reflexes were mildly brisk without Babinski signs. The remainder of her neurological examination, including cognitive, cerebellar, and bladder functions, was normal.
Laboratory studies showed an elevation of the serum alkaline phosphatase at 1,812 IU (normal, <219 IU). The initial pressure of the cerebrospinal fluid was 250 mm H,O, but analysis of the fluid found normal results. Skull radiography found the characteristic cotton wool appearance of Paget's disease, thickening, and a basilar impression of the skull. Cranial magnetic resonance imaging (MRI) and computed tomography (CT) scan showed ventricular dilatation of the lateral ventricles (Fig. 1A) with narrowing of the fourth ventricle. There were no abnormalities in the basal ganglia and periventricular areas on MRI. Single photon emission computed tomography (SPECT) showed diffuse hypoperfusion in the cerebral cortex. A cisternogram disclosed obstructive hydrocephalus. Lumbar MRI showed no abnormalities. Histology of the bone biopsy showed evidence of Paget's disease. We made a diagnosis of noncommunicating obstructive hydrocephalus due to Paget's disease of bone.
Daily intramuscular calcitonin, intravenous pamidronate (Aredia; Ciba Geigy Japan Limited) and oral levodopa/ carbidopa (600/60 mg) treatment over the course of 1 month failed to lead to improvement of the hemifacial spasm, deaf-