A rare case of Chiari malformation secondary to Paget's dise
The present case has been reported in the journal Neurology India.

A 76-year old man presented with increasing head circumference with otherwise normal neurological findings. Physical examination revealed a striking deformity of the skull with a maximum circumference of 68 cm. His serum alkaline phosphatase (ALP) level was elevated at 322 IU/L (normal 45–125 IU/L). The serum calcium and intact parathyroid hormone levels were within normal limits.

The hemogram parameters, such as white blood cell count, red blood cell count and hemoglobin were also within the normal range. Computed tomography (CT) scans disclosed marked thickening of the bone, widening of the diploe, and diffuse thickening of the skull base.

Sagittal T2-weighted images revealed widening of the diploic space, distortion and flattening of the cerebral and cerebellar hemispheres, platybasia with basilar invagination, reduction of the volume of the posterior fossa, and 10 mm downward displacement of the cerebellar tonsils into the foramen magnum.

Review of magnetic resonance (MR) imaging of the head obtained 3 years earlier had demonstrated mild Chiari malformation More Details with only 4 mm cerebellar tonsillar descent into the upper cervical canal. A progressive Chiari malformation secondary to skull Paget's disease was suggested.

The following day, a discussion was held with the patient and his family regarding the management options. He was treated with biphosphonate to prevent progression of the neurological involvement; a long-term follow-up was required to assess the improvement in the condition.

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