Hypertrophic Spinal Pachymeningitis

Abstract

YPERTROPHIC cervical pachymeningirls is a clinical syndrome characterized by compression of the spinal roots and spinal cord caused by a marked inflammatory hypertrophy of the cervical dura mater. The pathological process can spread to the leptomeninges and give rise, because of the progressive compression, to areas of softening of the spinal cord, where it is also possible to find cavities similar to those in syringomyelia. 26,29,4~ The cause of the disease is still unknown26,3~ The clinical picture is still that first described by Charcot in 18696 and Joffroy in 1873, TM who divided the evolution of the disease into three stages. The first stage is characterized by intermittent radicular pains in the neck, shoulders, and arms, which gradually become continuous. In the second stage, muscular weakness and atrophy develop in the arms. Finally, spastic paralysis of the legs, loss of various modalities of sensation, paresis of bowel and bladder, and respiratory disturbances characterize a third stage. The disease has been described in the cer-vico_thoraeic,S,2s,29.3t ,37 thoracic,~4 z~,25.3s.~9 and lumbar 1,11 regions, or along the entire extension of the spinal column, 6,24,27 with symptoms varying according to the level of the lesion. We are reporting five cases of hypertrophic spinal pachymeningitis operated upon during 1955 to 1965. We have excluded from this series 14 cases of chronic epidural inflammatory processes and 30 cases of spinal arachnoiditis operated on in the same period, even though some of these showed a moderate thickening of the spinal dura mater. Case Reports Case 1. S.S., a 3~-year-old male, entered the clinic on January 18, 1955, suffering from continuous pains in the buttocks, and numbness and weakness in both legs, of 3 weeks duration. He had no sphincteric dis

Idiopathic Hypertrophic Spinal Pachymeningitis

“…Surgical decompression of the spinal cord is recommended for HSP to alleviate neurological symptoms and sequelae (2,5,10). The surgical removal of the posterior surface of the dura beyond the apparent limit of the lesion is useful for decreasing recurrences (7,14). Urgent surgical decompression and duralplasty were essential in our patient to prevent irreversible damage to the nervous system, because our patient exhibited rapidly advanced signs of myelopathy and the etiology was not defined.…”

“…HSP is an uncommon cause of nerve root and spinal cord compression and typically involves the cervical and thoracic spinal cord levels (4,7,8). The signs and symptoms of HSP gradually develop from those of nerve root compression to spinal cord compression (1, 7), and almost all patients that suffer from HSP experience progressive paraparesis (4).…”

Long-term outcome of idiopathic hypertrophic thoracic pachymeningitis

“…Males are affected more frequently than females (3:2). It commonly involves the cervical and thoracic dura [1,5,7,12] and presents initially as progressive radicular symptoms, with muscle weakness and atrophy as the second stage, and paraplegia, loss of bladder function, bowel disturbance, and respiratory distress caused by intercostal and diaphragmatic denervation as the third stage [2].…”

Non-obstructive idiopathic pachymeningitis cervicalis hypertrophica.

“…-8 11 12 The preoperative periods reported were usually less than a few years. The diagnostic importance of complete myelographic blockage is clear from the reported cases.3 48 11 Partial block has been exceptionally rare and considered to occur probably only in the earlier stage of the disease process.2 12 The repeatedly normal Queckenstedt tests and myelograms, 13 and I1 years after the onset, delayed the correct diagnosis and proper management of our cases, and also make them unusual.…”