✓ In his 1756 text, Observations pratiques sur les maladies de l'urèthre et sur plusiers faits convulsifs, Nicolas André coined the term “tic douloureux.” He believed that this pain originated from compression of facial sensory peripheral nerves. Using scientific observation and experimentation to confirm this hypothesis, he reproduced the tic pain and treated it by using careful efforts to remove adhesions from the nerve with a caustic solution of mercury water. Believing that recurrence of the pain was a result of early closure of the wound, with recompression of the nerve being the direct cause, André prevented recompression by ensuring open wound drainage. André's surgical technique of using cauterizing stones ensured that there was minimal blood loss and little danger of rebleeding and recompression of the nerve by an accumulated blood clot. His case reports include lengthy follow-up periods that documented the benefits of his procedures, which were confirmed by testimonials from uninvolved colleagues. Although remembered for the two words, “tic douloureux,” Nicolas André has long been ignored for his prescient treatment and scientific analysis of a disease for which the modern standard of care has only been defined during the last generation.
Jeffrey A. Brown, Catherine Coursaget, Mark C. Preul, and Devdutta Sangvai
Mark C. Preul, William Feindel, T. Forcht Dagi, Joseph Stratford, and Gilles Bertrand
✓ The contributions of Arthur Elvidge (1899–1985), Wilder Penfield's first neurosurgical recruit, to the development of neurosurgery have been relatively neglected, although his work in brain tumors extended the previous work of Percival Bailey and Harvey Cushing. He published rigorous correlations of clinical and histological information and formulated a revised, modern nosology for neuroepithelial tumors, including a modern histological definition of glioblastoma multiforme. Well ahead of his time, he believed that glioblastoma was not strictly localized and was the first to comment that the tumor frequently showed “satellitosis.” He was the first neurosurgeon in North America to use angiography as a radiographic aid in the diagnosis of cerebrovascular disease. Having studied with Egas Moniz, he was the first to detail the use of angiographic examinations specifically for demonstrating cerebrovascular disorders, believing that it would make possible routine surgery of the intracranial blood vessels. Seeking to visualize all phases of angiography, he was the impetus behind the design of one of the first semi-automatic film changers. Elvidge and Egas Moniz made the first observations on thrombosis of the carotid vessels independently of each other. Elvidge elucidated the significance of embolic stroke and commented on the ischemic sequelae of subarachnoid hemorrhage. Besides his contributions to neurosurgery, he codiscovered the mode of transmission of poliomyelitis. Elvidge's soft-spoken manner, his dry wit and candor, mastery of the understatement, love of exotic travel, and consummate dedication to neurosurgery made him a favorite of patients, neurosurgery residents, nurses, and other hospital staff. His accomplishments and example as teacher and physician have become part of neurosurgery's growing legacy.
Mark C. Preul, Richard Leblanc, Fernando Cendes, Francois Dubeau, David Reutens, Roberto Spreafico, Giorgio Battaglia, Massimo Avoli, Pierre Langevin, Douglas L. Arnold, and Jean-Guy Villemure
✓ Cerebral dysgenesis is a subject of interest because of its relationship to cerebral development and dysfunction and to epilepsy. The authors present a detailed study of a 16-year-old boy who underwent surgery for a severe seizure disorder. This patient had dysgenesis of the right hemisphere, which was composed of a giant central frontoparietal nodular gray matter heterotopia with overlying large islands of cortical dysplasia around a displaced central fissure. Exceptional insight into the function, biochemistry, electrophysiology, and histological structure of this lesion was obtained from neurological studies that revealed complementary information: magnetic resonance (MR) imaging, fluoro-2-deoxy-d-glucose positron emission tomography (PET), functional PET scanning, proton MR spectroscopic (1H-MRS) imaging, intraoperative cortical mapping and electrocorticography, in vitro electrophysiology, and immunocytochemistry. These studies demonstrated compensatory cortical reorganization and showed that large areas of heterotopia and cortical dysplasia in the central area may retain normal motor and sensory function despite strikingly altered cytoarchitectonic organization and neuronal metabolism. Such lesions necessitate appropriate functional imaging studies prior to surgery and cortical mapping to avoid creating neurological deficits. Integrated studies, such as PET, 1H-MRS imaging, cortical mapping, immunocytochemistry, and electrophysiology may provide information on the function of developmental disorders of cerebral organization.
Mark C. Preul, Joseph Stratford, Gilles Bertrand, and William Feindel
✓ Neurosurgeons are well known for being productive researchers and innovators. Few, however, have possessed the prolific ingenuity of William Cone. In 1934, he and Wilder Penfield were cofounders of the Montreal Neurological Institute where, until 1959, he filled the twin roles of neurosurgeon-in-chief and neuropathologist.
Because he did not find writing easy, many of his technical inventions and refinements remained unpublished. His numerous innovations included the extensive use of twist-drill technique for biopsy, drainage for subdural hematoma and cerebral abscess, and ventriculography. In the mid-1940's, he developed power tools driven by nitrogen that led to the modern, universally used air-driven tool systems. He had a special interest in the treatment of spinal dysfunction, for which he invented the Cone-Barton skull-traction tongs along with the Cone spinal operating table. He also devised operative procedures for vertebral fracture-dislocation and craniospinal anomalies. For the maintenance of muscle tone in the paralyzed bladder, he constructed a tidal drainage system. He introduced and popularized ventriculoperitoneal shunting techniques and carried out some of the earliest experimental trials to treat brain infections with sulphonamide and antibiotic drugs. He designed his own set of surgical suction devices, bone rongeurs, and a personal suction “air-conditioning” system for each surgeon. He had a keen early interest in intracranial tumors, and also demonstrated on monkeys how subdural mass lesions caused pupillary dilation and mesial temporal lobe damage due to cerebral compression. His work for the military during World War II on effects of altitude on brain pressure remained classified for many years. The first clipping and excision of an intracranial aneurysm is attributed to Cone.
Although Penfield was known as “the Chief,” Cone was referred to as “the Boss.” His fervent dedication to provide total care to his patients was expressed in round-the-clock vigils; he did not separate “nursing” from “surgical” care. Ultimately, Cone's driving passion for perfection led in part to his tragic death. His accomplishments, inventions, and his example as teacher and physician have become part of neurosurgery's collective legacy.
Report of three cases
Mark C. Preul, Richard Leblanc, Donatella Tampieri, Yves Robitaille, and Ronald Pokrupa
✓ Spinal angiolipomas are distinct, benign lesions composed of mature lipocytes admixed with abnormal blood vessels. Three new cases of spinal angiolipoma are presented and 34 previously reported cases are analyzed. The 37 total cases (23 females and 14 males) ranged in age from 17 to 73 years (mean 43 years; median 45 years). The mean age of the female patients was older than that for the males (45.0 vs. 41.6 years; p < 0.001, Student's t-test) and most were peri- or postmenopausal. Prior to diagnosis, 97% of the patients had weakness of the lower extremities, 94% had sensory dysfunction, 84% had hyperreflexia and spasticity, 51% had sphincter dysfunction, and 41% had back pain lasting from 1 to 180 months (mean 28 months). Five (22%) of the 23 female patients were pregnant and two had exhibited significant weight gain coincident with the onset of symptoms. The angiolipomas were extradural in 35 patients and intramedullary in two; seven of the extradural lesions infiltrated the surrounding bone. The tumors extended from C-6 to L-4 and had a predilection for the midthoracic region (53% of cases). Plain radiographs were abnormal in 11 (39%) of 28 patients and in all patients with bone infiltration. Myelograms were abnormal in 97% of 32 patients and showed a complete block in 63% of patients. Computerized tomography (CT) and magnetic resonance (MR) imaging revealed the fat-density lesions in all cases studied. There was vascular enhancement in three of five cases with contrast-infused CT and in the one case with gadolinium-infused MR imaging. All patients improved following resection of the epidural lesions and internal decompression of the intramedullary lesions.
It is concluded that spinal angiolipomas predominantly affect women. They involve the thoracic (especially the midthoracic) region, and produce symptoms and signs of spinal compression and, in some cases, bone erosion and pathological fractures. Their symptomatology can be exacerbated by pregnancy and weight gain, suggesting that vascular engorgement and the presence of obesity influence their evolution. Their preponderance in older, peri-, or postmenopausal women, and their clinical exacerbation in pregnant women support a role for hormonal influence. Magnetic resonance imaging is the investigation of choice for the diagnosis of these lesions. Surgery is universally successful in relieving symptoms.
The role of the “Cushing ritual” and influences from the European experience
Mark C. Preul and William Feindel
✓ Wilder Penfield left two great legacies: the development of successful surgical treatment of epilepsy and the establishment with his colleagues of the Montreal Neurological Institute as a world-renowned medical center, “dedicated to relief of pain and suffering and to the study of neurology.” That Harvey Cushing's surgical ritual (which stemmed from the painstaking operative methods of Halsted) played a paramount role in the origins of Penfield's surgical technique is revealed by a set of notes and drawings by Penfield during repeated visits in the 1920's to Cushing's clinic at the Peter Bent Brigham Hospital.
Penfield's intellectual approach to the nervous system was derived from his studies with Sherrington, Holmes, Cajal, and Hortega. His eclectic surgical style emerged from his familiarity with the operating techniques of Halsted, Dandy, Horsley, Sargent, Cushing, Frazier, Whipple, Leriche, and Foerster. Penfield's debt to these teachers is documented in his memoirs and in an unpublished report on European neurosurgery which he sent to the Rockefeller Foundation in 1928.
Mark C. Preul, Phillip B. Long, Jeffrey A. Brown, Manuel E. Velasco, and Michael T. Weaver
✓ The histopathological and autonomic effects of percutaneous trigeminal ganglion compression for trigeminal neuralgia were studied in New Zealand White rabbits. Drops in mean arterial blood pressure of 38% and in heart rate of 30% were observed during compression (p < 0.0001). Corneal reflex, pinprick sensation, and mastication strength were intact in 13 of 14 rabbits after compression. These findings resembled the effects of percutaneous compression in humans and suggested that the New Zealand White rabbit is a useful model for the study of percutaneous compression.
Trigeminal sensory roots and ganglia from 14 rabbits killed at intervals from 1 to 84 days after percutaneous compression were sectioned and stained using immunoperoxidase for neurofilaments, hematoxylin and eosin, luxol fast blue, and cresyl echt violet. Focal axonal damage and demyelination were present 7 days after compression. No difference could be detected in the perikaryonal distribution of neurofilaments between compressed and control trigeminal ganglia. Focal demyelination and Schwann cell proliferation preceding remyelination were present in the trigeminal sensory root at 84 days. Differential injury of axons compared to trigeminal ganglion cell bodies suggests that axonal regeneration is possible and may contribute to the recovery of motor and sensory function in patients after percutaneous compression.
Experience in 22 patients and review of the literature
Jeffrey A. Brown and Mark C. Preul
✓ Between 1983 and 1988, a percutaneous trigeminal ganglion compression (PTGC) procedure for trigeminal neuralgia was performed on 22 patients. All patients were initially relieved of their pain. There were three recurrences (14%); two of these patients underwent a second PTGC procedure and one a partial trigeminal nerve root section. Follow-up examination 3 to 53 months after the procedure showed that all patients were free of pain. Morbidity included persistent minor hypesthesia in five patients, persistent minor dysesthesias in three, persistent minor weakness in three, aseptic meningitis in one, transient sixth nerve palsy in one, and transient otalgia in three. None of the patients had either anesthesia dolorosa or an absent corneal reflex.