Cervical Dumbbell Ganglioneuroma Producing Spinal Cord Compression

Letter to Editor

References A B

1. Bed TR, Kaur S, Kumar B. Red grain mycetoma of the scalp (Actinomadura pelletieri). A case report from India. Mycopathologia 1978;63:127-8. 2. Venugopal PV, Venugopal TV. Red grain mycetoma of the scalp due to Actinomadura pelletieri in Madurai. Indian J Pathol Microbiol 1990;33:384-6. 3. Mathur DR, Bharadwaj V, Vaishnav K, Ramdeo IN. Red grain mycetoma caused Actinomadura pelletieri in western Rajasthan. Report of two cases. Indian J Pathol Microbiol 1993;36:486-8 4. Fahal AH, el-Toum EA, el-Hassan AM, Mahgoub-ES, Gumma SA. A prelimi- nary study on the ultrastructure of Actinomadura pelletieri and its host tissue reaction. J Med Vet Mycol 1994;32:343-8. 5. Ndiaye B, Develoux M, Langlade MA, Kane A. Actinomycotic mycetoma. Aprospos of 27 cases in Dakar: medical treatment with cotrimoxazole. Ann Figure 2: Postoperative MRI of cervical spine: A. Transverse image Dermatol Venereol 1994;121:161-5. after intraspinal operation shows no tumour in spinal canal with remaining paraspinal mass; B.transverse image after extraspinal approache reveals complete tumor removal Accepted on 10-04-2005

Cervical dumbbell ganglioneuroma producing spinal cord compression

Sir, A 39-year old man presented with complains of progressive weakness and numbness of all four limbs for six months. There was moderate spastic tetraparesis that was more marked on Figure 3: The pathologic finding of tumor tissue shows mature ganglion cells inside characteristic wave and loose stroma the left side, and hypoesthesia below the C5 dermatome. (HE, original magnification x 100). Magnetic resonance imaging (MRI) showed a large ex- tramedullary dumbbell mass at the C4-C5 level. The tumor intraforaminal mass was performed through the foramen was hypointense on the T1 and hyperintense on T2 images. which had been already enlarged by tumor growth. After four The spinal cord was severely compressed [Figure 1 A and B]. weeks the patient underwent second operation. The paraspinal A two-staged operation was performed to resect the tumor. extravertebral component of tumor was excised through the First, the patient was operated through a posterior cervical left lateral cervical approach [Figure 2B]. The vertebral ar- approach. Wide laminectomy of C4 and C5 was done. The tery was dissected off the surface of encapsulated tumor. mass was solid, well capsulated, elastic, moderately vascular- At a three-year follow-up the patient had regained the mo- ised, purely extradural and ventrolaterally located to the spi- tor strength in all four limbs. There was no radiographic signs nal cord. The lesion originated from cervical nerve C5, which of recurrence. was resected with tumor [Figure 2A]. resection of Histological examination of both tumor masses confirmed that the lesion was a ganglioneuroma [Figure 3].

Discussion

Kyoshima et al.[1] surveyed the literature on the subject and identified a total of only five pathologically confirmed cases of cervical spine ganglioneuromas. One patient was an 18-month old child and rest of the patients were young adults. Von Reck- linghausen’s disease was present in two patients. The symp- A B toms spinal cord compression were present in all reported

Figure 1: Preoperative MRI of cervical spine: A. Sagital image shows cases. Two patients had bilateral tumors. The origin of tumors extramedullary mass with slightly heterogeneous enhancement at C4- was sensory root ganglion or cervical nerve. In all the reported C5 vertebral level; B. transverse image reveals intraspinal dumbbell shaped mass extending to the left paraspinal region through the C4-5 cases, the tumor growth was in dumbbell pattern. Intraspinal interevertebral foramen. extradural growth was observed in three patients, while in-

370 Neurology India | September 2005 | Vol 53 | Issue 3 370 CMYK Letter to Editor tradural extension was seen in two patients. Bilateral upper limb weakness with relative sparing of lower On MRI about 75% of ganglioneuromas are isointense and limbs is usually seen in lesions involving the medullary decus- 25% are hypointense on T1 images. Most of them are sation of the pyramidal tracts, or cervical spinal cord. Such a hyperintense on T2 images. The non-homogeneous appear- clinical syndrome due to lesions occurring bilaterally in the ance corresponds to areas of cystic degeneration, hemorrhage motor cortex is a rare event. These bilateral cortical lesions or necrotic degeneration.[4,5] producing brachial diplegia are usually infarcts secondary to Ganglioneuromas are well encapsulated tumors and can be cerebral hypoperfusion following shock or aortic surgery. Cer- completely excised. Even when they are intradural, the tumor ebral tumor causing such paralysis is extremely rare. could be removed without cord injury because they are not A 37-year-old Nepalese national was admitted with seven- week history of gradually progressive worsening weakness of adherent to the spinal cord.[1] This and previously reported both arms. Weakness involved predominantly the shoulders, cases indicate that spinal ganglioneuromas could be completely elbows and to a lesser extent, the wrist movements. Hands removed and cured. were relatively spared. He was unable to raise his arms or flex Danilo V. Radulovi, D. Branislav, Milica K. his elbows. He had remained ambulatory, continent, seizure- Skender-Gazibara,*Nikoli M. Igor free, with no visual or gait disturbances. For two days prior to Institute for Neurosurgery, Belgrade, Serbia & Montenegro and admission, he had complained of dull, generalized headache *Institute for Pathology, Medical faculty University of Belgrade, accompanied by one episode of vomiting. Serbia & Montenegro E-mail: [email protected] Clinical examination revealed well-built and nourished nor- motensive male, with no abnormality of higher mental func- References tions. Funduscopy revealed early bilateral papilledema. There was no nystagmus or involvement of facial or of lower cranial 1. Kyoshima K, Sakai K, Kanaji M, Oikawa S, Kobayashi S, Sato A, Nakayama nerves. Motor system examination revealed power in both del- J. Symmetric dumbbell ganglioneuromas of bilateral C2 and C3 roots with toids to be grade 0/5, that in elbow flexors 1/5 with wasting of intradural extension associated with von Recklinghausen’s disease: case report. Surg Neurol 2004;61:468-73 deltoids. Tone was increased in both upper limbs with brisk 2. Maggi G, Dorato P, Trischitta V, Varone A, Civetta F. Cervical dumbbell gangli- biceps and triceps jerks. No fasciculations were observed. oneuroma in an eighteen month old child. A case report. J Neurosurg Sci There was no sensory impairment. MRI brain showed bilat- 1995;39:257-60 3. Ugarriza LF, Cabezudo JM, Ramirez JM, Lorenzana LM, Porras LF. Bilateral eral frontal convexity space occupying lesions with surround- and symmetric C1-C2 dummbell ganglioneuromas producing severe spinal cord ing edema [Figure 1]. compression. Surg Neurol 2001;55:228-31 After initial treatment with cerebral decongestants and dex- 4. Ichikawa T, Ohtomo K, Araki T, Fujimoto H, Nemoto K, Nanbu A, Onoue M, Aoki K. Ganglioneuroma: Computed tomography and magnetic resonance fea- amethasone, the right sided tumor was excised by craniotomy, tures. Br J Radiol 1996;69:114-21 while the left sided tumor was biopsied stereotactically at a 5. Lonergan GJ, Schwab CM, Suarez ES, Carlson CL. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. later date. Histopathology of the excised specimen confirmed Radiographics 2002;22:911-34 both the tumors to be glioblastoma multiforme. The syndrome of disproportionate weakness of the upper Accepted on 19-04-2005

Multicentric glioma presenting as man-in-the-barrel syndrome

Sir, Primary motor cortex is somatotopically organized, and the motor representation in the precentral gyrus forms a motor homunculus – the leg and perineum is represented over the medial aspect of the motor strip, and the arm and the hand over the convexity. It is well known that precise and circum- scribed weakness may affect one limb only if the appropriate area of the motor cortex or its projection pathway is selec- Figure 1: MRI brain showing bilateral frontal convexity space occupying lesions with surrounding [1] tively damaged. edema

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