Letters to Editor

Sachin A. Borkar, G. D. Satyarthee, B. S. Sharma, Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India. E-mail: [email protected] DOI: 10.4103/0028-3886.53271 References

1. Samii M, Klekamp J. Surgical results of 100 intramedullary tumors in relation to accompanying . Neurosurgery 1994;35:865-73. 2. Pai SB, Krishna KN. Secondary holocord syringomyelia with spinal hemangioblastoma: A report of two cases. Neurol India 2003;51:67-8. 3. Sarikaya S, Acikgöz B, Tekkök IH, Güngen YY. Conus ependymoma with holocord syringohydromyelia and syringobulbia. J Clin Neurosci 2007;14:901-4. 4. Moser FG, Tuvia J, LaSall P, Llana J. Ependymoma of the spinal nerve root: Case report. Neurosurgery 1992;31:962-4. 5. Kumar J, Kumar A, Gupta S. Neurological pictures. Chiari I malformation with holocord syrinx. J Neurol Neurosurg Psychiatry 2007;78:146. 6. Isu T, Abe H, Iwasaki Y, Akino M, Koyanagi I, Hida K, et al. Diagnosis and surgical treatment of spinal hemangioblastomas. No Shinkei Geka 1991;19:149-55. Figure 1: Gadolinium-enhanced magnetic resonance imaging of the spine showing a well-defined heterogenously contrast-enhancing mass 7. Rengachary SS, Blount JP. Hemangioblastomas In: Wilkins RH, at D11–L1 level with accompanying holocord syringohydromyelia Rengachary SS, editors. Neurosurgery. Vol. 1. Mc Graw Hill; 1996. p.1205-19.

MRI spine done did not show any evidence of residual Accepted on 25-03-2009 tumor and partial resolution of syrinx.

Spinal ependymomas are the most common ‘Garland sign’ in amyotrophic intramedulary tumor in adults and commonly involves conus medullaris.[4] Holocord syrinx lateral sclerosis is more commonly reported in association with Chiari I malformation[5] and also in association with spinal Sir, hemangioblastomas.[2] Spinal hemangioblastomas are A 37-year-old lady presented with pure motor, asymmetrical onset, progressive quadriparesis with highly vascular tumors and account for 1.6–2.1% of wasting of hands and feet of one year duration, with all primary spinal cord tumors.[6] Up to 50% cases of dysphagia, dysarthria, and emotional incontinence of spinal hemangioblastomas are associated with syrinx four months duration. On examination, she had upper [7] formation. However, holocord syringohydromyelia motor neuron (UMN) and lower motor neuron (LMN) in association with spinal cord tumors is extremely signs in bulbar muscles, upper and lower limbs without rare. The pathophysiology of syringohydromyelia involvement of extraocular movements. Rest of the associated with intramedullary spinal cord tumors neurological examination was normal. She satisfied appears to be most likely due to transudation of fluid the El Escorial criteria for definite amyotrophic lateral from the pathological tumor vessels. The other possible sclerosis (ALS). Her electrodiagnostic studies showed mechanisms include obstruction to CSF flow in the evidence of preganlionic neurogenic lesion involving central canal and extracellular perimedulary fluid flow. bulbar, cervical, thoracic, and lumbosacral spinal Regardless of histology, the higher the spinal level, the segments. Her work up for secondary causes of anterior more likely the syrinx formation.[1,2] horn cell disease was negative. She was evaluated with magnetic resonance imaging Gadolinium-enhanced MRI of spine is the investigation of (MRI) of the brain and spinal cord. MRI brain showed choice for intramedullary tumors with syringomyelia. The all the characteristic features of ALS including T2 presence of an associated syrinx suggests the resectability hyperintensity extending along corticospinal tract of the tumor, because it indicates a displacing rather than from centrum semiovale to crus cerebri bilaterally. an infiltrating tumor. Patients with syringomyelia tend T2 hypointensity was noted in precentral gyrus. MRI to recover from surgery earlier. No specific treatment of cervical spine showed T2 hyperintensity involving the the syrinx is necessary and the syrinx usually resolves anterolateral column of the spinal cord. The coronal T2 with excision of the tumor, as in our case.[1] image was forming a characteristic ‘garland pattern’

354 India | May-Jun 2009 | Vol 57 | Issue 3 Letters to Editor

Figure 1: (a) Magnetic resonance imaging axial FLAIR sequence showing hyperintensity along corticospinal tracts at the level of crus cerebri; (b) posterior limb of internal capsule; (c) corona radiata; (d) and centrum semiovale; (e) MRI axial T2 sequence showing hypointensity in motor cortex bilaterally (arrow heads); (f) MRI coronal T2 sequence showing hyperintensity along corticospinal tract (arrows) and corpus callosum forming a ‘Garland pattern’; (g) MRI axial T2 sequence of the cervical cord showing hyperintensity in anteroalateral segments with sparing of posterior segment of hyperintensity extending along corticospinal tracts References as well as involving corpus callosum [Figure 1]. This peculiar imaging appearance has hitherto not been 1. Waragai M. MRI and clinical features in amyotrophic lateral sclerosis. Neuroradiology 1997;39:847-51. reported in a classical case of ALS. T2-weighted image 2. Van Zandijcke M, Casselman J. Involvement of corpus callosum in showing high signal intensity involving the corticospinal amyotrophic lateral sclerosis shown by MRI. Neuroradiology 1995;37:287-8. 3. Van der Knaap MS, Ramesh V, Schiffmann R, Blaser S, Kyllerman M, tract extending into the anterolateral column of the spinal Gholkar A, et al. Alexander disease: Ventricular garlands and cord has been described.[1] Involvement of the corpus abnormalities of the medulla and spinal cord. Neurology 2006:66:494-8. callosum has also been reported.[2] In addition, T2- 4. Lewis MR, Deavers MT, Silva EG, Malpica A. Ovarian involvement by metastatic colorectal adenocarcinoma: Still a diagnostic challenge. Am weighted MRI typically demonstrates low signal intensity J Surg Pathol 2006;30:177-84. in the motor cortex; this finding has been attributed to T2 5. Sorger K, Gessler U, Hübner FK, Köhler H, Schulz W, Stühlinger W, et al. Subtypes of acute postinfectious glomerulonephritis. Synopsis of [1] shortening due to iron deposition. A lesion distribution clinical and pathological features. Clin Nephrol 1982;17:114-28. simulating a garland has been described with Alexander disease, metastatic colorectal adenocarcinoma, and acute Accepted on 31-03-2009 postinfectious glomerulonephritis.[3-5] Comprehensive rural Atma Ram Bansal, Gopal Krishna Dash, Ashalatha Radhakrishnan, Chandrasekharan surveillance programme in Kesavadas1, Muraleedharan Nair Uttarakhand state of India Departments of Neurology, and 1Imaging Sciences & Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Sir, Technology, Trivandrum, Kerala, India. Uttarakhand is the youngest state of India covering hills E-mail: [email protected] of Himalayas. We conducted community-based cross- DOI: 10.4103/0028-3886.53273 sectional study to determine the prevalence and profile

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