Neurology India Free Full Text at and March, 2008 C O N T E N T S Vol

Neurology India Free full text at www.neurologyindia.com and www.bioline.org.br/ni March, 2008 C O N T E N T S Vol. 56 Issue 1

Editorial

‘Aqualisation’ of neuraxis: Wondrous neuraqua CSF 1 Manu Kothari, Atul Goel ...... 1

View and Review

Organization of neurology services in India: Unmet needs and the way forward Mandaville Gourie-Devi ...... 4

Original Articles

Endoscopic management of brain abscesses Yad Ram Yadav, Mallika Sinha, Neha, Vijay Parihar ...... 13

Pattern of cerebellar perfusion on single photon emission computed tomography in subcortical hematoma: A clinical and computed tomography correlation Jayantee Kalita, Usha K. Misra, Prasen Ranjan, P. K. Pradhan ...... 17

Imaging features in Hirayama disease Hemant A. Sonwalkar, Rakesh S. Shah, Firosh K. Khan, Arun K. Gupta, Narendra K. Bodhey, Surjith Vottath, Sukalyan Purkayastha ...... 22

Delayed habituation in Behcet’s disease Sefa Gulturk, Melih Akyol, Hulusi Kececi, Sedat Ozcelik, Ziynet Cınar, Ayse Demirkazık ...... 27

Erythrocyte indicators of oxidative changes in patients with graded traumatic head injury Chandrika D. Nayak, Dinesh M. Nayak, Annaswamy Raja, Anjali Rao ...... 31

Repeat gamma knife radiosurgery for recurrent or refractory trigeminal neuralgia Liang Wang, Zhen-wei Zhao, Huai-zhou Qin, Wen-tao Li, Hua Zhang, Jian-hai Zong, Jian-Ping Deng, Guo-dong Gao ...... 36

Taste dysfunction in vestibular schwannomas Rabi Narayan Sahu, Sanjay Behari, Vimal K. Agarwal, Pramod J. Giri, Vijendra K. Jain ...... 42

Surgical management of traumatic intracranial pseudoaneurysms: A report of 12 cases Xiang Wang, Jin-Xiu Chen, Chao You, Min He ...... 47

Expression of truncated dystrophin cDNAs mediated by a lentiviral vector Sun Shunchang, Chen Haitao, Chen Weidong, He Jingbo, Peng Yunsheng ...... 52

Gamma knife radiosurgery for glomus jugulare tumors: Therapeutic advantages of minimalism in the skull base Manish S. Sharma, A. Gupta, S. S. Kale, D. Agrawal, A. K. Mahapatra and B. S. Sharma ...... 57

Neurology India | January-March 2008 | Vol 56 | Issue 1 111 Neurology India Free full text at www.neurologyindia.com and www.bioline.org.br/ni March, 2008 C O N T E N T S Vol. 56 Issue 1

Case Reports

Subarachnoid hemosiderin deposition after subarachnoid hemorrhage on T2*-weighted MRI correlates with the location of disturbed cerebrospinal ﬂ uid ﬂ ow on computed tomography cisternography Yoshifumi Horita, Toshio Imaizumi, Yuji Hashimoto, Jun Niwa ...... 62

Anesthesia management of awake craniotomy performed under asleep-awake-asleep technique using laryngeal mask airway: Report of two cases Gadhinglajkar Shrinivas Vitthal, Rupa Sreedhar, Mathew Abraham ...... 65

High cervical C3-4 ‘disc’ compression associated with basilar invagination Atul Goel ...... 68

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing: Response to antiepileptic dual therapy Ravi Gupta, Manjeet S. Bhatia ...... 71

Correlation of autism with temporal tubers in tuberous sclerosis complex Kavitha Kothur, Munni Ray, Prahbhjot Malhi ...... 74

Non-traumatic carotid dissection and stroke associated with anti-phospholipid antibody syndrome: Report of a case and review of the literature Benzi M. Kluger, Richard L. Hughes, C. Alan Anderson, Kathryn L. Hassell ...... 77

Osteoma of anterior cranial fossa complicated by intracranial mucocele with emphasis on its radiological diagnosis Jinhu Ye, Hui Sun, Xin Li, Jianping Dai ...... 79

Vasospasm after transsphenoidal pituitary surgery: A case report and review of the literature Manish Kumar Kasliwal, Ravinder Srivastava, Sumit Sinha, Shashank S. Kale, Bhawani S. Sharma ...... 81

Chondromyxoid ﬁ broma of the seventh cervical vertebra Ashish Jonathan, Vedantam Rajshekhar, Geeta Chacko ...... 84

Acute progressive midbrain hemorrhage after topical ocular cyclopentolate administration Tarkan Calisaneller, Ozgur Ozdemir, Erkin Sonmez, Nur Altinors ...... 88

Letters to Editor

Digital subtraction angiography laboratory with inbuilt CT (DynaCT): Application during intracranial anurysm embolization ...... 90

Concomitant tuberculous and pyogenic cerbellar abscess in a patient with pulmonary tuberculosis ...... 91

Drug complianceafter stroke andmyocardial infarction:Is complementary medicine an issue? ...... 93

112 Neurology India | January-March 2008 | Vol 56 | Issue 1 Neurology India Free full text at www.neurologyindia.com and www.bioline.org.br/ni

March, 2008 C O N T E N T S Vol. 56 Issue 1

Multiple intracranial developmental venous anomalies associated with complex orbitofacial vascular malformation ...... 93

Nitrofurantoin-induced peripheral neuropathy:A lesson to be re-learnt ...... 94

Posterior longitudinal ligament cyst as a rare cause of lumbosacral radiculopathy with positive straight leg raising test ...... 96

Aqueductal stenosis caused by an atypical course of a deep collector vein draining bilateral cerebellar developmental venous anomalies ...... 97

Recovery of increased signal intensity of the cervical cord on magnetic resonance imaging after surgery for spontaneous spinal epidural hematoma causing hemiparesis ...... 98

Simultaneous thalamic and cerebellar hypertensive hemorrhages ...... 100

Neuroimages

MRI and MRA in spontaneous intracranial arterial dissection S. Raghavendra, Sanjeev V. Thomas, Krishnamoorthy Thamburaj, Bejoy Thomas ...... 102

Shunt catheter migration into pulmonary arteries Miikka Korja, Matti K. Karvonen, Arto Haapanen, Reijo J. Marttila ...... 103

Susceptibility weighted imaging in holohemispheric venous angioma with cerebral hemiatrophy Sivaraman Somasundaram, Chandrasekharan Kesavadas, Bejoy Thomas ...... 104

Forthcoming Events ...... 105

Instructions to Authors ...... 106

Referees List - 2007 ...... 000???

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Neurology India | January-March 2008 | Vol 56 | Issue 1 113 Original Article

Imaging features in Hirayama disease

Hemant A. Sonwalkar, Rakesh S. Shah1, Firosh K. Khan1, Arun K. Gupta, Narendra K. Bodhey, Surjith Vottath, Sukalyan Purkayastha Department of Imaging Sciences and Interventional Radiology, 1Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Purpose: To evaluate the MR Þ ndings in clinically suspected literature for the diagnosis of this entity. The present cases of Hirayama disease. Materials and Methods: The study summarizes these different MRI findings and pre and post contrast neutral and ß exion position cervical evaluates their distribution among the studied patient MR images of eight patients of clinically suspected Hirayama population. disease were evaluated for the following Þ ndings: localized lower cervical cord atrophy, asymmetric cord ß attening, Materials and Methods abnormal cervical curvature, loss of attachment between the posterior dural sac and subjacent lamina, anterior Eight patients with clinical suspicion of Hirayama shifting of the posterior wall of the cervical dural canal disease were evaluated with MR imaging. The pre and and enhancing epidural component with ß ow voids. The post contrast MR imaging was done in neutral and distribution of the above features in our patient population flexion position of cervical spine and imaging findings was noted and correlated with their clinical presentation were correlated with their neurological presentation, and electromyography Þ ndings. Observations: Although disease course and electromyography findings. All the lower cervical cord atrophy was noted in all eight cases of patients evaluated were males between 11-24 years suspected Hirayama disease, the rest of the Þ ndings were of age with the mean age of 18.5 years. The criteria variably distributed with asymmetric cord ß attening, abnormal for patient selection were weakness and wasting cervical curvature, anterior shifting of the posterior wall of predominantly in C7, C8 and T1 myotomes in one upper the cervical dural canal and enhancing epidural component limb or asymmetrically in both upper limbs, insidious seen in six out of eight (75%) cases. An additional Þ nding of onset in teens or early twenties, initial fast progression thoracic extension of the enhancing epidural component was for one to three years followed by arrest of disease or also noted in Þ ve out of eight cases. Conclusion: Dynamic relatively benign course, irregular coarse tremors in the post contrast MRI evaluation of cervicothoracic spine is an fingers of the affected hand[s], mild transient worsening accurate method for the diagnosis of Hirayama disease. of symptoms on exposure to cold, electromyography Key words: Dynamic magnetic resonance imaging, Hirayama evidence of chronic denervation in the clinically or disease, monomelic amyotrophy subclinically affected muscles and absence of objective sensory loss.[1] Electrophysiological study included nerve conduction study (NCS) and electromyography Juvenile muscular atrophy of the distal upper limb (EMG). Motor conduction studies were done in median [Hirayama disease] is a rare disease affecting primarily and ulnar nerves (and radial in few cases) of both upper young men in the second to third decades.[1,2] Hirayama limbs and peroneal and tibial nerves of one lower limb. disease, juvenile muscular atrophy of the distal upper Sensory conductions were studied from median, ulnar extremity, is also known as monomelic amyotrophy,[3] and sural nerves of corresponding limbs. Needle EMG benign focal amyotroph,[4] juvenile muscular atrophy of studies were done in muscles of C7 - T1 myotomes a unilateral upper extremity[5] and juvenile asymmetric including the cervical paraspinals. segmental spinal muscular atrophy.[6] Hirayama disease The MR imaging was done on a superconducting differs from the known types of motor neuron diseases 1.5-T system (Signa, GE Medical Systems, Milwaukee, because of its nonprogressive behavior and pathologic Wisconsin). The neutral-position MR protocol included findings of focal ischemic changes in the anterior horn of transverse and sagittal T1-weighted (spin echo, repetition the lower cervical cord.[2,7] Various magnetic resonance time msec/echo time msec of 500-600/15-20), transverse imaging (MRI) features have been described in the T2*-weighted (gradient-echo, 400-500/15-20, flip

Arun K. Gupta Department of Radiology, SCTIMST, Trivandrum - 695 011, Kerala, India. E-mail: [email protected]

22 Neurology India | January-March 2008 | Vol 56 | Issue 1 Sonwalkar, et al.: Imaging features in Hirayama disease angle of 20°-30°) MR imaging and sagittal T2-weighted MR imaging (fast spin echo, 3000-3500/90-140 [effective]). The flexion MR imaging protocol consisted of sagittal T1-weighted (500-600/15-20) and T2-weighted (4,000/85-99, matrix size of 256 × 256) MR imaging and transverse T2*-weighted MR imaging (400-500/15-20, flip angle of 20°-30°, matrix size of 256 × 192) with approximately 30-40° neck flexion by using an indigenous positioning sponge. Post gadolinium transverse and sagittal T1 weighted images in flexion and extension were obtained in all cases. Section thickness was 4 mm with 1-mm gap for both sagittal and transverse MR imaging with all sequences. Lower cervical cord was defined as the cord between C4 and C7. The following features were evaluated: localized lower cervical cord atrophy, asymmetric cord flattening, abnormal cervical curvature, loss of attachment between the posterior dural sac and subjacent lamina, anterior shifting of the posterior wall of the cervical dural canal Figure 1: Neutral and ß exion position post contrast fat saturated sagittal and enhancing epidural component with flow voids. An MRI images of patient no. 5 showing the anterior displacement of the dural sac and enhancing posterior epidural component with ß ow voids additional feature detected was the thoracic extension of the enhancing epidural component. Localized cord atrophy was defined as a decrease in cord size in to the thecal sac, which disappeared on neutral position, comparison with the normal cord above and that below was noted. the affected level confirmed on axial images. Asymmetric cord flattening was evaluated on transverse Results MR images. To avoid confusion with cord compression due to adjacent spurs or herniated disks, cord flattening was Clinical features defined as flattening without a narrowed or obliterated All the patients were male with mean age of 18.5 years adjacent subarachnoid space. An elliptic spinal cord (range 11-24) years at the time of clinical examination. was considered normal, a pear-shaped spinal cord was The duration of illness at the time of clinical examination considered asymmetric cord flattening and a triangular and electrophysiology study was three to 12 months in spinal cord was considered symmetric cord flattening. seven patients and 48 months in the eighth (patient Cervical curvature was classified according to the no. 4). Major symptoms included hand wasting (8/8), principles suggested by Guigui et al.[8] and Batzdorf and grip weakness (6/8), tremulousness of fingers (4/8). The Batzdorff.[9] Cervical curvature was measured according to patient who presented after four years of illness also the relationship of the dorsal aspect of the vertebral bodies had forearm wasting, weakness and dysaesthesia. On C3 through C6 to a line drawn from the dorsocaudal aspect clinical examination, all the patients had pure motor, of the vertebral body C2 to the dorsocaudal aspect of the asymmetrical, upper limb, lower motor neuron type of vertebral body C7. By definition, normal lordotic cervical involvement [Table 1]. None of the patients had any curvature is curvature in which no part of the dorsal aspect neck pain or radicular symptoms. of the vertebral bodies C3 through C6 crosses the line from C2 through C7. An abnormal [straight or kyphotic] curvature is curvature in which part or all of the dorsal Table 1: Clinical Þ ndings aspects of the vertebral bodies C3 through C6 meet or Clinical Þ ndings No. of patients Hand wasting [intrinsic muscles]- asymmetrical 6 cross through the line from C2 through C7 [Figure 1]. For Forearm wasting 4 evaluating the loss of attachment between the posterior Brachioradialis sparing [oblique atrophy] 3 dural sac and subjacent lamina, the lamina was defined as Arm wasting [biceps > triceps] 1* the part of vertebra between junctions of laminae medially Hand weakness 8 Forearm weakness [FDP > FDS > WF > PQ] 6 and laterally by a tangential line along the medial aspect of Minipoly myoclonus 5 the pedicle. This was divided equally into three parts. More Brisk deep tendon reß exes 1† than 33.3% loss of attachment between the posterior dural Bilateral involvement 6 sac and subjacent lamina was considered significant.[10] FDP - Flexor digitorum profundus FDS - Flexor digitorum superÞ cialis, WF - Wrist ß exors PQ - Pronator quadratus *The patient who presented late. No elbow or On flexion studies, anterior displacement of dural sac and shoulder weakness in spite of arm wasting, †No other upper motor neuron features. appearance of enhancing epidural component posterior MRI shows dynamic changes

Neurology India | January-March 2008 | Vol 56 | Issue 1 23 Sonwalkar, et al.: Imaging features in Hirayama disease

Table 2: Electrophysiological Þ ndings Electrophysiologic Þ ndings (patients number) 1 2 3 4 5 6 7 8 Distal latency - Ulnar N ↑ ↑ ↑ N ↑ N N Median N N N ↑ N ↑ N N Radial N N N N N ↑ N N CMAP - Ulnar N ↓ ↓ ↓ ↓ ↓ N ↓ Median N N N ↓ N N N N Radial N N N N N ↓ N N F wave latency-Ulnar N ↑ ↑ ± 0 0 N N Median N N N 0 0 N N N Radial N N N N N N N N SSEP - - N - - - - - EMG I, D I, FCR I, FCR FCU I I I I I Bilateral involvement + + - + - - + + CMAP - Compound muscle action potential, SSEP - Somatosensory evoked potential, EMG - Electromyography, I - Intrinsic hand muscles [Þ rst dorsal interosseus, abductor pollicis brevis mostly tested], D - Deltoid, FCR - Flexor carpi radialis, FCU - Flexor carpi ulnaris, ↑ - Prolonged, ↓ - Reduced, N - Normal, ± - Inconsistent, 0 - Absent

Electrophysiology Table 3: magnetic resonance imaging features Nerve conduction study and EMG were done in all Magnetic resonance imaging features No. of patients the patients within a week of the clinical examination. Localized lower cervical cord atrophy 8 In addition, somatosensory evoked potentials (SSEP) Asymmetric cord ß attening 6 were studied in one patient. Ulnar compound muscle Abnormal cervical curvature 6 Loss of attachment between the posterior action potential (CMAP) was reduced in six patients dural sac and subjacent lamina 4 while median and radial in one each. Distal latencies Anterior shifting of the posterior wall of the cervical and F-wave latencies were variably affected. Conduction dural canal 6 velocities were normal in all nerves. Sensory conduction Enhancing epidural component in lower cervical region 6 Thoracic extension of the enhancing epidural component 5 study was absolutely normal in all the patients. Median Prominent epidural ß ow voids 4 SSEP done in one patient was normal. All the eight Parenchymal signal changes in lower cervical cord 3 patients showed denervation changes (fibrillations, positive sharp waves, large amplitude potentials but no polyphasics or fasciculations) mainly in C8-T1 muscles. anterior shifting of the posterior wall of the cervical Cervical paraspinal EMG was done in six patients and dural canal and enhancing epidural component. The all were normal [Table 2]. thickness of the enhancing epidural component was, however, variable ranging from 1.5 to 6 mm. No relation Imaging ﬁ ndings [Table 3] was found between the duration of illness and thickness All eight cases of suspected Hirayama disease showed of the epidural component. An additional finding of lower cervical cord atrophy with asymmetry noted in thoracic extension of the enhancing epidural component six of eight cases [Figure 2]. These six cases were also was also noted in five cases. Abnormally prominent showing other findings like abnormal cervical curvature, posterior epidural flow voids were noted in four cases. Loss of attachment between the posterior dural sac and subjacent lamina was noted in four of these cases [Figure 3].

Follow-up Follow-up was available in six patients. One patient (patient no. 5) was initially evaluated in June 1996 when clinical and electrophysiology features were suggestive of Hirayama’s disease but MRI (no dynamic study done) was negative. Repeat examination after eight years (September 2004) showed mild progression (increased wasting and reflex loss). Repeat MRI done was suggestive of Hirayama’s disease in the dynamic study. The other five patients had last follow-up after four to 11 months of presentation (four using cervical

Figure 2: Axial neutral position gradient echo MR image from the cervical collar, including patient no.3 whose MRI is negative). spine of patient no.4 showing asymmetric cord ß attening All the five had static course clinically.

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lower cervical spinal cord.[1,7] The chronic circulatory disturbance resulting from repeated or sustained flexion of the neck may produce gliosis and localized cord atrophy at the lower cervical region.[15] Different pathophysiological mechanisms have been proposed for the venous engorgement seen on flexion studies. Firstly, the negative pressure in the posterior spinal canal resulting from anterior shifting of the dural canal is thought to increase the flow to the posterior internal vertebral venous plexus causing their prominence. Second, the posture of neck flexion decreases the venous drainage of the jugular veins, impeding the venous return of internal vertebral venous plexus causing their prominence and engorgement.[16] Finally, the compressed anterior internal vertebral venous plexus caused by anterior displacement of the dural Figure 3: Neutral axial and sagittal ß exion FSE T2 MRI images showing canal increases the burden of the posterior internal loss of attachment between the posterior dural sac and subjacent lamina, [17] signal changes, anterior displacement of the dural sac at the cervicotho- vertebral venous plexus leading to its distension. racic junction The extensions of posterior epidural enhancement in the thoracic region found in our cases suggest that Discussion jugular vein compression, as a cause of prominence of the epidural venous plexus is less likely. The pathogenesis of this entity is debated. An The various MR imaging features described in assumption of imbalanced growth between the patient’s Hirayama disease are as follows: localized lower cervical vertebral column and spinal canal contents has been cord atrophy, asymmetric cord flattening, parenchymal suggested.[6,11,12] This imbalanced growth will cause signal changes in lower cervical cord, abnormal cervical disproportional length between the patient’s vertebral curvature, loss of attachment between the posterior column and the spinal canal contents causing a tight dural sac and subjacent lamina, anterior shifting of the dural sac and anterior displacement of posterior dural posterior wall of the cervical dural canal, enhancing wall when the neck is flexed. The different growth rates epidural component in lower cervical and thoracic region between male and female patients have been proposed and prominent posterior epidural flow voids suggestive to be the factor related to the male preponderance of dilated epidural venous plexus. The findings reported of Hirayama disease by Toma et al.[13] The incidence more frequently are asymmetrical/symmetrical atrophy of atopic disorders and levels of serum IgE and mite of lower cervical cord, prominence and enhancement antigen-specific IgE were also found to be higher in of posterior epidural venous plexus on flexion studies patients with Hirayama disease than in the general and anterior shifting of posterior dural sac on flexion. population suggesting that atopy may be a contributing Loss of attachment between the posterior dural sac factor for Hirayama disease.[14] and subjacent lamina on neutral position, anterior In a normal spine, the spinal dura mater is a loose shifting of the posterior wall of the cervical dural canal, sheath that is anchored in the vertebral canal by the enhancing epidural component in the lower cervical and nerve roots and by its attachment to the periosteum thoracic region and prominent posterior epidural flow in two places: one at the foramen magnum and the voids suggestive of dilated epidural venous plexus on dorsal surfaces of C2 and C3 and the other at the flexion studies are reported as highly suggestive for the coccyx. Normally, the slack of the dura mater can diagnosis of Hirayama disease.[1,6,10,12,15,17] compensate for the increased length in flexion. In Early diagnosis and therapeutic intervention in the patients with Hirayama disease, however, the tight form of cervical collar therapy to prevent neck flexion dural sac can result in separation of the posterior dural may minimize the functional disability of the young sac from its subjacent lamina and various degrees of patients. This induces a premature arrest of disease abnormal cervical curvature (straight or kyphotic). On progression and is more beneficial in those cases with neck flexion, the tight dural sac cannot compensate shorter duration of illness. Since the progressive stage for the increased length of the posterior wall, which is expected to cease in a few years, application of a causes anterior shifting of the posterior dural wall cervical collar for three to four years generally has been and consequent compression of the cord against the advocated.[18] In selected patients, encouraging results posterior margin of adjacent vertebral bodies. This have also been obtained with surgical intervention, compression may cause microcirculatory disturbances which involves mainly cervical decompression and/or in the territory of the anterior spinal artery in the fusion with or without duraplasty.[19]

Neurology India | January-March 2008 | Vol 56 | Issue 1 25 Sonwalkar, et al.: Imaging features in Hirayama disease

Conclusion 8. Guigui P, Benoist M, Deburge A. Spinal deformity and instability after multilevel cervical laminectomy for spondylotic myelopathy. Spine 1998;23:440-7. Dynamic post contrast MRI evaluation of 9. Batzdorf U, Batzdorff A. Analysis of cervical spine curvature in patients cervicothoracic spine is a helpful method in arriving at with cervical spondylosis. Neurosurgery 1988;22:827-36. the correct diagnosis of Hirayama disease and should 10. Chi-Jen Chen, Hui-Ling Hsu, Ying-Chi Tseng, Rong-Kuo Lyu, Chiung- be an essential part of the protocol in cases with high Mei Chen, Ying-Chih Huang, et al. Hirayama flexion myelopathy: Neu- tral-position MR imaging findings-importance of loss of attachment. suspicion of motor neuron disease. Radiology 2004;231:39-44. 11. Kikuchi S, Tashiro K, Kitagawa K, Iwasaki Y, Abe H. A mechanism of Acknowledgment juvenile muscular atrophy localized in the hand and forearm (Hirayama’s disease): Flexion myelopathy with tight dural canal in flexion. Rinsho Shinkeigaku 1987;27:412-9. Authors wish to thank the Director, Sree Chitra Tirunal 12. Mukai E, Sobue I, Muto T, Takahashi A, Goto S. Abnormal radiological Institute for Medical Sciences and Technology, Trivandrum, findings on juvenile-type distal and segmental muscular atrophy of upper Kerala, India for providing the facilities and for the kind extremities. Rinsho Shinkeigaku 1985;25:620-6. permission to present this report. 13. Toma S, Shiozawa Z. Amyotrophic cervical myelopathy in adolescence. J Neurol Neurosurg Psychiatry 1995;58:56-64. References 14. Kira J, Ochi H. Juvenile muscular atrophy of the distal upper limb (Hirayama disease) associated with atopy. J Neurol Neurosurg Psychiatry 2001;70:798-801. 1. Hirayama K. Non-progressive juvenile spinal muscular atrophy of the distal 15. Schroder R, Keller E, Flacke S, Schmidt S, Pohl C, Klockgether T, et al. upper limb [Hirayama’s disease]. In: De Jong JM, Handbook of clinical neu- MRI findings in Hirayama’s disease: Flexion-induced cervical myelopathy rology. Vol 15. Elsevier: Amsterdam, the Netherlands; 1991. p. 107-20. or intrinsic motor neuron disease? J Neurol 1999;246:1069-74. 2. Hirayama K, Toyokura Y, Tsubaki T. Juvenile muscular atrophy of 16. Mukai E, Matsuo T, Muto T, Takahashi A, Sobue I. Magnetic resonance unilateral upper extremity: A new clinical entity. Psychiatr Neurol Jpn imaging of juvenile-type distal and segmental muscular atrophy of upper 1959;61:2190-7. extremities. Clin Neurol (Tokyo) 1987:27:99-107. 3. Gourie Devi M, Suresh TG, Shankar SK. Monomelic amyotrophy. Arch 17. Chi-Jen Chen, Chiung-Mei Chen, Chia-Lun Wu, Long-Sun Ro, Sien-Tsong Neurol 1984;41:388-94. Chen, Tsong-Hai Lee. Hirayama disease: MR diagnosis. AJNR Am J 4. Biondi A, Dormont D, Weitzner I Jr, Bouche P, Chaine P, Bories J. Neuroradiol 1998;19:365-8. MR imaging of the cervical cord in juvenile amyotrophy of distal upper 18. Tokumaru Y, Hirayama K. Cervical collar therapy for juvenile muscular extremity. AJNR Am J Neuroradiol 1989;10:263-8. atrophy of distal upper extremity [Hirayama disease]: Results from 38 5. Kohno M, Takahashi H, Yagishita A, Tanabe H. “Disproportion theory” of cases. Rinsho Shinkeigaku 2001;41:173-8. the cervical spine and spinal cord in patients with juvenile cervical flexion 19. Kohno M, Takahashi H, Ide K, Yamakawa K, Saitoh T, Inoue K. Surgi- myelopathy: A study comparing cervical magnetic resonance images with cal treatment for patients with cervical flexion myelopathy. J Neurosurg those of normal controls. Surg Neurol 1998;50:421-30. 1999;91:33-42. 6. Pradhan S, Gupta RK. Magnetic resonance imaging in juvenile asymmetric segmental spinal muscular atrophy. J Neurol Sci 1997;146:133-8. 7. Hirayama K, Tomonaga M, Kitano K, Yamada T, Kojima S, Arai K. Accepted on 24-10-2007 Focal cervical poliopathy causing juvenile muscular atrophy of distal Source of Support: Sree Chitra Tirunal Institute for Medical Sciences upper extremity: A pathological study. J Neurol Neurosurg Psychiatry and Technology, Kerala, India. Conß ict of Interest: None declared. 1987;50:285-90.

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