[Downloaded free from http://www.neurologyindia.com on Tuesday, June 09, 2015, IP: 61.16.135.116]
Letters to Editor
b
a
Figure 2: (a) An intraoperative microscopic view demonstrating the VA fenestration consisting of a medial (black asterisk) and lateral (white asterisk) portion, through which a hypoglossal nerve (arrow) passes. The REZ of the facial nerve is compressed by the medial portion of the VA fenestration (black asterisk). Arrowhead: Left facial nerve. (b) An intraoperative microscopic view showing displacement of the medial side of the VA fenestration (black asterisk) from the REZ by a dissector. Arrow: Left hypoglossal nerve; arrowhead: Left facial nerve
any part of the fenestration due to incomplete fusion. Furthermore, the hypoglossal nerve sometimes passes through the VA fenestration. A macroscopic investigation demonstrated that the hypoglossal nerve passed through the VA fenestration in 4 of 7 cases of VA fenestration.[3] The authors suggested that this anatomical structure is caused by a remnant of the primitive lateral basilovertebral anastomosis in the fetal period. Padget reported that the anastomosis is related to the formation of AICA and PICA and appears transiently lateral to the VA surrounding the hypoglossal nerve and then disappears. VA fenestration is speculated to be a remnant of the anastomosis. When VA is transposed during MVD, excessive tension to the hypoglossal nerve should be avoided to prevent injury to the nerve.[4]
Shunsuke Nomura1, Takakazu Kawamata1,2, Tadasuke Tominaga1, Yoshikazu Okada2 Department of Neurosurgery, Tokyo Women’s Medical
1
University, Yachiyo Medical Center, Chiba, 2Department of Neurosurgery, Tokyo Women’s Medical University, Tokyo, Japan E‑mail: [email protected]
References 1.
2.
3. 4.
444
Gruber TJ, Ogilvy CS, Hauck EF, Levy EI, Hopkins LN, Siddiqui AH. Endovascular treatment of a large aneurysm arising from a basilar trunk fenestration using the waffle‑cone technique. Neurosurgery 2010;67:ons140‑4. Kim JP, Chung JC, Chang WS, Chung SS, Chang JW. Outcomes of surgical treatment for hemifacial spasm associated with the vertebral artery: Severity of compression, indentation, and color change. Acta Neurochir (Wien) 2012;154:501‑8. Higashi N, Sone C, Yasutaka S, Seki Y. On the fenestration of the vertebral and basilar arteries. Kaibogaku Zasshi 1992;67:308‑16. Padget DH. The development of the cranial arteries in the human embryo. Contrib Embryol 1948;32:205‑61.
Access this article online Website:
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www.neurologyindia.com DOI: 10.4103/0028-3886.158260 PMID: xxxxx
Wall‑eyed bilateral internuclear ophthalmoplegia Sir, The introduction of the term ‘wall‑eyed bilateral internuclear ophthalmoplegia’ (WEBINO) is attributed to Lubow, according to Hoyt and Daroff in 1971. It is a rare neuro‑ophthalmic condition describing a disorder of ocular motility and characterized by large angle exotropia in primary gaze and internuclear ophthalmoplegia (INO) bilaterally.[1] The etiology of the lesion differs greatly and includes inflammatory, toxic, infectious, degenerative, traumatic, postsurgical, demyelinating and neoplastic conditions, but midbrain infarction and autoimmune diseases (e.g., multiple sclerosis) are the most frequently reported lesion.[2] A 19‑year‑old lady with no previous comorbidities presented to the emergency department with complaints of tingling sensation in the left upper limb, vomiting, uneasiness, stuttering speech, and painless diplopia on vertical gaze that had commenced a day earlier. The patient had no previous history of any systemic disease.
Neurology India / May 2015 / Volume 63 / Issue 3
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Letters to Editor
On general examination, she was conscious and following commands and her vitals parameters were stable with a pulse rate of 100/min and a blood pressure of 150/80 mmHg. Her neurological examination revealed a left‑sided upper motor neuron type of facial palsy. There was no motor weakness. However, all her reflexes were exaggerated with bilateral extensor plantars. The ocular motility examination showed bilateral exotropia (i.e. “wall‑eyed”) in the primary position. Attempted horizontal saccades revealed bilateral INO (i.e., bilateral adduction palsy with dissociative abducting eye nystagmus). Down gaze was intact, but up gaze and convergence were defective. The patient also developed skew deviation of the eyes with up beating nystagmus. The pupils measured 4 mm in dim illumination and constricted
normally to direct light. There was no ptosis [Figure 1]. Her routine laboratory parameters (complete blood count, random blood sugar, coagulation profile, fasting lipid profile, liver, renal and thyroid function tests), were within normal range. However, her plasma homocysteine levels were found to be raised (15.92 μmol/L [normal range: 3.7–13.9 μmol/L]). The brain magnetic resonance imaging demonstrated an irregular‑shaped acute infarct in the midline of mesencephalic tegmentum involving bilateral medial longitudinal fasciculus (MLF), the periaqueductal gray matter, and the oculomotor nuclei [Figure 2]. On the clinical examination and neuroimaging, the diagnosis of a midbrain infarct secondary to basilar artery thrombosis
c
b
d
a
e Figure 1: Ocular motility of the patients in all the directions (a) primary gaze; (b) left gaze; (c) upward gaze; (d) right gaze; and, (e) downward gaze
a
d
b
c
e
Figure 2: (a-c) Magnetic resonance imaging of the brain revealed areas of diffusion restriction seen in caudal midbrain involving the central region of the midbrain and right crus of the pons. This area appears bright on the diffusion-weighted image with low apparent diffusion coefficient value (yellow arrow). (d and e) Magnetic resonance angiography showed the thrombosed basilar artery (yellow arrow)
Neurology India / May 2015 / Volume 63 / Issue 3
445
[Downloaded free from http://www.neurologyindia.com on Tuesday, June 09, 2015, IP: 61.16.135.116]
Letters to Editor
leading to WEBINO syndrome was made. The patient was managed conservatively with antiplatelet therapy and ocular motility exercises. After 6 weeks of treatment, her dizziness and diplopia subsided, and vertical gaze restriction improved, but the right‑sided ocular adduction palsy persisted. The oculomotor alterations exhibited by the patient correspond to a clinical condition described only occasionally in the literature and known by the acronym WEBINO. This peculiar form of bilateral INO is characterized by a restriction of adduction in both the eyes with accompanying contralateral abducting nystagmus, exotropia in primary gaze position, and impairement of convergence. It may also be associated with vertical gaze palsy, up‑beat nystagmus, and skew deviation.[3] The pathogenesis of WEBINO syndrome is still a matter of debate. Bilateral MLF damage producing bilateral INO, in conjunction with abnormalities of the medial rectus subnuclei (MRSN) of the ventral oculomotor nuclear complex, culminating in bilateral exotropia and bilateral convergence impairment, may be considered as the possible cause of WEBINO syndrome. Since MRSN neurons are scattered within the MLF at the level of the ponto‑mesencephalic junction, an injury at this level could affect both these structures. Variable degree of vertical gaze abnormalities in WEBINO is explained by the frequent concomitant involvement of rostral interstitial nucleus of MLF or the interstitial nucleus of Cajal in the midbrain‑thalamic region.[3,4] Hyperhomocysteinemia is one of the causes of thrombus formation.[5] The present case had hyperhomocysteinemia. There was also thrombus formation within the mid and distal basilar artery, which could have led to the WEBINO syndrome. Management of WEBINO is centered on eliminating diplopia, which is one of the most common complaints reported by patients. So far, the top two modalities for managing diplopia are surgery for strabismus and botulinum toxin injections.[3]
Abhishek Agarwal, Sony Vyas, Subodh Banzal, Rajesh Kumar Jha Department of Medicine, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India E‑mail: [email protected]
References 1.
2. 446
Hoyt WF, Daroff RB. Supranuclear disorders of ocular control systems in man: Clinical, anatomical, and physiological correlations. In: Bach‑Y‑Rita P, Colins CC, editors. The Control of Eye Movements. New York: Academic Press; 1971. p. 223‑6. Wu YT, Cafiero‑Chin M, Marques C. Wall‑eyed bilateral inter nuclear
3. 4. 5.
ophthalmoplegia: Review of pathogenesis, diagnosis, prognosis and management. Clin Exp Optom 2015;98:25‑30. Chakravarthi S, Kesav P, Khurana D. Wall‑eyed bilateral inter nuclear ophthalmoplegia with vertical gaze palsy. QJM 2014;107:165. Beh SC, Frohman EM. WEBINO and the return of the King’s Speech. J Neurol Sci 2012;315:153‑5. Undas A, Brozek J, Szczeklik A. Homocysteine and thrombosis: From basic science to clinical evidence. Thromb Haemost 2005;94:907‑15. Access this article online
Website:
Quick Response Code
www.neurologyindia.com DOI: 10.4103/0028-3886.158263 PMID: xxxxx
Hypokalemic paralysis as a primary presentation of Fanconi’s syndrome and distal renal tubular acidosis in a patient with primary Sjogren’s syndrome Sir, Primary Sjogren’s Syndrome (pSS) is a disease of the exocrine glands presenting with manifestations related to dry eyes and dry mouth.[1,2] Non‑exocrine organ systems may also be involved, including the skin, lungs, gastrointestinal tract, central and peripheral nervous system, musculo‑skeletal apparatus, and kidney.[3,4] Hypokalemic paralysis is a very rare primary presentation of pSS. We report a case of a 39‑year old female patient, a known case of seizure disorder on regular treatment with oral sodium valproate 200mg twice daily and oral phenobarbitone 30 mg twice daily. She presented with sudden onset of quadriparasis that occurred a day prior to her admission. There was no history of trauma, insect or animal bites, loose motions, headache or altered sensorium. She was afebrile with a pulse rate of 78/min in sinus rhythm and a BP of 120/80 mm/Hg. She was shifted to the intensive care unit. On systemic examination, she had hypotonia with grade I power in all four limbs. All deep tendon reflexes were depressed and the superficial reflexes including the plantar reflex were absent. Her neck holding was, however, present. The systemic examination was within normal limits.
Neurology India / May 2015 / Volume 63 / Issue 3
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Letters to Editor
b
a
Figure 2: (a) An intraoperative microscopic view demonstrating the VA fenestration consisting of a medial (black asterisk) and lateral (white asterisk) portion, through which a hypoglossal nerve (arrow) passes. The REZ of the facial nerve is compressed by the medial portion of the VA fenestration (black asterisk). Arrowhead: Left facial nerve. (b) An intraoperative microscopic view showing displacement of the medial side of the VA fenestration (black asterisk) from the REZ by a dissector. Arrow: Left hypoglossal nerve; arrowhead: Left facial nerve
any part of the fenestration due to incomplete fusion. Furthermore, the hypoglossal nerve sometimes passes through the VA fenestration. A macroscopic investigation demonstrated that the hypoglossal nerve passed through the VA fenestration in 4 of 7 cases of VA fenestration.[3] The authors suggested that this anatomical structure is caused by a remnant of the primitive lateral basilovertebral anastomosis in the fetal period. Padget reported that the anastomosis is related to the formation of AICA and PICA and appears transiently lateral to the VA surrounding the hypoglossal nerve and then disappears. VA fenestration is speculated to be a remnant of the anastomosis. When VA is transposed during MVD, excessive tension to the hypoglossal nerve should be avoided to prevent injury to the nerve.[4]
Shunsuke Nomura1, Takakazu Kawamata1,2, Tadasuke Tominaga1, Yoshikazu Okada2 Department of Neurosurgery, Tokyo Women’s Medical
1
University, Yachiyo Medical Center, Chiba, 2Department of Neurosurgery, Tokyo Women’s Medical University, Tokyo, Japan E‑mail: [email protected]
References 1.
2.
3. 4.
444
Gruber TJ, Ogilvy CS, Hauck EF, Levy EI, Hopkins LN, Siddiqui AH. Endovascular treatment of a large aneurysm arising from a basilar trunk fenestration using the waffle‑cone technique. Neurosurgery 2010;67:ons140‑4. Kim JP, Chung JC, Chang WS, Chung SS, Chang JW. Outcomes of surgical treatment for hemifacial spasm associated with the vertebral artery: Severity of compression, indentation, and color change. Acta Neurochir (Wien) 2012;154:501‑8. Higashi N, Sone C, Yasutaka S, Seki Y. On the fenestration of the vertebral and basilar arteries. Kaibogaku Zasshi 1992;67:308‑16. Padget DH. The development of the cranial arteries in the human embryo. Contrib Embryol 1948;32:205‑61.
Access this article online Website:
Quick Response Code
www.neurologyindia.com DOI: 10.4103/0028-3886.158260 PMID: xxxxx
Wall‑eyed bilateral internuclear ophthalmoplegia Sir, The introduction of the term ‘wall‑eyed bilateral internuclear ophthalmoplegia’ (WEBINO) is attributed to Lubow, according to Hoyt and Daroff in 1971. It is a rare neuro‑ophthalmic condition describing a disorder of ocular motility and characterized by large angle exotropia in primary gaze and internuclear ophthalmoplegia (INO) bilaterally.[1] The etiology of the lesion differs greatly and includes inflammatory, toxic, infectious, degenerative, traumatic, postsurgical, demyelinating and neoplastic conditions, but midbrain infarction and autoimmune diseases (e.g., multiple sclerosis) are the most frequently reported lesion.[2] A 19‑year‑old lady with no previous comorbidities presented to the emergency department with complaints of tingling sensation in the left upper limb, vomiting, uneasiness, stuttering speech, and painless diplopia on vertical gaze that had commenced a day earlier. The patient had no previous history of any systemic disease.
Neurology India / May 2015 / Volume 63 / Issue 3
[Downloaded free from http://www.neurologyindia.com on Tuesday, June 09, 2015, IP: 61.16.135.116]
Letters to Editor
On general examination, she was conscious and following commands and her vitals parameters were stable with a pulse rate of 100/min and a blood pressure of 150/80 mmHg. Her neurological examination revealed a left‑sided upper motor neuron type of facial palsy. There was no motor weakness. However, all her reflexes were exaggerated with bilateral extensor plantars. The ocular motility examination showed bilateral exotropia (i.e. “wall‑eyed”) in the primary position. Attempted horizontal saccades revealed bilateral INO (i.e., bilateral adduction palsy with dissociative abducting eye nystagmus). Down gaze was intact, but up gaze and convergence were defective. The patient also developed skew deviation of the eyes with up beating nystagmus. The pupils measured 4 mm in dim illumination and constricted
normally to direct light. There was no ptosis [Figure 1]. Her routine laboratory parameters (complete blood count, random blood sugar, coagulation profile, fasting lipid profile, liver, renal and thyroid function tests), were within normal range. However, her plasma homocysteine levels were found to be raised (15.92 μmol/L [normal range: 3.7–13.9 μmol/L]). The brain magnetic resonance imaging demonstrated an irregular‑shaped acute infarct in the midline of mesencephalic tegmentum involving bilateral medial longitudinal fasciculus (MLF), the periaqueductal gray matter, and the oculomotor nuclei [Figure 2]. On the clinical examination and neuroimaging, the diagnosis of a midbrain infarct secondary to basilar artery thrombosis
c
b
d
a
e Figure 1: Ocular motility of the patients in all the directions (a) primary gaze; (b) left gaze; (c) upward gaze; (d) right gaze; and, (e) downward gaze
a
d
b
c
e
Figure 2: (a-c) Magnetic resonance imaging of the brain revealed areas of diffusion restriction seen in caudal midbrain involving the central region of the midbrain and right crus of the pons. This area appears bright on the diffusion-weighted image with low apparent diffusion coefficient value (yellow arrow). (d and e) Magnetic resonance angiography showed the thrombosed basilar artery (yellow arrow)
Neurology India / May 2015 / Volume 63 / Issue 3
445
[Downloaded free from http://www.neurologyindia.com on Tuesday, June 09, 2015, IP: 61.16.135.116]
Letters to Editor
leading to WEBINO syndrome was made. The patient was managed conservatively with antiplatelet therapy and ocular motility exercises. After 6 weeks of treatment, her dizziness and diplopia subsided, and vertical gaze restriction improved, but the right‑sided ocular adduction palsy persisted. The oculomotor alterations exhibited by the patient correspond to a clinical condition described only occasionally in the literature and known by the acronym WEBINO. This peculiar form of bilateral INO is characterized by a restriction of adduction in both the eyes with accompanying contralateral abducting nystagmus, exotropia in primary gaze position, and impairement of convergence. It may also be associated with vertical gaze palsy, up‑beat nystagmus, and skew deviation.[3] The pathogenesis of WEBINO syndrome is still a matter of debate. Bilateral MLF damage producing bilateral INO, in conjunction with abnormalities of the medial rectus subnuclei (MRSN) of the ventral oculomotor nuclear complex, culminating in bilateral exotropia and bilateral convergence impairment, may be considered as the possible cause of WEBINO syndrome. Since MRSN neurons are scattered within the MLF at the level of the ponto‑mesencephalic junction, an injury at this level could affect both these structures. Variable degree of vertical gaze abnormalities in WEBINO is explained by the frequent concomitant involvement of rostral interstitial nucleus of MLF or the interstitial nucleus of Cajal in the midbrain‑thalamic region.[3,4] Hyperhomocysteinemia is one of the causes of thrombus formation.[5] The present case had hyperhomocysteinemia. There was also thrombus formation within the mid and distal basilar artery, which could have led to the WEBINO syndrome. Management of WEBINO is centered on eliminating diplopia, which is one of the most common complaints reported by patients. So far, the top two modalities for managing diplopia are surgery for strabismus and botulinum toxin injections.[3]
Abhishek Agarwal, Sony Vyas, Subodh Banzal, Rajesh Kumar Jha Department of Medicine, Sri Aurobindo Medical College and PG Institute, Indore, Madhya Pradesh, India E‑mail: [email protected]
References 1.
2. 446
Hoyt WF, Daroff RB. Supranuclear disorders of ocular control systems in man: Clinical, anatomical, and physiological correlations. In: Bach‑Y‑Rita P, Colins CC, editors. The Control of Eye Movements. New York: Academic Press; 1971. p. 223‑6. Wu YT, Cafiero‑Chin M, Marques C. Wall‑eyed bilateral inter nuclear
3. 4. 5.
ophthalmoplegia: Review of pathogenesis, diagnosis, prognosis and management. Clin Exp Optom 2015;98:25‑30. Chakravarthi S, Kesav P, Khurana D. Wall‑eyed bilateral inter nuclear ophthalmoplegia with vertical gaze palsy. QJM 2014;107:165. Beh SC, Frohman EM. WEBINO and the return of the King’s Speech. J Neurol Sci 2012;315:153‑5. Undas A, Brozek J, Szczeklik A. Homocysteine and thrombosis: From basic science to clinical evidence. Thromb Haemost 2005;94:907‑15. Access this article online
Website:
Quick Response Code
www.neurologyindia.com DOI: 10.4103/0028-3886.158263 PMID: xxxxx
Hypokalemic paralysis as a primary presentation of Fanconi’s syndrome and distal renal tubular acidosis in a patient with primary Sjogren’s syndrome Sir, Primary Sjogren’s Syndrome (pSS) is a disease of the exocrine glands presenting with manifestations related to dry eyes and dry mouth.[1,2] Non‑exocrine organ systems may also be involved, including the skin, lungs, gastrointestinal tract, central and peripheral nervous system, musculo‑skeletal apparatus, and kidney.[3,4] Hypokalemic paralysis is a very rare primary presentation of pSS. We report a case of a 39‑year old female patient, a known case of seizure disorder on regular treatment with oral sodium valproate 200mg twice daily and oral phenobarbitone 30 mg twice daily. She presented with sudden onset of quadriparasis that occurred a day prior to her admission. There was no history of trauma, insect or animal bites, loose motions, headache or altered sensorium. She was afebrile with a pulse rate of 78/min in sinus rhythm and a BP of 120/80 mm/Hg. She was shifted to the intensive care unit. On systemic examination, she had hypotonia with grade I power in all four limbs. All deep tendon reflexes were depressed and the superficial reflexes including the plantar reflex were absent. Her neck holding was, however, present. The systemic examination was within normal limits.
Neurology India / May 2015 / Volume 63 / Issue 3
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
