[Downloaded free from http://www.neurologyindia.com on Thursday, March 05, 2015, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor
[Figure 2] showed the entire shunt catheter lying inside the cranium, confirming the upward migration. The CT head also showed gross hydrocephalus, with a thin cortical mantle. Endoscopy-assisted removal of the migrated shunt with placement of a new shunt on the opposite side was planned. Under general anesthesia, a zero degree, rigid endoscope was introduced through the Kocher’s point inside the ventricle where the coiled and migrated shunt was seen. The tip of the shunt was found adherent to the choroid plexus. Attempts at separation of the adherent shunt resulted in mild intraventricular bleeding. In spite of constant saline irrigation and bipolar coagulation, the bleeding did not stop. Finally, the procedure was abandoned. An external ventricular drain (EVD) was placed inside the ventricular cavity. In the post-operative period, the child continued to be dull but the anterior fontanel remained lax. There was blood-tinged CSF emerging from the EVD. In spite of our best efforts, the child expired after 24 hours. The ventriculoperitoneal shunt procedure is a deceptively simple operation with a wide range of complications. Mechanical shunt failure is the most common complication. Complete intracranial migration of the ventriculo-peritoneal shunt is extremely rare [1] with a reported incidence of 0.1–0.4% of all shunt procedures. [2,3] On reviewing the database in PubMed and Google Scholar, we found only nine reported cases of total intracranial shunt migration. The factors that may be responsible for the migration of the shunt are malnourishment (less subcutaneous tissue for anchorage), anemia, sepsis, and a thin cortical mantle.[4] Excessive neck movements producing a windlass effect coupled with a large potential subgaleal space created for chamber positioning, dilated ventricles with negative suctioning pressure, or a positive intra-abdominal pressure may be responsible for the migration. [5] The technical factors include a large burr hole, larger dural opening, re-exploration leading to poor local tissue availability for anchorage, and an improper shunt securing. [6] The “Chabbra” shunt, having a cylindrical chamber can easily pass into the cranium.[7] Shunt migration may be prevented by taking precautions during the procedure, especially in high-risk patients. If the shunt has recently migrated proximally and is not considered adherent to the choroid plexus, the decision to remove it may be taken. In case of any suspicion of adherence of the shunt tubing to the choroid plexus or the ventricular ependyma, the shunt should be left in situ. Optimum creation of the subgaleal space for the shunt chamber, a smaller burr hole, a smaller dural opening, and proper anchorage of the chamber to the pericranium, are some of the measures that may be useful in obviating this complication.[8]
Rakesh Kumar Sharma, Mayank Bansal, Manish Agrawal, Ashok Gupta, Virendra deo Sinha Department of Neurosurgery, Sawai Man Singh Medical College, Jaipur, Rajasthan, India E-mail: [email protected]
References 1.
2. 3.
4.
5. 6. 7.
8.
Ghritlaharey RK, Budhwani KS, Shrivastava DK, Gupta G, Kushwaha AS, Chanchlani R, et al. Trans-anal protrusion of ventriculo-peritoneal shunt catheter with silent bowel perforation: Report of ten cases in children. Pediatr Surg Int 2007;23:575-80. Acharya R, Bhutani A, Saxena H, Madan VS. Complete migration of ventriculoperitoneal shunt into the ventricle. Neurol Sci 2002;23:75-7. Nadkarni TD, Menon RK, Dange NN, Desai KI, Goel A. Cranial migration of complete ventriculoperitoneal shunt assembly. J Clin Neurosci 2007;14:92-4. Oluwole KE, Abiodun AA. Complete intraventricular migration of a ventriculo-peritoneal shunt-A case report and brief literature review. Afr J Neurol Sci 2007;26:69-74. Agarwal A, Kakani A. Total migration of a ventriculo-peritoneal shunt catheter into the ventricles. J Pediatr Neurosci 2004;6:88-9. Ali MN, Atif SM, Khan S. Intracranial migration of a ventriculoperitoneal shunt. J Coll Physicians Surg Pak 2008;18:382-3. Choudhury AR. Avoidable factors that contribute to the complications of ventriculoperitoneal shunt in childhood hydrocephalus. Childs Nerv Syst 1990;6:346-9. Naik V, Phalak M, Chandra PS. Total intracranial shunt migration. J Neurosci Rural Pract 2013;4:95-6. Access this article online
Website:
Quick Response Code
www.neurologyindia.com DOI: 10.4103/0028-3886.152673 PMID: xxxxx
Intractable yawning caused by foramen magnum meningioma in a patient with neurofibromatosis type 2 Sir, Yawning is a stereotyped event seen in all vertebrates. Neural networks in the brainstem, autonomic nervous system, hypothalamus, and limbic system may be involved in the physiology of yawning. Tumor-related yawning has rarely been reported.[1,2] We present a rare case of a patient with a meningioma at foramen magnum who presented with intractable yawning. After total removal of the tumor, the recurrent yawning resolved completely.
Neurology India / January 2015 / Volume 63 / Issue 1
107
[Downloaded free from http://www.neurologyindia.com on Thursday, March 05, 2015, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor
protuberance and spinous process of C2. The tumor was dissected meticulously from the surrounding structures, and total excision was performed. It was histopathologically diagnosed as a psammomatous grade 1 meningioma.
Figure 1: Preoperative axial, coronal and sagittal T1 weighted contrast MRI of the patient showing homogenously enhancing mass lesion at the right side of foramen magnum extending down to C1 level. The lesion has shifted medulla oblongata
Figure 2: Postoperative axial, coronal and sagittal T1 weighted contrast MRI of the patient showing complete removal of the lesion
A 17-year-old girl was admitted with complaints of headache for 2 years. Her neurological examination revealed no abnormality other than hearing deficit in the right ear. Her magnetic resonance imaging (MRI) of the brain revealed hydrocephalus, bilateral vestibular schwannomas with the right one being 23 × 25 × 22 mm and the left one,14 × 8 × 6 mm, in size. She also had a falcine meningioma, that was 13 × 10 × 11 mm in size. Due to the presence of these bilateral vestibular schwannomas, she fulfilled the diagnostic criteria of neurofibromatosis type 2 (NF2). The patient underwent a ventriculo-peritoneal shunt and had radiosurgery for the right vestibular schwannoma and the falcine meningioma. The left vestibular schwannoma was left untreated as it was small in size and she had completely preserved hearing on that side. Fourteen months after the administration of stereotactic radiosurgery, the patient was again admitted to the hospital with the complaint of an involuntarily tilt of her head to the right side. Any attempts to correct the head position precipitated intractable yawning. She denied any sleeping disorder. The patient’s Epworth Sleepiness Scale was 2/24. Her cranial MRI, revealed an additional meningioma, 10 × 12 × 15 mm in size located toward the right side of the foramen magnum extending down to the C1 level [Figure 1]. The patient had no additional neurological deficits. She was operated through a posterior approach using a dorsal midline skin incision between the external occipital 108
The patient had no additional neurological deficits after surgery. A postoperative cranio-vertebral junction MRI confirmed complete tumor removal [Figure 2]. Following surgery, the patient was able to hold her head in the normal anatomical position. The phenomenon of intractable yawning on correction of head position had resolved completely. At follow-up after 9 months, there was no recurrence of the yawning phenomenon. Yawning involves wide opening of the mouth with concurrent deep inspiration, followed by a slow expiration. This complex and well-coordinated process resembles a classical reflex because once it starts, it is completed without any influence from an external stimuli.[2] However, yawning is not a simple reflex because it has a complex spatio-temporal organization with contributions from facial and respiratory muscles, and other component systems. It is proposed that the complex neuronal network that regulates yawning may be located in the brainstem reticular formation. This is supported by the observation that yawning also occurs in anencephalic newborns in whom only medulla oblongata exists as a brain structure.[3] In our patient, the symptom was probably associated with the meningioma that was located at the foramen magnum and C1 level. The involuntarily tilting of her head to the side of the tumor relieved pressure on medulla. This made her involuntarily maintain that position of the head prior to tumor removal. Yawning started with correction of the head position because this maneuver, in all likelihood, stretched the medulla and increased tumor pressure on it. In addition, yawning disappeared immediately after surgery and did not recur. There are two cases in the literature that report the phenomenon of intractable yawning associated with brain tumors. One is related to a hemangioblastoma of the IVth ventricle, and the other is related to a mature teratoma of supramedial cerebellum.[1,4] Our unique case reports yawning triggered by an extra-axial lesion at the level of foramen magnum.
Yaşar Bayri, Bahattin Tanrıkulu1, Fatih Bayraklı, Demet Yalçınkaya Koç2, Adnan Dağçınar Departments of Neurosurgery, Marmara University Faculty of Medicine, Istanbul, 1Neurosurgery, Marmara University Pendik Research and Training Hospital, Istanbul, 2Department of
Neurology India / January 2015 / Volume 63 / Issue 1
[Downloaded free from http://www.neurologyindia.com on Thursday, March 05, 2015, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor
Anesthesiology, Marmara University Institute of Neurological Sciences, Istanbul, Turkey E-mail: [email protected]
References 1.
2. 3. 4.
Saura H, Beppu T, Matsuura H, Asahi S, Uesugi N, Sasaki M, et al. Intractable yawning associated with mature teratoma of the supramedial cerebellum. J Neurosurg 2014;121:387-9. Argiolas A, Melis MR. The neuropharmacology of yawning. Eur J Pharmacol 1998;343:1-16. Askenasy JJ, Askenasy N. Inhibition of muscle sympathetic nerve activity during yawning. Clin Auton Res 1996;6:237-9. Arai K, Kita K, Komiyama A, Hirayama K, Saeki N, Nagao K. Progressive dysautonomia in hemangioblastoma in the region of the fourth ventricle. No To Shinkei 1986;38:195-200. Access this article online
Website:
Quick Response Code
www.neurologyindia.com
Examination revealed bradykinesia, generalized dystonia, prominent orofacial-dyskinesias, rigidity in all four limbs (most marked in the left upper limb) and preserved muscle stretch reflexes. Bilateral ocular fundii were normal with no Kayser-Fleischer ring. Investigations revealed a normal hemogram, liver and kidney function tests, normal serum creatine kinase, and normal arterial blood gas, lactate, and blood ammonia. Magnetic resonance imaging of the brain was normal. Serum copper and ceruloplasmin along with 24-hour urinary copper were normal. Urinary gas chromatography-mass spectroscopy and plasma acylcarnitine profile were normal. There was no response to oral L-Dopa. The child showed some improvement on transient midazolam infusion, trihexyphenidyl, oral diazepam, and physical rehabilitation. In view of rapid-onset dystonia with cranio-caudal gradient of progression, preserved cognition and normal magnetic resonance imaging of the brain, a possibility of rapid-onset dystonia Parkinsonism (Dystonia 12) were kept and genetic studies were planned.
DOI: 10.4103/0028-3886.152675 PMID: xxxxx
Subacute sclerosing panencephalitis masquerading as rapid-onset dystonia-Parkinsonism in a child
After 1-month of onset of symptoms, the child developed generalized myoclonic jerks with deterioration in cognition. The electroencephalography (EEG) showed generalized periodic epileptiform discharges [Figure 1]. Immunoglobulin G (IgG) antibody titer against measles was 104.0 in serum (normal
[Figure 2] showed the entire shunt catheter lying inside the cranium, confirming the upward migration. The CT head also showed gross hydrocephalus, with a thin cortical mantle. Endoscopy-assisted removal of the migrated shunt with placement of a new shunt on the opposite side was planned. Under general anesthesia, a zero degree, rigid endoscope was introduced through the Kocher’s point inside the ventricle where the coiled and migrated shunt was seen. The tip of the shunt was found adherent to the choroid plexus. Attempts at separation of the adherent shunt resulted in mild intraventricular bleeding. In spite of constant saline irrigation and bipolar coagulation, the bleeding did not stop. Finally, the procedure was abandoned. An external ventricular drain (EVD) was placed inside the ventricular cavity. In the post-operative period, the child continued to be dull but the anterior fontanel remained lax. There was blood-tinged CSF emerging from the EVD. In spite of our best efforts, the child expired after 24 hours. The ventriculoperitoneal shunt procedure is a deceptively simple operation with a wide range of complications. Mechanical shunt failure is the most common complication. Complete intracranial migration of the ventriculo-peritoneal shunt is extremely rare [1] with a reported incidence of 0.1–0.4% of all shunt procedures. [2,3] On reviewing the database in PubMed and Google Scholar, we found only nine reported cases of total intracranial shunt migration. The factors that may be responsible for the migration of the shunt are malnourishment (less subcutaneous tissue for anchorage), anemia, sepsis, and a thin cortical mantle.[4] Excessive neck movements producing a windlass effect coupled with a large potential subgaleal space created for chamber positioning, dilated ventricles with negative suctioning pressure, or a positive intra-abdominal pressure may be responsible for the migration. [5] The technical factors include a large burr hole, larger dural opening, re-exploration leading to poor local tissue availability for anchorage, and an improper shunt securing. [6] The “Chabbra” shunt, having a cylindrical chamber can easily pass into the cranium.[7] Shunt migration may be prevented by taking precautions during the procedure, especially in high-risk patients. If the shunt has recently migrated proximally and is not considered adherent to the choroid plexus, the decision to remove it may be taken. In case of any suspicion of adherence of the shunt tubing to the choroid plexus or the ventricular ependyma, the shunt should be left in situ. Optimum creation of the subgaleal space for the shunt chamber, a smaller burr hole, a smaller dural opening, and proper anchorage of the chamber to the pericranium, are some of the measures that may be useful in obviating this complication.[8]
Rakesh Kumar Sharma, Mayank Bansal, Manish Agrawal, Ashok Gupta, Virendra deo Sinha Department of Neurosurgery, Sawai Man Singh Medical College, Jaipur, Rajasthan, India E-mail: [email protected]
References 1.
2. 3.
4.
5. 6. 7.
8.
Ghritlaharey RK, Budhwani KS, Shrivastava DK, Gupta G, Kushwaha AS, Chanchlani R, et al. Trans-anal protrusion of ventriculo-peritoneal shunt catheter with silent bowel perforation: Report of ten cases in children. Pediatr Surg Int 2007;23:575-80. Acharya R, Bhutani A, Saxena H, Madan VS. Complete migration of ventriculoperitoneal shunt into the ventricle. Neurol Sci 2002;23:75-7. Nadkarni TD, Menon RK, Dange NN, Desai KI, Goel A. Cranial migration of complete ventriculoperitoneal shunt assembly. J Clin Neurosci 2007;14:92-4. Oluwole KE, Abiodun AA. Complete intraventricular migration of a ventriculo-peritoneal shunt-A case report and brief literature review. Afr J Neurol Sci 2007;26:69-74. Agarwal A, Kakani A. Total migration of a ventriculo-peritoneal shunt catheter into the ventricles. J Pediatr Neurosci 2004;6:88-9. Ali MN, Atif SM, Khan S. Intracranial migration of a ventriculoperitoneal shunt. J Coll Physicians Surg Pak 2008;18:382-3. Choudhury AR. Avoidable factors that contribute to the complications of ventriculoperitoneal shunt in childhood hydrocephalus. Childs Nerv Syst 1990;6:346-9. Naik V, Phalak M, Chandra PS. Total intracranial shunt migration. J Neurosci Rural Pract 2013;4:95-6. Access this article online
Website:
Quick Response Code
www.neurologyindia.com DOI: 10.4103/0028-3886.152673 PMID: xxxxx
Intractable yawning caused by foramen magnum meningioma in a patient with neurofibromatosis type 2 Sir, Yawning is a stereotyped event seen in all vertebrates. Neural networks in the brainstem, autonomic nervous system, hypothalamus, and limbic system may be involved in the physiology of yawning. Tumor-related yawning has rarely been reported.[1,2] We present a rare case of a patient with a meningioma at foramen magnum who presented with intractable yawning. After total removal of the tumor, the recurrent yawning resolved completely.
Neurology India / January 2015 / Volume 63 / Issue 1
107
[Downloaded free from http://www.neurologyindia.com on Thursday, March 05, 2015, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor
protuberance and spinous process of C2. The tumor was dissected meticulously from the surrounding structures, and total excision was performed. It was histopathologically diagnosed as a psammomatous grade 1 meningioma.
Figure 1: Preoperative axial, coronal and sagittal T1 weighted contrast MRI of the patient showing homogenously enhancing mass lesion at the right side of foramen magnum extending down to C1 level. The lesion has shifted medulla oblongata
Figure 2: Postoperative axial, coronal and sagittal T1 weighted contrast MRI of the patient showing complete removal of the lesion
A 17-year-old girl was admitted with complaints of headache for 2 years. Her neurological examination revealed no abnormality other than hearing deficit in the right ear. Her magnetic resonance imaging (MRI) of the brain revealed hydrocephalus, bilateral vestibular schwannomas with the right one being 23 × 25 × 22 mm and the left one,14 × 8 × 6 mm, in size. She also had a falcine meningioma, that was 13 × 10 × 11 mm in size. Due to the presence of these bilateral vestibular schwannomas, she fulfilled the diagnostic criteria of neurofibromatosis type 2 (NF2). The patient underwent a ventriculo-peritoneal shunt and had radiosurgery for the right vestibular schwannoma and the falcine meningioma. The left vestibular schwannoma was left untreated as it was small in size and she had completely preserved hearing on that side. Fourteen months after the administration of stereotactic radiosurgery, the patient was again admitted to the hospital with the complaint of an involuntarily tilt of her head to the right side. Any attempts to correct the head position precipitated intractable yawning. She denied any sleeping disorder. The patient’s Epworth Sleepiness Scale was 2/24. Her cranial MRI, revealed an additional meningioma, 10 × 12 × 15 mm in size located toward the right side of the foramen magnum extending down to the C1 level [Figure 1]. The patient had no additional neurological deficits. She was operated through a posterior approach using a dorsal midline skin incision between the external occipital 108
The patient had no additional neurological deficits after surgery. A postoperative cranio-vertebral junction MRI confirmed complete tumor removal [Figure 2]. Following surgery, the patient was able to hold her head in the normal anatomical position. The phenomenon of intractable yawning on correction of head position had resolved completely. At follow-up after 9 months, there was no recurrence of the yawning phenomenon. Yawning involves wide opening of the mouth with concurrent deep inspiration, followed by a slow expiration. This complex and well-coordinated process resembles a classical reflex because once it starts, it is completed without any influence from an external stimuli.[2] However, yawning is not a simple reflex because it has a complex spatio-temporal organization with contributions from facial and respiratory muscles, and other component systems. It is proposed that the complex neuronal network that regulates yawning may be located in the brainstem reticular formation. This is supported by the observation that yawning also occurs in anencephalic newborns in whom only medulla oblongata exists as a brain structure.[3] In our patient, the symptom was probably associated with the meningioma that was located at the foramen magnum and C1 level. The involuntarily tilting of her head to the side of the tumor relieved pressure on medulla. This made her involuntarily maintain that position of the head prior to tumor removal. Yawning started with correction of the head position because this maneuver, in all likelihood, stretched the medulla and increased tumor pressure on it. In addition, yawning disappeared immediately after surgery and did not recur. There are two cases in the literature that report the phenomenon of intractable yawning associated with brain tumors. One is related to a hemangioblastoma of the IVth ventricle, and the other is related to a mature teratoma of supramedial cerebellum.[1,4] Our unique case reports yawning triggered by an extra-axial lesion at the level of foramen magnum.
Yaşar Bayri, Bahattin Tanrıkulu1, Fatih Bayraklı, Demet Yalçınkaya Koç2, Adnan Dağçınar Departments of Neurosurgery, Marmara University Faculty of Medicine, Istanbul, 1Neurosurgery, Marmara University Pendik Research and Training Hospital, Istanbul, 2Department of
Neurology India / January 2015 / Volume 63 / Issue 1
[Downloaded free from http://www.neurologyindia.com on Thursday, March 05, 2015, IP: 202.177.173.189] || Click here to download free Android application for this journal Letters to Editor
Anesthesiology, Marmara University Institute of Neurological Sciences, Istanbul, Turkey E-mail: [email protected]
References 1.
2. 3. 4.
Saura H, Beppu T, Matsuura H, Asahi S, Uesugi N, Sasaki M, et al. Intractable yawning associated with mature teratoma of the supramedial cerebellum. J Neurosurg 2014;121:387-9. Argiolas A, Melis MR. The neuropharmacology of yawning. Eur J Pharmacol 1998;343:1-16. Askenasy JJ, Askenasy N. Inhibition of muscle sympathetic nerve activity during yawning. Clin Auton Res 1996;6:237-9. Arai K, Kita K, Komiyama A, Hirayama K, Saeki N, Nagao K. Progressive dysautonomia in hemangioblastoma in the region of the fourth ventricle. No To Shinkei 1986;38:195-200. Access this article online
Website:
Quick Response Code
www.neurologyindia.com
Examination revealed bradykinesia, generalized dystonia, prominent orofacial-dyskinesias, rigidity in all four limbs (most marked in the left upper limb) and preserved muscle stretch reflexes. Bilateral ocular fundii were normal with no Kayser-Fleischer ring. Investigations revealed a normal hemogram, liver and kidney function tests, normal serum creatine kinase, and normal arterial blood gas, lactate, and blood ammonia. Magnetic resonance imaging of the brain was normal. Serum copper and ceruloplasmin along with 24-hour urinary copper were normal. Urinary gas chromatography-mass spectroscopy and plasma acylcarnitine profile were normal. There was no response to oral L-Dopa. The child showed some improvement on transient midazolam infusion, trihexyphenidyl, oral diazepam, and physical rehabilitation. In view of rapid-onset dystonia with cranio-caudal gradient of progression, preserved cognition and normal magnetic resonance imaging of the brain, a possibility of rapid-onset dystonia Parkinsonism (Dystonia 12) were kept and genetic studies were planned.
DOI: 10.4103/0028-3886.152675 PMID: xxxxx
Subacute sclerosing panencephalitis masquerading as rapid-onset dystonia-Parkinsonism in a child
After 1-month of onset of symptoms, the child developed generalized myoclonic jerks with deterioration in cognition. The electroencephalography (EEG) showed generalized periodic epileptiform discharges [Figure 1]. Immunoglobulin G (IgG) antibody titer against measles was 104.0 in serum (normal
