Symptoms - of the Disease

Fact Explanation
Introduction Syringobulbia is a rare disease entity which is intimately related with syringomyelia. Syringobulbia is the formation of fluid filled cavity or so called syrinx in the medulla. whereas the syringomyelia is the similar occurrence in the spinal cord. The occurrence of syringobulbia can be due to the extension of syringomyelia, but it also occurs as an isolated illness. There would be a slit like gap in lower brain stem causing compression and related cranial nerve palsies. This syringobulbia cleft is found in investigations and in autopsy. The entire atiology for the illness is yet to be found and current evidence suggest it is closely related to the obstruction of the CSF circulation. These obstruction could be due to a congenital malformation such as Arnold-chiari malformation, inflammatory conditions such as basal arachnoiditis (post infectious, post irradiation, inflammatory), other masses at the base of skull such as arachnoid cysts, rheumatoid arthritis pannus, occipital encephalocele and tumors. Other causes would be related to the trauma or can be idiopathic. The disease syringobulbia can affect both gender with slight male predominance. It is usually present since birth and manifests before age of 30 years. [1][2][3][4]
Headache Headache is the commonest symptom and it is typically pounding posteriolateral (occipital) episodic pain. This headache is associated with sinister features such as aggravation with straining and vomiting. [1][5]
Dizziness or vertigo . Significant number of patients may complain the vertigo which is again episodic. This symptom is due to the involvement of vestibular nuclei and vestibular compartment of the 8th cranial nerve. This symptom could persists post operatively as well. [1]
Hearing impairment and tinnitus These symptoms could be either unilateral or bilateral. These are mainly due to the involvement of cochlear compartment of the 8th cranial nerves and related nuclei. when these are associated with vertigo diagnosis could mistakenly directed towards a pathology of ear. [1]
Voice disturbance The voice disturbances could be hoarseness, dysphonia or slurred speech. This could be due to the palatal palsy, cord palsy or involvement of tonque which are secondary to involvement of 9th, 10th, 11th and 12th cranial nerves. Children may rarely present with stridor even. [1]
Paraesthasia of the face Due to the involvement of trigeminal nerve there could be bilateral or unilateral sensory impairment for pain and temperature on face. [1][6]
Dysphagia Due to the palatal and pharyngeal wall paralysis patients may have neurological type of dysphagia and reflux. [1]
Ptosis This is drooping of eye lid which could be unilateral or rarely bilateral. This is usually partial and due to the involvement of ascending sympathetic bundle causing Horner's syndrome. [1]
Diplopia This is double vision due to involvement of extraocular nervous system either the particular cranial nerve or the nerve nucleus. The commonest is abducent nerve palsy due to nuclear involvement. [1]
Facial asymmetry The facial asymmetry with mouth deviation, absent of nasolabial folds, inability to close eyes, inability to make wrinkles on fore head is due to facial nerve and nuclear involvement. This can be either upper or lower motor type. [1]
Features of syringomyelia Syringomyelia mainly involves the cervical spine. Therefore the initial symptoms in syringomyelia involves the upper limb. Initial phases the pain and temperature sensation is impaired due to the compression on decussating spinothalamic tracts. The distribution ill be upper limbs with the trunk in a shawl like pattern. Later on patient may develop impairment of light touch, vibration and proprioception. Syrinx may extend to the anterior horns and may complain the motor weakness of limbs. Autonomic dysfunction also can be associated. Therefore when the features of syringobulbia is associated with these symptoms the extension of syringomyelia has to be considered. [2][3][4][7][8][9]
References
  1. MORGAN D, WILLIAMS B. Syringobulbia: a surgical appraisal. J Neurol Neurosurg Psychiatry [online] 1992 Dec, 55(12):1132-1141 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1015326
  2. HEISS JD, SUFFREDINI G, SMITH R, DEVROOM HL, PATRONAS NJ, BUTMAN JA, THOMAS F, OLDFIELD EH. Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery: Clinical article J Neurosurg Spine [online] 2010 Dec, 13(6):10.3171/2010.6.SPINE10200 [viewed 25 December 2014] Available from: doi:10.3171/2010.6.SPINE10200
  3. AVELLANEDA FERNáNDEZ A, ISLA GUERRERO A, IZQUIERDO MARTíNEZ M, AMADO VáZQUEZ ME, BARRóN FERNáNDEZ J, CHESA I OCTAVIO E, DE LA CRUZ LABRADO J, ESCRIBANO SILVA M, FERNáNDEZ DE GAMBOA FERNáNDEZ DE ARAOZ M, GARCíA-RAMOS R, GARCíA RIBES M, GóMEZ C, INSAUSTI VALDIVIA J, NAVARRO VALBUENA R, RAMóN JR. Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment) BMC Musculoskelet Disord [online] , 10(Suppl 1):S1 [viewed 25 December 2014] Available from: doi:10.1186/1471-2474-10-S1-S1
  4. AGRAWAL A, SHETTY MS, PANDIT L, SHETTY L, SRIKRISHNA U. Post-traumatic syringomyelia Indian J Orthop [online] 2007, 41(4):398-400 [viewed 25 December 2014] Available from: doi:10.4103/0019-5413.37006
  5. WILLIAMS B. Chronic herniation of the hindbrain Ann R Coll Surg Engl [online] 1981 Jan, 63(1):9-17 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2493879
  6. IRO H, BUMM K, WALDFAHRER F. Rehabilitation of the trigeminal nerve GMS Curr Top Otorhinolaryngol Head Neck Surg [online] :Doc12 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3201012
  7. WOLFE KC, POMA R. Syringomyelia in the Cavalier King Charles spaniel (CKCS) dog Can Vet J [online] 2010 Jan, 51(1):95-102 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2797361
  8. LANDI A, NIGRO L, MAROTTA N, MANCARELLA C, DONNARUMMA P, DELFINI R. Syringomyelia associated with cervical spondylosis: A rare condition World J Clin Cases [online] 2013 Jun 16, 1(3):111-115 [viewed 25 December 2014] Available from: doi:10.12998/wjcc.v1.i3.111
  9. RENE HUDSON B, COOK C, GOODE A. Identifying Myelopathy Caused by Thoracic Syringomyelia: A Case Report J Man Manip Ther [online] 2008, 16(2):82-88 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2565119

Symptoms - of Complications

Fact Explanation
History of fall This is due to the associated verigo and visual disturbances. So with the progression of the disease they may have history of falls.[1]
History of thermal and other injuries to the face This is due to the paraesthasia of the face due to involvement of the trigeminal nerve. [1][2]
History of reflux, regurgitation and aspiration Due to the palatal and pharyngeal wall malfunctioning patients may get aspirated and aspiration pneumonia.[1]
History of road traffic accidents These could be due to the associated visual and hearing disturbances as mentioned above. [3]
Other complications related to the features of syringomyelia These are thermal and other injuries to the limbs and the trunk due to sensory loss, motor weakness and autonomic involvement. Decubitas ulcers, contractures, orthostatic pneumonia, deep vein thrombosis can occur in extensive diseases due to bed bound status. Urinary incontinence, urinary tract infection and autonomic instability can be due to the autonomic involvement. [4][5][6]
References
  1. MORGAN D, WILLIAMS B. Syringobulbia: a surgical appraisal. J Neurol Neurosurg Psychiatry [online] 1992 Dec, 55(12):1132-1141 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1015326
  2. BORSOOK D. Neurological diseases and pain Brain [online] 2012 Feb, 135(2):320-344 [viewed 25 December 2014] Available from: doi:10.1093/brain/awr27
  3. SEWELL RA, POLING J, SOFUOGLU M. THE EFFECT OF CANNABIS COMPARED WITH ALCOHOL ON DRIVING Am J Addict [online] 2009, 18(3):185-193 [viewed 25 December 2014] Available from: doi:10.1080/10550490902786934
  4. SHENOY S, RAJA A. Acute aspiration pneumonia due to bulbar palsy: an initial manifestation of posterior fossa convexity meningioma J Neurol Neurosurg Psychiatry [online] 2005 Feb, 76(2):296-298 [viewed 25 December 2014] Available from: doi:10.1136/jnnp.2004.040444
  5. PLAUM PE, RIEMER G, FRøSLIE KF. Risk factors for pressure sores in adult patients with myelomeningocele – a questionnaire-based study Cerebrospinal Fluid Res [online] :14 [viewed 25 December 2014] Available from: doi:10.1186/1743-8454-3-14
  6. ABDELAAL E, WHITE P, LEWIS KE, REDFERN RM, HARRISON NK. Recurrent axillary vein thrombosis as a manifestation of syringomyelia J R Soc Med [online] 2003 Dec, 96(12):595-597 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC539662

Risk Factors

Fact Explanation
History of congenital malformation Arnold-chiari malformation is associated with syringomyelia and syringobulbia. Therefore the history of congenital malformations should be asked. [1][2]
History of meningitis, spinal tuberculosis, cerebral tumors These pathologies could lead to CSF flow obstruction and secondary syrinx formation. [3][4][5]
History of spinal trauma Trauma to the spine can be associated with syrinx formation. There fore such history has to be elicited. [6][7]
References
  1. MUSTAPHA B, CHKOURA K, ELHASSANI M, AHTIL R, AZENDOUR H, KAMILI ND. Difficult intubation in a parturient with syringomyelia and Arnold–Chiari malformation: Use of Airtraq™ laryngoscope Saudi J Anaesth [online] 2011, 5(4):419-422 [viewed 25 December 2014] Available from: doi:10.4103/1658-354X.87274
  2. AVELLANEDA FERNáNDEZ A, ISLA GUERRERO A, IZQUIERDO MARTíNEZ M, AMADO VáZQUEZ ME, BARRóN FERNáNDEZ J, CHESA I OCTAVIO E, DE LA CRUZ LABRADO J, ESCRIBANO SILVA M, FERNáNDEZ DE GAMBOA FERNáNDEZ DE ARAOZ M, GARCíA-RAMOS R, GARCíA RIBES M, GóMEZ C, INSAUSTI VALDIVIA J, NAVARRO VALBUENA R, RAMóN JR. Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment) BMC Musculoskelet Disord [online] , 10(Suppl 1):S1 [viewed 25 December 2014] Available from: doi:10.1186/1471-2474-10-S1-S1
  3. RAMANATHAN SR, AHLUWALIA T. Rare complication: Acute syringomyelia due to tuberculoma and tubercular meningitis J Neurosci Rural Pract [online] 2010, 1(2):123-125 [viewed 25 December 2014] Available from: doi:10.4103/0976-3147.71734
  4. CAPLAN LR, NOROHNA AB, AMICO LL. Syringomyelia and arachnoiditis. J Neurol Neurosurg Psychiatry [online] 1990 Feb, 53(2):106-113 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC487945
  5. WANG CC. Adult Medulloblastoma Associated with Syringomyelia: A Case Report Cancer Biol Med [online] 2012 Jun, 9(2):137-140 [viewed 25 December 2014] Available from: doi:10.3969/j.issn.2095-3941.2012.02.011
  6. SQUIER MV, LEHR RP. Post-traumatic syringomyelia. J Neurol Neurosurg Psychiatry [online] 1994 Sep, 57(9):1095-1098 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073135
  7. AGRAWAL A, SHETTY MS, PANDIT L, SHETTY L, SRIKRISHNA U. Post-traumatic syringomyelia Indian J Orthop [online] 2007, 41(4):398-400 [viewed 25 December 2014] Available from: doi:10.4103/0019-5413.37006

Signs - of the Disease

Fact Explanation
Voice disturbances Patient may have a hoarse voice or slurred speech due to the involvement palate, tongue and vocal cords. [1]
Nystagmus Nystagmus will be obvious in majority in rotatory pattern. This is greatly due to the involvement of vestibular nuclei and the 8th cranial nerve. [1][2]
Horner's syndrome Due to the compression on ascending sympathetic trunk Horner's syndrome can occur. This includes partial ptosis, meiosis and hemifacial loss of seating. [1]
Parasthaesia Due to the involvement of trigeminal nerve and the related nuclei pain and temperature sensation of the face can be affected. [1]
Facial nerve palsy Due to the involvement of either the facial nerve or the nucleus patients may have upper motor or lower motor type facial nerve palsy. [1]
Opthalmoplegia Involvement of abducent nerve nucleus can cause impaired abduction of the eye and opthalmoplegia. [1]
Palatal palsy Due to the involvement of 9th, 10th nerves palatal palsy can occur. On examination palsy of the uvula, absent gag reflex and palatal analgesia can be elicited. [1]
Accessory nerve palsy Not commonly few patients may have weakness accessory nerve causing weak sternocleidomastoid and traphizius. [1]
Hypoglossal nerve palsy Hypoglossal nerve palsy causes tongue fasciculation and tongue palsy.[1]
Features of syringomyelia Dissociated sensory impairement involving cervical level and below is obvious. Initially the pain and temperature is impaired due to affected decussating spinothalamic tracts. Later in the disease cause other sensory modalities are also involved including light touch, vibration and proprioception. Sensory signs are obvious bilaterally though it can be asymmetrical. Motor weakness can be elicited in latter part of the disease with hyporelexia of arms and spastic paraplegia of lower limbs. Hyporeflexia in upper limb is due to anterior horn involvement due to expanding syrinx. Bladder bowel incontinence can be seen with involvement of anal sphincter tone. [3][4][5]
Ear examination Ear examination should be carried out to exclude ear pathologies causing similar symptoms. [6]
Cerebellar examination Complete cerebellar examination ill reveal associated cerebellar involvement as well. [7]
References
  1. MORGAN D, WILLIAMS B. Syringobulbia: a surgical appraisal. J Neurol Neurosurg Psychiatry [online] 1992 Dec, 55(12):1132-1141 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC101532
  2. GONG JW, JIANG J, SUN ZH. Jerky see-saw nystagmus in internuclear ophthalmoplegia from a lower pontine lesion Int J Ophthalmol [online] , 5(5):652-654 [viewed 25 December 2014] Available from: doi:10.3980/j.issn.2222-3959.2012.05.24
  3. WOLFE KC, POMA R. Syringomyelia in the Cavalier King Charles spaniel (CKCS) dog Can Vet J [online] 2010 Jan, 51(1):95-102 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2797361
  4. LANDI A, NIGRO L, MAROTTA N, MANCARELLA C, DONNARUMMA P, DELFINI R. Syringomyelia associated with cervical spondylosis: A rare condition World J Clin Cases [online] 2013 Jun 16, 1(3):111-115 [viewed 25 December 2014] Available from: doi:10.12998/wjcc.v1.i3.111
  5. RENE HUDSON B, COOK C, GOODE A. Identifying Myelopathy Caused by Thoracic Syringomyelia: A Case Report J Man Manip Ther [online] 2008, 16(2):82-88 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2565119
  6. THOMPSON TL, AMEDEE R. Vertigo: A Review of Common Peripheral and Central Vestibular Disorders Ochsner J [online] 2009, 9(1):20-26 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3096243
  7. NELSON JA, VIIRRE E. The Clinical Differentiation of Cerebellar Infarction from Common Vertigo Syndromes West J Emerg Med [online] 2009 Nov, 10(4):273-277 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2791733

Signs - of Complications

Fact Explanation
Evidence of thermal and other injuries to the face These injuries can happen due to unawareness about the injurious stimuli due to parasthaesia of the face with trigeminal nerve involvement. [1][2]
Corneal ulcerations The facial nerve palsy will impair the closure of eye and causes corneal ulceration due to persistent exposure of the eye to exterior. [1][3][4]
Evidence of aspiration Due to the palatal and pharyngeal wall palsy the swallowing doesn't take place properly and causes aspiration. Coarse capitations will be audible during auscultation. [5]
Other complications of syringomyelia Progressive weakness causes inability to mobilize and patients may become either wheelchair or bed bound. In mobility causes and there can be features of bed sores, hypopstatic pneumonia, deep vein thrombosis. Contractures may also develop in prolonged illness. [5][6][7]
Generalized cachexia Cachexia may cause due to chronic ill health and dysphagia and associated complications. [8]
Respiratory distress This may be an emergency and life threatening. This occurs due to the intercostal muscle weakness and phrenic nerve weakness. [9][10]
References
  1. MORGAN D, WILLIAMS B. Syringobulbia: a surgical appraisal. J Neurol Neurosurg Psychiatry [online] 1992 Dec, 55(12):1132-1141 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1015326
  2. YILMAZ HH, GORMEZ O, HASTAR E, YILDIRIM D, AKSOY MC. Garlic Burn in a Patient with Trigeminal Neuralgia: A Case Report Eur J Dent [online] 2010 Jan, 4(1):88-90 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2798796
  3. ARAMIDEH M, KOELMAN J, DEVRIESE P, VANDERWERF F, SPEELMAN J. Thixotropy: a novel explanation for the cause of lagophthalmos after peripheral facial nerve palsy Br J Ophthalmol [online] 2002 Aug, 86(8):839 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC177124
  4. SOCOLOVSKY M, PáEZ MD, MASI GD, MOLINA G, FERNáNDEZ E. Bell's palsy and partial hypoglossal to facial nerve transfer: Case presentation and literature review Surg Neurol Int [online] :46 [viewed 25 December 2014] Available from: doi:10.4103/2152-7806.95391
  5. RAGHAVENDRAN K, NEMZEK J, NAPOLITANO LM, KNIGHT PR. Aspiration-Induced lung injury Crit Care Med [online] 2011 Apr, 39(4):818-826 [viewed 25 December 2014] Available from: doi:10.1097/CCM.0b013e31820a856b
  6. WAKE WT. Pressure Ulcers: What Clinicians Need to Know Perm J [online] 2010, 14(2):56-60 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2912087
  7. THURAIRAJAH P, BROWNE S, BONDESON J. A painful swollen calf J R Soc Med [online] 2003 May, 96(5):236-237 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC539479
  8. SAKUMA K, YAMAGUCHI A. Sarcopenia and cachexia: the adaptations of negative regulators of skeletal muscle mass J Cachexia Sarcopenia Muscle [online] 2012 Jun, 3(2):77-94 [viewed 25 December 2014] Available from: doi:10.1007/s13539-011-0052-4
  9. BULLOCK R, TODD NV, EASTON J, HADLEY D. Isolated central respiratory failure due to syringomyelia and Arnold-Chiari malformation. BMJ [online] 1988 Dec 3, 297(6661):1448-1449 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1835174
  10. AL BASHAPSHE A, BHATIA H, AZIZ S. Acute respiratory failure as a first manifestation of syringomyelia Lung India [online] 2010, 27(2):93-95 [viewed 25 December 2014] Available from: doi:10.4103/0970-2113.63614

Differential Diagnoses

Fact Explanation
Stroke In strokes the onset is sudden and mostly occurs in elderly patients. Symptoms may improve with the time and the limb weakness is greatly unilateral. Imaging ith CT or MRI may conclude the diagnosis.[1]
Space occupying lesions of the brain Tumors such as meningioma, gliomas, medulla blastomas, vascular malformations may cause progressive dissociative neurological manifestations according to the site, involving the cranial nerves and limb. Imaging would help for the diagnosis. [2][3]
Cerebro pontine angle tumours CP angle tumours also causes involvement of 5th, 7th and 8th cranial nerves with similar symptoms. The ultimate diagnosis can be reached by proper imaging.[4]
Ear pathologies Ear pathologies such as Meniere's disease, vestibular neuritis, middle ear infection can cause hearing and vertigo. Again imaging is essential for the conclusive diagnosis. [5]
Hydrocephalus Again progressive symptoms with headache, unsteady gait, incontinence will be seen. May be secondary to an obstructing lesion. Clinical signs of cranial nerve and limb involvement is not that much obvious. Imaging is a must for definitive differentiation.[6][7]
Guillen barre syndrome Mainly an ascending paralysis with out objective sensory weakness. Disease process is faster than in syringomyelia or syringobulbia. Nerve conduction study gives the diagnosis. [8]
Motor neuron disease Progressive weakness with bulbar involvement can be seen with combined upper and lower motor features. Opthalomoplegia doesn't occur typically. Nerve conduction study and EMG can give the diagnosis. [9]
Spinal Cord Hemorrhage, Spinal Cord Infarction, Spinal Cord Trauma, Spinal Epidural Abscess Onset is rapid again with dissociative symptoms. History and comorbidities will give a clue. Imaging is essential for the differentiation. Cranial nerves are spared.[10][11]
References
  1. DAVIS S, LEES K, DONNAN G. Treating the acute stroke patient as an emergency: current practices and future opportunities Int J Clin Pract [online] 2006 Apr, 60(4):399-407 [viewed 25 December 2014] Available from: doi:10.1111/j.1368-5031.2006.00873.x
  2. WILLIAMS B, TIMPERLEY WR. Three cases of communication syringomyelia secondary to midbrain gliomas. J Neurol Neurosurg Psychiatry [online] 1977 Jan, 40(1):80-88 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC492608
  3. WANG CC. Adult Medulloblastoma Associated with Syringomyelia: A Case Report Cancer Biol Med [online] 2012 Jun, 9(2):137-140 [viewed 25 December 2014] Available from: doi:10.3969/j.issn.2095-3941.2012.02.011
  4. MALLUCCI C, WARD V, CARNEY A, O'DONOGHUE G, ROBERTSON I. Clinical features and outcomes in patients with non-acoustic cerebellopontine angle tumours J Neurol Neurosurg Psychiatry [online] 1999 Jun, 66(6):768-771 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1736400
  5. THOMPSON TL, AMEDEE R. Vertigo: A Review of Common Peripheral and Central Vestibular Disorders Ochsner J [online] 2009, 9(1):20-26 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3096243
  6. SHPRECHER D, SCHWALB J, KURLAN R. Normal Pressure Hydrocephalus: Diagnosis and Treatment Curr Neurol Neurosci Rep [online] 2008 Sep, 8(5):371-376 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2674287
  7. DE OLIVEIRA MF, PINTO FC, NISHIKUNI K, BOTELHO RV, LIMA AM, ROTTA JM. Revisiting Hydrocephalus as a Model to Study Brain Resilience Front Hum Neurosci [online] :181 [viewed 25 December 2014] Available from: doi:10.3389/fnhum.2011.00181
  8. WINER JB. Guillain Barré syndrome Mol Pathol [online] 2001 Dec, 54(6):381-385 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1187127
  9. TALBOT K. Motor neurone disease Postgrad Med J [online] 2002 Sep, 78(923):513-519 [viewed 25 December 2014] Available from: doi:10.1136/pmj.78.923.513
  10. MACKENZIE A, LAING R, SMITH C, KAAR G, SMITH F. Spinal epidural abscess: the importance of early diagnosis and treatment J Neurol Neurosurg Psychiatry [online] 1998 Aug, 65(2):209-212 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2170211
  11. KIM JS, LEE SH. Spontaneous Spinal Subarachnoid Hemorrhage with Spontaneous Resolution J Korean Neurosurg Soc [online] 2009 Apr, 45(4):253-255 [viewed 25 December 2014] Available from: doi:10.3340/jkns.2009.45.4.253

Investigations - for Diagnosis

Fact Explanation
CT scan After detailed clinical history and examination imaging is the investigation is syringomyelia or syringobulbia is suspected. CT scan shows the skeletal structure better than the MRI and can be carried out to detect bone deformities such as atlantoaxial dislocation, vertebral fractures or lesions. [1][2]
MRI scan MRI scan is the investigation of choice for the diagnosis. It has to be done in both T1 and T2 weighted images and have to look at on both axial and transverse views. when a tumor or post traumatic syrinx is suspected scanning should be contrasted with Gadolinium. MRI shows the extent of cyst or cysts with associated deformities and tumors. Magnetic resonance angiography is helpful in detecting vascular malformations. [1][2]
Myelography Myelography can be carried out combined with CT when MRI can not be performed. [3]
Nerve conduction studies This is greatly important in excluding other diagnosis such as Guillen Barre syndrome, Motor neuron disease, anterior horn cell disease. [4]
Pure tone audiogram and tympanometry These tests can be performed to assess the status of ear and the degree of deafness [5]
References
  1. MORGAN D, WILLIAMS B. Syringobulbia: a surgical appraisal. J Neurol Neurosurg Psychiatry [online] 1992 Dec, 55(12):1132-1141 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1015326
  2. IBRAHIM GM, KAMALI-NEJAD T, FEHLINGS MG. Arachnoiditis ossificans associated with syringomyelia: An unusual cause of myelopathy Evid Based Spine Care J [online] 2010 Aug, 1(2):46-51 [viewed 25 December 2014] Available from: doi:10.1055/s-0028-1100914
  3. GNANALINGHAM KK, JOSHI SM, SABIN I. Thoracic arachnoiditis, arachnoid cyst and syrinx formation secondary to myelography with Myodil, 30 years previously Eur Spine J [online] 2006 Oct, 15(Suppl 5):661-663 [viewed 25 December 2014] Available from: doi:10.1007/s00586-006-0204-y
  4. PUGDAHL K, FUGLSANG‐FREDERIKSEN A, DE CARVALHO M, JOHNSEN B, FAWCETT PR, LABARRE‐VILA A, LIGUORI R, NIX WA, SCHOFIELD IS. Generalised sensory system abnormalities in amyotrophic lateral sclerosis: a European multicentre study J Neurol Neurosurg Psychiatry [online] 2007 Jul, 78(7):746-749 [viewed 25 December 2014] Available from: doi:10.1136/jnnp.2006.098533
  5. KENNEDY V, WILSON C, STEPHENS D. When a normal hearing test is just the beginning J R Soc Med [online] 2006 Aug, 99(8):417-420 [viewed 25 December 2014] Available from: doi:10.1258/jrsm.99.8.417

Investigations - for Management

Fact Explanation
To assess the fitness for surgery The management is essentially surgical. So for the preparation of surgery which involve the brain stem thorough investigations should be done. Those are as follow.[1][2]
FBC To see the haemoglobin and the platelet level pre-operatively.[1][2]
Serum creatine and electrolytes Pre-operative renal function and electrolyte level should be known. Renal functions has to be monitored and electrolytes has to be optimized.[1][2]
Thromboelastogram Platelet functions status can be assessed and optimized for minimum bleeding. This is really indicated if patient is [3] on anti-platelets[4]
Clotting profile. PT/INR and APTT Clotting status shoul be assessed and optimized for minimum bleeding. [3]
ECG and 2D Echocardiogram In patient with suggestive history of cardiac compromise or complications, Cardiac status has to be assessed and optimized pre-operatively.[2][5]
Chest xray and lung function tests In patients which suggestive history of lung diseases and suspected compromise,Respiratory status has to be assessed and optimized pre-operatively. [1][2]
Serum protein levels In patients who are undernourished and has underlying conditions which are susceptible to affect their body regulation of protein Serum protein levels should be assessed and optimized prior to the surgery. [6]
References
  1. ZAMBOURI A. Preoperative evaluation and preparation for anesthesia and surgery Hippokratia [online] 2007, 11(1):13-21 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2464262
  2. RANASINGHE P, PERERA YS, SENARATNE JA, ABAYADEERA A. Preoperative testing in elective surgery: Is it really cost effective? Anesth Essays Res [online] 2011, 5(1):28-32 [viewed 25 December 2014] Available from: doi:10.4103/0259-1162.84177
  3. GOH KY, TSOI W, FENG C, WICKHAM N, POON WS. Haemostatic changes during surgery for primary brain tumours J Neurol Neurosurg Psychiatry [online] 1997 Sep, 63(3):334-338 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2169692
  4. RIVARD GE, BRUMMEL K, MANN KG, FAN L, HOFER A, COHEN E. Evaluation of the Profile of Thrombin Generation during the Process of Whole Blood Clotting as Assessed by Thromboelastography J Thromb Haemost [online] 2005 Sep, 3(9):2039-2043 [viewed 25 December 2014] Available from: doi:10.1111/j.1538-7836.2005.01513.x
  5. QUADER N, RIGOLIN VH. Two and three dimensional echocardiography for pre-operative assessment of mitral valve regurgitation Cardiovasc Ultrasound [online] , 12(1):42 [viewed 25 December 2014] Available from: doi:10.1186/1476-7120-12-42
  6. WARNOLD I, LUNDHOLM K. Clinical significance of preoperative nutritional status in 215 noncancer patients. Ann Surg [online] 1984 Mar, 199(3):299-305 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1353396

Management - General Measures

Fact Explanation
Emergency management Respiratory paralysis could occur due to the intercostal muscle involvement and phrenic nerve involvement. So if presented with respiratory distress securing of airway, endotracheal intubation and ventilator support may be needed. [1][2]
Health education This should include the rarity of the disease and possible complications of the disease. Patient and the family members should be thoroughly emphasized about the complexity of the surgical procedure and possible sinister complications before the surgery. [1][3]
Naso Gastric feeding and nutrition when the patients are having severe dysphagia they should be fed via NG tube. when necessary parenteral nutrition may also be needed for pre-operative optimization. [4]
Covering the eyes and arteficial tears when there is a facial nerve weakness there is a risk of corneal ulceration due to open eye lids. So the covering of affected eye and administration of artificial tears may prevent the corneal ulcers. In persistent cases tarsoraphy can be done. [5]
Balance training, physiotherapy and rehabilitation, speech therapy Balancing should be trained because due to the vertigo there is a risk of fall. Physiotherapy has to be arranged in patients with facial nerve palsy (including for electrical nerve stimulation) and motor weakness with syringomyelia. Rehabilitation is essential in inoperable cases. Speech theraphy has to be arranged for patients with palatal and vocal cord involvement. [6]
Avoidance of injurious agents to the face and the regions with paraesthaesia. Patient should be aware about the parasthaesia of the affected region and have to take measures to prevent injuries. Such as avoiding exposure to sunlight.[6]
Urinary cathterization and bladder bowel care and prevention of bed sores when the patient is having autonomic dysfunction due to syringomyelia, bladder and bowel care has to be arranged. Bed sore should be prevented by frequent turning, using and air or water mattress. [7]
Prevention of deep vein thrombosis This has to to be done in immobilizing patients. It can be achieved by physiotherapy, mobilization, good hydration, stocking and anticoagulation with lo molecular weight heparin. [8]
Analgesics and muscle relaxants These can be used for symptomatic management [9]
References
  1. MORGAN D, WILLIAMS B. Syringobulbia: a surgical appraisal. J Neurol Neurosurg Psychiatry [online] 1992 Dec, 55(12):1132-1141 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1015326
  2. NOGUéS MA, GENé R, ENCABO H. Risk of sudden death during sleep in syringomyelia and syringobulbia. J Neurol Neurosurg Psychiatry [online] 1992 Jul, 55(7):585-589 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC489171
  3. FINK C, DIENER MK, BRUCKNER T, MüLLER G, PAULSEN L, KELLER M, BüCHLER MW, KNEBEL P. Impact of preoperative patient education on prevention of postoperative complications after major visceral surgery: study protocol for a randomized controlled trial (PEDUCAT trial) Trials [online] :271 [viewed 25 December 2014] Available from: doi:10.1186/1745-6215-14-271
  4. SURA L, MADHAVAN A, CARNABY G, CRARY MA. Dysphagia in the elderly: management and nutritional considerations Clin Interv Aging [online] 2012:287-298 [viewed 25 December 2014] Available from: doi:10.2147/CIA.S23404
  5. FINSTERER JOSEF. Management of peripheral facial nerve palsy. Eur Arch Otorhinolaryngol [online] December, 265(7):743-752 [viewed 25 December 2014] Available from: doi:10.1007/s00405-008-0646-4
  6. AVELLANEDA FERNáNDEZ A, ISLA GUERRERO A, IZQUIERDO MARTíNEZ M, AMADO VáZQUEZ ME, BARRóN FERNáNDEZ J, CHESA I OCTAVIO E, DE LA CRUZ LABRADO J, ESCRIBANO SILVA M, FERNáNDEZ DE GAMBOA FERNáNDEZ DE ARAOZ M, GARCíA-RAMOS R, GARCíA RIBES M, GóMEZ C, INSAUSTI VALDIVIA J, NAVARRO VALBUENA R, RAMóN JR. Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment) BMC Musculoskelet Disord [online] , 10(Suppl 1):S1 [viewed 25 December 2014] Available from: doi:10.1186/1471-2474-10-S1-S1
  7. SHAH D, BADLANI G. Treatment of Overactive Bladder and Incontinence in the Elderly Rev Urol [online] 2002, 4(Suppl 4):S38-S43 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1476020
  8. GALSON SK. PREVENTION OF DEEP VEIN THROMBOSIS AND PULMONARY EMBOLISM Public Health Rep [online] 2008, 123(4):420-421 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2430635
  9. BORSOOK D. Neurological diseases and pain Brain [online] 2012 Feb, 135(2):320-344 [viewed 30 December 2014] Available from: doi:10.1093/brain/awr271

Management - Specific Treatments

Fact Explanation
Surgical management Specific management is essentially surgical. Any secondary cause has to be detected if present. Surgery should not be delayed to prevent complications. Surgical options would be rerouting the CSF via shunts or tubes. Fluoroscopic guided aspiration also can be attempted. Measures have to be taken to eneucleate the underlying tumors and correct the malformations if practical.[1]
References
  1. MORGAN D, WILLIAMS B. Syringobulbia: a surgical appraisal. J Neurol Neurosurg Psychiatry [online] 1992 Dec, 55(12):1132-1141 [viewed 25 December 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1015326