History

Fact Explanation
Introduction to the disease Syringomyelia is a generic term used to discribe a development of fluid filled cavity or syrinx within the spinal cord.[1].Four types of syringomyelia has been found. They are as follows. 1. Type 1 is syringomyelia with obstruction of the foramen magnum and dilation of the central spinal canal. A) Associated with Chiari type I malformation B) Associated with other obstructive lesions of the foramen magnum 2.Type II is Idiopathic or syringomyelia without obstruction of the foramen magnum 3.Type III is syringomyelia with other diseases of the spinal cord. A) Tumors of spinal cord B)Traumatic myelopathy C) Pachy meningitis and Spinal arachnoiditis D) Myelomalacia following compression of the spinal cord 4.Type IV is Pure hydromyelia,which is found to be associated with hydrocephalus. [7].
History of trauma or tumor The exact pathogenisis of syringomyelia not clearly understood. It can occur following trauma or secondary to a tumor or either due to some craniocervical, intracerebral pathology or may be idiopathic.[1]. It is suggest that trauma may cause increased tonsillar ectopia which leads to disruption of previous equilibrium. This may cause either ischemic dysfunction of the tonsils and brainstem or malcirculation of cerebrospinal fluid around the cervicomedullary junction.[3].
History of foramen magnum or spinal pathologies The common form, primary syrinx formation within the cord parenchyma is found to be associated with either foramen magnum or spinal pathologies. They obstruct the CSF flow. Chiari I malformation, cervical stenosis, basilar impression, occipital encephalocele, and arachnoiditis belong to this category.[2].
Pain Clinical symptoms of syringomyelia may vary, depending on its longitudinal location along the neuraxis and its position within the cord. Classically, the first clinical manifestation is pain. Type of pain can be vary. It can be either central type pain , pain localized to the syrinx site or radicular pain. Expansion of syrinx can produce exacerbation of symptoms and also postural changes or Valsalva like maneuvers found to be exacerbate symptoms.[2].
Paresthesia Paresis and paresthesias followed by pain. And some studies suggest that sensory disturbance is the common manifestation of syringomyelia.[2].
Sensory disturbance Common presentation. It can appears in a “cape-like distribution,”.It can be either in a pattern of an ascending sensory level, or as a dissociated sensory loss.[2].
Headache One of the most frequent symptom ohf affected individuals is Suboccipital headache. Coughing, sneezing, or bowel movement like valsalva manoeuvres may cause exacerbation of the headache. Sometimes headache may present with non specific features.[7].
Weakness and wasting of the hands Chronic progressive neuro degenerative or developmental disorder of the spinal cord in syringomyelia can be manifest as painless weakness and wasting of the hands and arm. It is a characteristic feature of the disease and may leads to segmental sensory loss of the dissociated type.[4].
Sudden or progressive respiratory disorders Resulted in case of Arnold-Chiari malformation, alone or in combination with syringomyelia. It may cause central alveolar hypoventilation sleep apnea as well as acute respiratory insufficiency due to aspiration in dysphagic patients.[4].
Central apnoea Compression of descending and ascending afferent pathways in the anterolateral region of the cervical spinal cord by the syrinx will leads to central apnea in patients with syringomyelia.[5].
Stiffness Stiffness in the back, shoulders, arms, or legs is another manifestation of syringomyelia.[6].
Paresis A manifestation of syringomyelia. Paraparesis and even tetraparesis in the setting of a cervical syrinx have been reported specially in the setting of a malfunctioning CSF shunts.[6]. Paresis is a symptom occurs due to loss of function, occurs when damage is severe and due to total destruction of nerve conduction.[7].
Allodynia An irritative symptom occurs when the lesion is less severe or when nerve conduction not totally disrupted. Allodynia causes a painful response to a non-painful stimulus. For an example brushing the skin with a cotton wad or sponge will produce pain.[7].
Hyperalgesia Another symptom occurred due to irritation. It will cause an excessively painful response to a mildly painful stimulus, even for a slight prick. [7].
Speech disturbances Speech disturbances found to be occurred in patients with Chiari syndrome and syringomyelia. Paralysis, weakness, or incoordination of the speech musculature may resulted due to craniocervical malformations can cause speech disorders. It can be either motor or functional disorders affecting phonation,articulation, resonance, and prosody. [7].
Dysphagia Swallowing difficulty may associated with syringomyelia.[7]. Those bulbar symptoms may resulted due to rostral extension of the lesion. This symptoms can leads to complications such as aspiration, cyanosis, stridor as well as apnea.[2].
Neck pain Frequent and occurs in the absence of radicular distribution. Affected individuals may complain continuous, burning, deep-seated discomfort in the shoulders, nape, chest, and upper limbs. Neck pain is found to be worsen with Valsalva manoeuvres.[7].
Vertigo Seen in patient with syringomyelia and associated chiari type 1 malformation. Positional vertigo is commonly occurs. Vertigo may be triggered by head movements.[7].
Tinnitus and aural fullness An otological symptom sometimes present in affected individuals.[7].
Hearing loss Occasionally some affected individuals may have sensoneuronal type hearing loss. Usually found in ontological evaluations. Sometimes associated with peripheral vestibulopathy. Symptoms usually have an insidious onset and a progressive course.[7].
Syncope Hind brain herniation is sometimes associated with syringomyelia. Headache and syncope are common feature in chronic hindbrain herniation, following coughing, sneezing or other physical exertion. Craniospinal pressure dissociation is one of the mechanism that explain the occurrence of headache. The temporarily higher intracranial pressure in hind brain herniation can impact the hernia at the foramen magnum resulting in a long lasting pressure difference. This interfere with the medullary baroreceptor reflex or by dysfunction of the midbrain reticular activating system causing syncope. Failure of the autonomic system due to excavation of the lateral horns of the grey matter of the thoracic cord is another explanation of syncope in syringomyelia.[8].
Anhidrosis Sympathetic nervous system innervates the eccrine sweat glands. Fibers begin in the cerebral cortex traverse the hypothalamus, and then cross in the medulla to the lateral horn of the spinal cord and then leave the spinal cord by forming synapses with the neurons which innervate the sweat gland. So that the presence of a cortical lesion, will produce contralateral anhidrosis where the lesion in medulla will produces either contralateral or ipsilateral anhidrosis. If there is any lesion distal to the medulla may leads to ipsilateral anhidrosis. In syringomyelia , anhidrosis is reported in face and upper part of the body.[1].
Charcot arthropathy It is a arthropathy resulting from loss of proprioception, pain and temperature perception. It is seen among 25% of patients with syringomyelia. About 80% of syringomyelic arthropathies involve the upper limb. Neurotraumatic and the neurovascular theories are used to describe the pathogenesis of charcot arthropathy. In Syringomyelia formation of cavities in lower cervical and upper thoracic segments may predispose to development of . Charcot arthropathy.[10]. It can develop early or late in the course of syringomyelia and the symptoms of neuropathic shoulder arthropathy may mask the symptoms of syringomyelia.[11].
Central cord syndrome (CCS) CCS is characterized by disproportionately more motor impairment of the upper extremities than lower extremities. and also bladder dysfunction, urinary retention, and varying degrees of sensory loss below the level of the lesion” can be identified.[9],[7].
Sphincter disturbance Less common symptom among syringomyelia patients.[2].
Sudden cardiopulmonary arrest or death Patients with CM-I and syringomyelia are at a higher risk of acute onset and serious neurological deterioration. Some of those patient can develop sudden cardiopulmonary arrest or death due to formation of syrinx causing rapid tonsilar herniation.[3].
References
  1. KUMAR S, VERMA A. Holocord syrinx presenting as hemi anhidrosis Indian Dermatol Online J [online] 2013, 4(2):109-111 [viewed 20 September 2014] Available from: doi:10.4103/2229-5178.110632
  2. PORENSKY P, MURO K, GANJU A. Nontraumatic Cervicothoracic Syrinx as a Cause of Progressive Neurologic Dysfunction J Spinal Cord Med [online] 2007, 30(3):276-281 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2031960
  3. OISHI M, HAYASHI Y, KITA D, FUKUI I, SHINOHARA M, HEISS JD, HAMADA JI. Rapidly progressing monoparesis caused by Chiari malformation type I without syringomyelia Surg Neurol Int [online] :79 [viewed 20 September 2014] Available from: doi:10.4103/2152-7806.113355
  4. 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 20 September 2014] Available from: doi:10.4103/0970-2113.63614
  5. 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 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC489171
  6. SCHNEIDER B, BIRTHI P, SALLES S. Arnold-Chiari 1 malformation type 1 with syringohydromyelia presenting as acute tetraparesis: A case report J Spinal Cord Med [online] 2013 Mar, 36(2):161-165 [viewed 20 September 2014] Available from: doi:10.1179/2045772312Y.0000000047
  7. 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 20 September 2014] Available from: doi:10.1186/1471-2474-10-S1-S1
  8. HAMPTON F, WILLIAMS B, LOIZOU LA. Syncope as a presenting feature of hindbrain herniation with syringomyelia. J Neurol Neurosurg Psychiatry [online] 1982 Oct, 45(10):919-922 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC491597
  9. MCKINLEY W, SANTOS K, MEADE M, BROOKE K. Incidence and Outcomes of Spinal Cord Injury Clinical Syndromes J Spinal Cord Med [online] 2007, 30(3):215-224 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2031952
  10. PANAGARIYA A, SHARMA AK. Bilateral Charcot arthropathy of shoulder secondary to syringomyelia: An unusual case report Ann Indian Acad Neurol [online] 2012, 15(3):202-204 [viewed 20 September 2014] Available from: doi:10.4103/0972-2327.99718
  11. GUNAY CUNEYD, ATALAR EBRU, ATAOGLU BAYBARS. An Unusual Presentation of Charcot Arthropathy Caused by Syringomyelia Mimicking a Soft Tissue Tumor. Case Reports in Orthopedics [online] 2014 December, 2014:1-4 [viewed 20 September 2014] Available from: doi:10.1155/2014/760861

Examination

Fact Explanation
Deficit of heat and /or cold sensations Dissociated sensory loss can be seen. [5].Syringomyelia is characterized by intraspinal cavity that selectively affect affects the spinothalamic tract lead into a predominant deficit of heat and/or cold sensations.[1].
Scoliosis About 25% of patients with chiari type 1 malformation may have a short, or bull-like neck.Levoscoliosis may be present if there is an association with syringomyelia.[2].It has been reported that 25–85% of patients with syringomyelia also have scoliosis. The exact parthenogenesis is not clear but scoliosis can be a musculoskeletal sign of an underlying syrinx. However some suggest that there are radicular lesions and CSF imbalance at the convex side of the scoliosis, ultimately give rise to the syrinx. And another hypothesis suggest that asymmetric syrinx expansion affects the medial motor nuclei in the anterior horn of the spinal cord leads to imbalance of the trunk muscles causing development of a scoliosis.[3].
Hypereflexia, Occurs due to upper motor neuron involvement in disease. Commonly seen in lower limbs.[2].
Spasticity Another manifestation of upper motor neuron involvement. It is a motor symptom occurs due to irritation, occurs when lesion is less severe or nerve conduction is not totally disrupted.[2].
Babinski reflex Babinski reflex positive in case of upper motor neuron involvement in expanding syrinx.[2].
Atrophy and weakness Occurs due to lower motor neuron involvement in syringomyelia. Commonly involves hands.[2].
Fasciculations Another manifestation of Lower motor neuron involvement.It is also a motor symptom occurs due to irritation, occurs when lesion is less severe or nerve conduction is not totally disrupted.[2].
Areflexia Commonly demonstrated in upper limbs.Occurs as a result of lower motor neuron involvement.[2].
Nystagmus A manifestation of cerebellar involvement.[2]. It occurs due to hindbrain herniation.[4].The most frequent other ocular manifestations are diplopia, photopsia, blurred vision, and photophobia. [2].
Ataxia, and dysmetria Another two signs due to cerebellar involvement.[2].
Vocal cord paralysis and soft palate weakness This is a presentation due to lower cranial nerves involvement .In severe cases compression of the spinal cord or medulla oblongata causes involvement of the motor or sensory pathways, or even lower cranial nerves.[2].
Lingual atrophy, cricopharyngeal achalasia and facial hypoaesthesia Features lower cranial nerve involvement. Lower cranial nerves found to be affected in 15-25% of cases.[2].
Absent gag reflex It is the most frequent physical sign that indicates the involvement of the lower cranial nerves.[2].
Trigeminal Paresthesia Found in syringobulbia that occurs due to syrinx extend into the medulla. Though the trigeninal nerve disorders are commonly reported, it is unlikely to nerve itself directly get affected. Trigeminal symptoms often originate from the spinal sensory nucleus in the upper cervical cord and also the accompanying fibers which form the descending trigeminal roof. Upward extension of the syrinx may leads to dissociated type sensory loss to progress in all three divisions, converging upon the tip of the nose and upper lip. [4].
Tongue atrophy and fasciculation Found in patients with syringobulbia as minifestation of brain stem sign in syringomyelia.Tongue found to be frequently involved specially in the late stage of the disease.Atrophy and fasciculation of the tongue can be seen.[4].
References
  1. DUCREUX D.. Mechanisms of central neuropathic pain: a combined psychophysical and fMRI study in syringomyelia. Brain [online] 2006 February, 129(4):963-976 [viewed 20 September 2014] Available from: doi:10.1093/brain/awl016
  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 20 September 2014] Available from: doi:10.1186/1471-2474-10-S1-S1
  3. KANGA I, WONG JJ, STERN PJ. Detection of syringomyelia in a pediatric patient with mild scoliosis: a case report J Can Chiropr Assoc [online] 2014 Mar, 58(1):16-23 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3924508
  4. MORGAN D, WILLIAMS B. Syringobulbia: a surgical appraisal. J Neurol Neurosurg Psychiatry [online] 1992 Dec, 55(12):1132-1141 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1015326
  5. PORENSKY P, MURO K, GANJU A. Nontraumatic Cervicothoracic Syrinx as a Cause of Progressive Neurologic Dysfunction J Spinal Cord Med [online] 2007, 30(3):276-281 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2031960

Differential Diagnoses

Fact Explanation
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) AIDP, so called Guillain-Barre syndrome is commonly present with sub acute muscular weakness with areflexia arising from the lower limbs. This gradually begins to ascend in more than 90% of cases. Autonomic dysfunction also can occur. Sympathetic and parasympathetic over activity simultaneously, rarely found in some patients.[1].
Amyotrophic Lateral Sclerosis (ALS) ALS is a progressive neurodegenerative disease. It involve both upper motor neuron as well as lower motor neurons. UMN signs include hyperreflexia, , increased muscle tone, weakness and extensor plantar response. weakness, muscle wasting, hyporeflexia, fasciculations and muscle cramps, are the common LMN signs. Patient may commonly present with asymmetric focal weakness of the extremities or bulbar findings such as dysarthria and dysphagia. The familial amyotrophic lateral sclerosis found to be inherited in an autosomal dominant, autosomal recessive as well as X-linked manner.[2].
Ankylosing spondylitis (AS) It is a seronegative spondyloarthropathy that predominantly seen in young men. It is difficult to diagnose in the early stages. Modified New York criteria is used for clinical diagnosis. It includes low back pain and stiffness for more than 3 months which improves with exercise but not relieved with rest, Limitation of lumbar spine motions in both frontal and sagittal planes and Limitation of chest expansion relative to normal values corrected for age and sex. radiological criteria include sacroilitis grade 2 bilaterally or grade 3-4 unilaterally. If radiological findings associated with at least 1 clinical criteria it is called definite AS.[3].
Brainstem gliomas Brainstem tumor are defined as lesions occurring in the midbrain, the pons, or the medulla oblongata and brainstem glioma is the most frequent tumor of the region. It has bimodal age distribution.The clinical symptoms and signs found to be closely related to the localization of the tumor. They may present with Visual disturbances, limb weakness, and gait disorders.Cranial nerve dysfunction and long-tract signs can found in brain stem lesions. Some patients may present withfacial myokymia or hemifacial spasm with a long history of facial nerve palsy. Involvement of V, VI, VIII, cranial nerves and palatal palsy as well as Pyramidal weakness, cerebellar signs can found during examination.[4].
Central pontine myelinolysis (CPM) CPM is an subacute demyelinating condition of the brain stem. It can occur as a complication of the treatment of patients with chronic hyponatremia due to rapid correction of sodium levels.[5]. They may initialy present with seizures due to hyponatraemia. later on present with dysarthria and dysphagia secondary to corticobulbar fibre involvement.Corticospinal tract involvement may leads to a flaccid quadriparesis which later becomes spastic. Pupillary as well as oculomotor abnormalities may resulted due to involvement of pons.[6].
Multiple sclerosis It is an inflammatory, demyelinating, neurodegenerative disorder of the central nervous system (CNS) of unknown etiology. Peak age of onset is around 20-40 years and found in twice common in women than in men. Around 30% of patients develop sensory disturbance of the limbs.Acute and sub acute motor dysfunction of the limbs found around 13% of patients and partial or complete visual loss found around 15% of patients. Other features include diplopia and gait dysfunction. Disease can be relapsing-remitting or progressive, and may be severe or mild. It can either involve the entire neuroaxis in a widespread fashion or it may predominantly affect the spinal cord and optic nerves.[7].
Spinal cord infarction Spinal cord infarction is a rare disease and often remains undiagnosed. Symptoms depend on the part of the cord involved.Around 70% of patients present with abrupt onset deficit with associated back pain.Anterior spinal artery infarcts often involve the central territory.Even though dissociative sensory loss is classically described, loss of proprioception found to be also occur. Areflexia, loss of vibration sense and weakness is found in PSA syndrome which is is relatively infrequent. Pure motor involvement is seen in some patients but it is consider as an atypical presentation. MRI is used for diagnosis and ASA infarcts will show “owl eye” appearance in MRI.[8].
References
  1. WANI BA, MISRA M, SHAH M, MUFTI S. Acute inflammatory demyelinating polyradiculoneuropathy presenting as complete heart block and Stoke-Adams attacks. Postgrad Med J [online] 1989 Feb, 65(760):103-104 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2429110
  2. KINSLEY L, SIDDIQUE T, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Amyotrophic Lateral Sclerosis Overview [online] 1993 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301623
  3. MCDERMAID C, MIOR S. Ankylosing spondylitis presenting to a chiropractic office: a report of two cases J Can Chiropr Assoc [online] 2000 Jun, 44(2):87-97 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2485496
  4. REYES-BOTERO G, MOKHTARI K, MARTIN-DUVERNEUIL N, DELATTRE JY, LAIGLE-DONADEY F. Adult Brainstem Gliomas Oncologist [online] 2012 Mar, 17(3):388-397 [viewed 20 September 2014] Available from: doi:10.1634/theoncologist.2011-0335
  5. KHOSYA S, MEENA H. Central pontine myelinolysis Indian J Med Res [online] 2013 May, 137(5):993-994 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3734697
  6. MARTIN R J. Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes. Journal of Neurology, Neurosurgery & Psychiatry [online] 2004 September, 75(suppl_3):iii22-iii28 [viewed 20 September 2014] Available from: doi:10.1136/jnnp.2004.045906
  7. STüVE O, OKSENBERG J, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Multiple Sclerosis Overview [online] 1993 [viewed 22 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301492
  8. SIVADASAN A, ALEXANDER M, PATIL AK, MANI S. Spectrum of clinicoradiological findings in spinal cord infarction: Report of three cases and review of the literature Ann Indian Acad Neurol [online] 2013, 16(2):190-193 [viewed 22 September 2014] Available from: doi:10.4103/0972-2327.112464

Investigations - for Diagnosis

Fact Explanation
MRI Diagnosis of chairi 1 malformation as well as syringomyelia with or without symptoms may carried out with the help of MRI.MRI can be used to study the volume of the posterior fossa as well as CSF flow dynamics. To establish the diagnosis of syringomyelia MRI of the complete spine have to be done including cervical, dorsal, and lumbar regions. [1].
Plain radiography Used to study bone anomalies.[1].
Computed Axial Tomography (CAT) Also used to study bone anomalies. Cranial CAT is found to be useful in monitoring hydrocephalus.[1].
EMG(electromyography) Performed to identify weaken muscle groups.[2].
Lumbar puncture Used to detect cerebrospinal fluid pressure changes as well as inflammatory conditions of the spinal cord.[3]. However CSF fluid analysis is usually not performed due to risk of herniation.[4].
Myelography It is carried out in special situations where MRI cannot be performed. It may show widening of cord and complete subarachnoid block.[4].
USS May useful in young children and thin patients. It can also be used intraoperatively after laminectomy to visualize syrinx cavities and septations.[4].
References
  1. AVELLANEDA FERNáNDEZ ALFREDO, ISLA GUERRERO ALBERTO, IZQUIERDO MARTíNEZ MARAVILLAS, AMADO VáZQUEZ MARíA EUGENIA, BARRóN FERNáNDEZ JAVIER, CHESA I OCTAVIO ESTER, DE LA CRUZ LABRADO JAVIER, ESCRIBANO SILVA MERCEDES, FERNáNDEZ DE ARAOZ MARTA FERNáNDEZ DE GAMBOA, GARCíA-RAMOS ROCíO, GARCíA RIBES MIGUEL, GóMEZ CARMEN, INSAUSTI VALDIVIA JOAQUíN, NAVARRO VALBUENA RAMóN, RAMóN JOSé R. Malformations of the craniocervical junction (chiari type I and syringomyelia: classification, diagnosis and treatment). Array [online] 2009 December [viewed 20 September 2014] Available from: doi:10.1186/1471-2474-10-S1-S1
  2. SCHWARTZ MS, STåLBERG E, SWASH M. Pattern of segmental motor involvement in syringomyelia: a single fibre EMG study. J Neurol Neurosurg Psychiatry [online] 1980 Feb, 43(2):150-155 [viewed 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC490490
  3. 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 20 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2565119
  4. SEN A. "Flow comp off": An easy technique to confirm CSF flow within syrinx and aqueduct Indian J Radiol Imaging [online] 2013, 23(1):97-100 [viewed 20 September 2014] Available from: doi:10.4103/0971-3026.113626

Investigations - Fitness for Management

Fact Explanation
Full blood count Performed as a basic investigation to rule out other infectious conditions like microbial infection.[1].
ESR(erythrocyte sedimentation rate) Carried out to rule out other inflammatory conditions specially causing neuropathic arthropathy.[1].
FBS(fasting blood sugar) Diabetes mellitus patients proven to develop charcot arthropathy specially in foot and ankle. FBS is performed to rule out associated DM.[1].
VDRL(venereal disease research laboratory test Syphilis patients proven to develop sensory disturbances and arthropathy around knee joint. VDRL is performed to rule out syphilis.[1].
Vitamin B12 level Performed to rule out associated B12 deficiency causing sensory disturbances.[1].
Crystal for gout and pseudogout Carried out to exclude gout and pseudogout.[1].
References
  1. PANAGARIYA A, SHARMA AK. Bilateral Charcot arthropathy of shoulder secondary to syringomyelia: An unusual case report Ann Indian Acad Neurol [online] 2012, 15(3):202-204 [viewed 20 September 2014] Available from: doi:10.4103/0972-2327.99718

Investigations - Followup

Fact Explanation
MRI MRI is a good tool to follow up patients following shunt surgery to assess the response.[1].
References
  1. SEN A. "Flow comp off": An easy technique to confirm CSF flow within syrinx and aqueduct Indian J Radiol Imaging [online] 2013, 23(1):97-100 [viewed 21 September 2014] Available from: doi:10.4103/0971-3026.113626

Management - General Measures

Fact Explanation
Analgesics and anti-inflammatory agents Useful in managing neuropathic pain. Those drugs include tramadol, buprenorphine, morphine dextropropoxyphene, oxycodonen, fentanyl and methadone. The analgesics act through specific receptors. Specially μ receptors. They distributed throughout the central and peripheral nervous system blocking them. They will give rise to various side effects such as Nausea, vomiting, sweating, dizzines, , sedation, vertigo and dry mouth. Anti-inflammatory agents and myorelaxants also useful in pain relief.[1].
Local anesthetics Also useful in relieving pain. It act mainly by inhibiting sodium influx through sodium specific ion channels in the neuronal cell membrane. Lidocaine and mexiletine belong to this category.Dizzine and arrhythmia are the commonly found side effects.[1].
Antidepressants Found to be useful in managing neuropathic pain. Examples include amitriptyline, venlafaxine and duloxetine. They act by inhibiting the re-uptake of the neurotransmitters such as norepinephrine and serotonin by neurons. They may give rise to variety of side effect profile including dry mouth, intense sedation, fatigue, diminished libido, nausea, insomnia, headache and weight loss.[1].
Anticonvulsants Anticonvulsants act by Inhibiting opening neuronal voltage dependent channels and GABA receptor. Usefull in managing pain. Such drugs include carbamazepine, pregabalin gabapentin and topiramate. Side effects include hepatotoxicity, drowsiness, fatigue, ataxia, vertigo, headache, blurred vision and gastrointestinal discomfort.[1].
Spinal or peripheral electrical stimulation They are non pharmacological managements used in pain management.Electrical stimulation is a potential option in case of symptoms of neuropathic pain are localized in a specific part of the body, such as one or both upper or lower limbs.And those options are free of major side effects.[1].
Speech therapy Paralysis, weakness, or incoordination of the speech musculature due to craniocervical malformations may leads to speech disorders. Speech therapist is need to improve Speech, swallowing, phonation, and also respiration. It can be carried out as a preventive measure or a palliative measure.[1].
Psychotherapy As this disease is a chronic condition, it can affect patient's psychological aspect. So that psychotherapy is a considered mandatory in the comprehensive management of patients with Chiari syndrome as well as syringomyelia. The main goal of this therapy is to reduce the psychosocial impact caused by the disease itself and there by improve the quality of life.[1]
Physical therapy Physical therapy is a mode of rehabilitation which is carried out in a multidisciplinary approach. It is carried out to relieve pain as well as to preserve the range of motion in the cervical spine and the shoulder joint. Postural treatment, specific kinesitherapy, general kinesitherapy, cognitive therapeutic exercise like variety of methods are used. The aim of physical therapy is reduce dependence,recover the patient's autonomy and improve quality of life.[1].
Occupational therapy Optimization of articular movements is the main goal of occupational therapy in order to prevent overloading of the upper limbs and neck.[ It is helpful in patient to improve quality of life.[1].
Reassurance, patient and family education with proper counselling Once diagnosis is made patient have to be reassured. Educate them well about the cause of disease, Its symptoms, how it progress and how if may affect day today life. Discuss them about the available treatment option and make a plan for follow up. Educate the patient and family members about the difficulties that may occur as the condition progresses such as swallowing, speech and respiratory difficulties. It is important tell them that patient may need helping hand from family members for day today works. As this is a chronic disease proper caring, maintaining good relationship as well as proper followup will need to improve patient's quality of life.[1].
References
  1. 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 21 September 2014] Available from: doi:10.1186/1471-2474-10-S1-S1

Management - Specific Treatments

Fact Explanation
Surgical decompression In symptomatic patients surgical decompression is performed.[1]. Most of the neurosurgeons like to follow up asymptomatic cases with clinical and imaging evaluations rather than going for a surgery. Majority of them favor posterior fossa decompression as the initial surgical approach. If there is associated case of chiari 1 malformation,suboccipital craniectomy, removal of the posterior arch of C1, and an augmentative duraplasty may performed as a baseline surgical approach. Where as the suboccipital posterior fossa decompression and atlas laminectomy are considered the standard surgical approach to a majority of patients who are symptomatic. However duraplasty is found to be associated with a lower risk of reoperation but has a greater risk for postoperative CSF leakage.[2]. And also these procedures carries a risk of scar tissue/adhesions around the cord and arachnoiditis.[3].
Syrinx shunting procedures Syringotomy or syringo peritoneal shunting is used for management of the CM-I-associated syringomyelia. It is indicated as a first-line surgical treatment in cases of extensive, large syringomyelic cavities. It has shown that syrinx shunting may results in faster decrease in the syrinx size when compared to posterior fossa decompression.[2]. Unfortunately, This proceedure has a trisk of shunt failure Where the reported incidence as high as 50% in some studies.[3].
Direct fluoroscopic drainage of a syrinx It is a minimally invasive procedure that only performed in selected group of patients. It is used in the workup and treatment of both tumor related spinal cord cysts and in case of syringomyelia. Clinicall improvement can be seen but most of the time transient.[3].
Terminal ventriculostomy, and neuroendoscopic surgery. Fourth/terminal ventriculostomy, and neuroendoscopic surgery are two other available surgical options to treat syringomyelia.[4].
References
  1. 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 21 September 2014] Available from: doi:10.1186/1471-2474-10-S1-S1
  2. SIASIOS JOHN, KAPSALAKI EFTYCHIA Z., FOUNTAS KOSTAS N.. Surgical Management of Patients with Chiari I Malformation. International Journal of Pediatrics [online] 2012 December, 2012:1-10 [viewed 21 September 2014] Available from: doi:10.1155/2012/640127
  3. SUDHEENDRA D, BARTINSKI WS. Direct Fluoroscopic Drainage of Symptomatic Post-Traumatic Syringomyelia: A Case Report and Review of the Literature Interv Neuroradiol [online] 2008 Dec 29, 14(4):461-464 [viewed 21 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3313816
  4. SEN A. "Flow comp off": An easy technique to confirm CSF flow within syrinx and aqueduct Indian J Radiol Imaging [online] 2013, 23(1):97-100 [viewed 21 September 2014] Available from: doi:10.4103/0971-3026.113626