Chiari 1 malformation and holocord syringomyelia presenting as abrupt onset foot drop

McMillan, Hugh J.; Sell, Erick; Nzau, Munyao; Ventureyra, Enrique C. G.

doi:10.1007/s00381-010-1275-y

Cited by 17 publications

(8 citation statements)

References 25 publications

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“…In patients older than four years old, such presentations were rare with approximately nine reported cases globally. [5][6][7][8][9][10][11] Similar to the present patient, the reported cases were found in patients with normal motor development. Patients presented with rapidly progressive leg weakness and loss of reflexes and were found to have holocord syrinx on MRI.…”

Section: Discussionsupporting

confidence: 89%

Progressive Flaccid Paraplegia in a Toddler due to Chiari Type I Malformation Complicated with Hydrocephalus and Syringomyelia. A Case Report

LaRocca

Whalen

et al. 2023

Child Neurology Open

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Chiari malformation is a clinico-radiological entity defined by herniation of rhombencephalic structures through the foramen magnum. The most common type, Chiari I, involves herniation of the cerebellar tonsils specifically. We present the case of a 2-year-old with three weeks of progressive bilateral leg weakness, absent reflexes, and the inability to walk. The patient was found to have Chiari I with hydrocephalus and syringomyelia. This is the youngest patient reported in the literature presenting with a clinical picture of spinal shock. Early recognition of this entity allows for proper treatment and improved outcomes.

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Section: Discussionsupporting

confidence: 89%

Progressive Flaccid Paraplegia in a Toddler due to Chiari Type I Malformation Complicated with Hydrocephalus and Syringomyelia. A Case Report

LaRocca

Whalen

et al. 2023

Child Neurology Open

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“…Almost all of them actually showed a complete recovery from their initial condition and, in the remaining ones, a significant improvement of the clinical deficits was recorded (see Table 1). The postoperative normalization of the neuroradiological and/or neurophysiological findings was frequent as well, especially in children [29]. Such a discrepancy between onset and outcome may have two main explanations.…”

Section: Clinical Findings and Outcomementioning

confidence: 87%

Abrupt clinical onset of Chiari type I/syringomyelia complex: clinical and physiopathological implications

et al. 2012

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Chiari I malformation (CI) continues to raise great interest among physicians due to the larger and larger number of newly diagnosed cases. The clinical and radiological picture and the management options of such a chronic disease are well acknowledged as well as those of the associated syringomyelia. Little is known, on the other hand, about abrupt clinical onset following decompensation of CI/syringomyelia complex. This review on the sudden onset of these two conditions shows that this is a very rare phenomenon; only 41 cases are being reported in the last three decades. In all these cases, acute onset was referable to CI/syringomyelia and the clinical course quickly precipitated. Motor deficits (36.5 %), respiratory failure (29 %), cranial nerve palsy (17 %), and cardiac arrest (14.5 %) were the most common findings, thus confirming that abrupt onset may have severe and life-threatening consequences. Indeed, sudden or early mortality accounted for 19.5 % of cases. In spite of that, most of the surviving subjects had an excellent outcome following either surgical or medical/rehabilitation treatment. Physiopathology of abrupt onset is attributed to the acute compression of the brainstem/upper cervical spinal cord by ectopic tonsils and syringobulbia/syringomyelia, frequently precipitated by a minor injury, followed by impairment of medullary baroreceptors and midbrain reticular substance (cardiac arrest, syncope), medullary chemoreceptors and phrenic nerve nuclei (respiratory failure), lower cranial nerve nuclei (cardiac arrest, cranial nerve palsy), and pyramidal tracts (motor deficits). About 87 % of patients of this review were asymptomatic prior to their acute onset. The problem of the management of asymptomatic subjects is still open.

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“…Early diagnosis of CMI and holocord syrinx is pivotal and prompt surgical decompression may lead to an excellent prognosis. [ 8 , 12 , 17 ] In our case in adulthood, acquired CMI was misdiagnosed as congenial origin and subsequent surgical decompression for CMI was performed with resulting in rapidly progressive foot drop later. However, treating the primary cause, that is, brain AVM, of CMI associated holocord syrinx by endovascular treatment can facilitate motor function recovery.…”

Section: Discussionmentioning

confidence: 88%

Acquired Chiari malformation Type I and holocord syringomyelia associated with a high-flow supratentorial fistulous arteriovenous malformations: A case report and literature review

Iampreechakul

Wangtanaphat

Hangsapruek

et al. 2022

Surgical Neurology International

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Background: Chiari malformation Type I (CMI) is generally considered a congenital lesion and typically associated with syringomyelia. Acquired CMI or adult Chiari malformation caused by intracranial mass is extremely rare. Brain arteriovenous malformations (AVMs) are characteristically symptomatic due to seizure, intracranial hemorrhage, or neurological deficit. We report an extremely rare case of an acquired CMI and extensive syringomyelia associated with a large supratentorial AVM. Case Description: A 35-year-old woman was referred to our institute after a diagnosis of CMI and extensive syringomyelia from whole-spine magnetic resonance imaging (MRI) due to complaining of low back pain radiating to the right leg for the past 1 month. She had intermittent headache for 2 years. The patient underwent suboccipital decompression and C1 laminectomy followed by duraplasty. Two months later, she developed severe right-sided sciatic pain and complete right foot drop. Follow-up MRI revealed progressive enlargement of a syrinx cavity at the lower spinal cord and a large right parieto-occipital AVM with markedly dilated cortical draining veins and diffuse engorgement of dural venous sinuses was detected. This AVM supplied mainly by enlarged cortical branches of the right middle cerebral artery and posterior cerebral artery with multiple dural supplies. Endovascular treatment of a high-flow fistulous AVM was successfully performed with N-butyl cyanoacrylate (NBCA) through the hypertrophic branches of the right middle cerebral artery. Four months after embolization, the patient had recovered completely from the right foot drop. Further staged embolization was planned to reduce the size and flow of the AVM before stereotactic radiosurgery. However, the patient was lost to follow-up due to financial reason. One year later, she developed sudden severe headache followed by alteration of conscious due to intraventricular hemorrhage from the AVM, leading to obstructive hydrocephalus requiring cerebrospinal fluid diversion. During a period of 2 years, the patient underwent several staged embolization with NBCA and Onyx. Final cerebral angiography after embolization demonstrated a significant reduction in size and flow of the brain AVM. A control whole-spine MRI revealed a significant reduction in syrinx size. At the end of embolization, the patient had no neurological deficit. However, she had suffered from persistent central neuropathic pain at the right lower extremity. The AVM remnant was further treated by stereotactic radiosurgery. Conclusion: Increased cerebral venous hypertension secondary to a high-flow supratentorial AVM leading to posterior fossa venous hypertension may play a major role in the pathogenesis of CMI, induced the formation of syringomyelia. Endovascular treatment of brain AVM, the underlying cause of CMI, resulted in a significant reduction of the size of the syrinx. The need for cranial imaging in initial evaluation of cases with adult Chiari malformation is important.

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Chiari 1 malformation and holocord syringomyelia presenting as abrupt onset foot drop

Cited by 17 publications

References 25 publications

Progressive Flaccid Paraplegia in a Toddler due to Chiari Type I Malformation Complicated with Hydrocephalus and Syringomyelia. A Case Report

Progressive Flaccid Paraplegia in a Toddler due to Chiari Type I Malformation Complicated with Hydrocephalus and Syringomyelia. A Case Report

Abrupt clinical onset of Chiari type I/syringomyelia complex: clinical and physiopathological implications

Acquired Chiari malformation Type I and holocord syringomyelia associated with a high-flow supratentorial fistulous arteriovenous malformations: A case report and literature review

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