*Siringomielia asociada a Malformación de Chiari I. Reporte de caso. [Syringomyelia associated with Chiari I Malformation. Case report.]

2021 ◽  
Vol 40 (03) ◽  
Author(s):  
Flor Wilson Giraldo ◽  
Hector Lezcano ◽  
Leonardo Barrios
Keyword(s):  
Cranial Nerves ◽  
Chiari I Malformation ◽  
Clinical Suspicion ◽  
Unknown Etiology ◽  
Type I ◽  
Imaging Studies ◽  
Structural Alterations ◽  
Truncal Ataxia ◽  
Chiari I ◽  
The Right

<p>Introducción: Las Malformaciones de Chiari (M.C.) son alteraciones estructurales a nivel del cerebelo de etiología en estudio. Muy raras, afectan al 0,5% de la población. Más de la mitad de los pacientes cursan con siringomielia. Caso clínico: Femenina de 47 años con antecedente de M.C. Tipo I, acude con cuadro de dos semanas de evolución de cefalea holocraneana de intensidad 8/10, asociado a mareo e inestabilidad a la marcha. Hallazgos positivos de alteración en los pares craneales III, VIII, IX; hipertonía, signo de Babinski y Hoffman positivos bilaterales; ataxia truncal; nistagmus a la derecha; fuerza muscular disminuida en miembros superiores e hiperreflexia. Discusión: Ante la sospecha clínica, y las múltiples alteraciones a nivel del cerebelo y bulbo, se procede a realizar estudios de imagen y se confirma el diagnóstico de siringomielia.</p><p><strong>Abstract: </strong></p><p>Introduction: Chiari malformations (C.M.) are structural alterations in cerebellum. They are of unknown etiology, at present in study. They are very rare and affect 0,5 % of the population. More than half of the patients have syringomyelia. Clinical case: A 47-year-old female with medical record of C.M. Type I, presents with a two-week evolution holocranial headache, 8/10 in intensity, associated with dizziness and gait instability. Positive findings of alteration in cranial nerves III, VIII, IX; hypertonia, Babinski and Hoffman bilateral sign; truncal ataxia; nystagmus on the right; decreased muscle strength in the upper limbs and hyperreflexia. Discussion: Due to clinical suspicion and multiple alterations in cerebellum and bulb, imaging studies are performed and the diagnosis of syringomyelia is confirmed.</p>

scholarly journals Imaging Findings in Chiari I Malformation with Syringomyelia in a Case of Charcot Shoulder

2011 ◽  
Vol 1 ◽  
pp. 46 ◽  
Author(s):  
Shantanu Kumar ◽  
Vineet Sharma ◽  
Santosh Kumar ◽  
Sonal Jain
Keyword(s):  
Shoulder Joint ◽  
Fluid Collection ◽  
Joint Space ◽  
Chiari I Malformation ◽  
Type I ◽  
Neuropathic Arthropathy ◽  
Chiari Malformation Type I ◽  
Lateral Dislocation ◽  
Chiari I ◽  
The Right

Neuropathic arthropathy of the shoulder is reported in only 5% of cases. Here, we report a rare case of neuropathic arthropathy of the shoulder, secondary to Chiari malformation Type I with associated syringomyelia, that remained undetected for four years. A 38-year-old female presented to our Department with a swelling over the right shoulder that had persisted for four years. X-ray of the joint showed destruction of the head of the right humerus, with typical blunt amputated appearance of the bone and increased joint space. Magnetic resonance imaging showed destruction and lateral dislocation of the head of the humerus. Large amount of fluid collection was seen in and around the right shoulder joint. Neuropathic osteoarthropathy can be defined as bone and joint changes that occurs secondary to loss of sensation. In our case, neuropathic shoulder joint was secondary to syringomyelia associated with Chiari I malformation.

Chiari Type I Malformation Presenting as Hemifacial Spasm: Case Report

Neurosurgery ◽  
2005 ◽  
Vol 57 (2) ◽  
pp. E371-E371 ◽  
Author(s):  
Mustafa Efkan Colpan ◽  
Zeki Sekerci
Keyword(s):  
Facial Nerve ◽  
Posterior Fossa ◽  
Hemifacial Spasm ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Type I Malformation ◽  
Downward Displacement ◽  
Facial Spasm ◽  
Chiari I

ABSTRACT OBJECTIVE AND IMPORTANCE: We report on a patient with a Chiari I malformation presenting with right hemifacial spasm. Clinicians should consider the downward displacement of the hindbrain as a rare cause of hemifacial spasm in Chiari I malformation. CLINICAL PRESENTATION: An 18-year-old man was admitted with right hemifacial spasm. The results of the neurological examination were normal except for the facial spasm. Magnetic resonance imaging demonstrated a Chiari I malformation without syringomyelia. After surgery, the hemifacial spasm completely resolved. INTERVENTION: Posterior fossa decompression, C1 laminectomy, and duraplasty were performed. CONCLUSION: The hemifacial spasm could be attributed to compression and/or traction of the facial nerve because of downward displacement of the hindbrain in Chiari I malformation. Compression and/or traction might create irritation of the facial nerve that causes hemifacial spasm. Resolution of the hemifacial spasm after posterior fossa decompression could explain the facial nerve irritation in Chiari I malformation. Clinicians should consider Chiari malformation as a cause of hemifacial spasm and posterior fossa decompression as a potential treatment.

scholarly journals Chiari I malformation and Noonan’s syndrome: Shared manifestations of RASopathy

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Megan Still ◽  
◽  
Patricia Miller ◽  
William Dodd ◽  
Rachel Moor ◽  
...  
Keyword(s):  
Case Report ◽  
Noonan Syndrome ◽  
Patient Population ◽  
Chiari I Malformation ◽  
Clinical Suspicion ◽  
Unique Case ◽  
Outflow Obstruction ◽  
Ventricular Outflow Obstruction ◽  
Chiari I

Neurologic sequalae of Noonan syndrome have been postulated in the literature. A topic of significance is the role of RASopathy in the shared pathophysiology of Noonan Syndrome and Chiari I malformation. In this unique case report, we present a patient with concomitant Noonan Syndrome and Chiari I with 4th ventricular outflow obstruction. The case highlights the importance of close clinical suspicion in this patient population. We utilize the case to delve into intricacies of the known pathophysiology and encourage ongoing investigation. Keywords: Noonan syndrome; Chiari I malformation; RASopathy.

scholarly journals Acquired Chiari I malformation due to lumboperitoneal shunt: A case report and review of literature

2019 ◽  
Vol 10 ◽  
pp. 78
Author(s):  
Aslam Hentati ◽  
Mohamed Badri ◽  
Kamel Bahri ◽  
Ihsen Zammel
Keyword(s):  
Chiari Malformation ◽  
Rare Complication ◽  
Chiari I Malformation ◽  
Clinical Feature ◽  
Type I ◽  
Cerebral Magnetic Resonance Imaging ◽  
Surgical Closure ◽  
Lumboperitoneal Shunt ◽  
Cerebral Mri ◽  
Chiari I

Background: The Type I malformations are supposed to be the result of mesodermal defects that create a congenitally small posterior fossa. However, Chiari malformation could be also “iatrogenic” and then called “acquired” Chiari I malformation. In this study, the authors report the clinical feature of a patient who developed a Chiari I malformation after lumboperitoneal shunt. Case Description: A 35-year-old woman has been suffering from idiopathic intracranial hypertension and rhinorrhea due to an anterior skull base defect. A valveless lumboperitoneal shunt followed by surgical closure of the defect was performed. Six months later, she suffered from major continuous occipital headaches. The neurological examination found a mild cerebellar gait ataxia and cerebellar dysarthria. The cerebral magnetic resonance imaging (MRI) showed a ptosis of the cerebellar tonsils and a disappearance of the cisterna magna; there was no syringomyelia. This herniation was not present before shunt was performed. A replacement of the lumboperitoneal shunt with a pressure-regulated valve chamber was performed. After a 1-year follow-up, the patient reports a marked decrease of the headache as well as the ataxia, and the last cerebral MRI showed resolution of the Chiari I malformation. Conclusions: Symptomatic acquired Chiari malformation with or without syringomyelia as a delayed complication after lumbar shunting is a rare complication, particularly reported in the pediatric population, but could also occur to adult patients. Treating these patients by correcting the shunt’s valve could be enough, but should be monitored, as it may fail to resolve the Chiari malformation even years after treatment.

Cystic Fibrosis and Chiari Type I Malformation: Autopsy Study of Two Infants with a Rare Association

2003 ◽  
Vol 6 (1) ◽  
pp. 88-93 ◽  
Author(s):  
Dinesh Rakheja ◽  
Yin Xu ◽  
Dennis K. Burns ◽  
Daniel L. Veltkamp ◽  
Linda R. Margraf
Keyword(s):  
Cystic Fibrosis ◽  
Mutational Analysis ◽  
Chiari I Malformation ◽  
Cftr Gene ◽  
Type I ◽  
Autopsy Study ◽  
Recurrent Pneumonia ◽  
Cftr Mutations ◽  
Chiari I ◽  
American Children

Cystic fibrosis (CF), an epithelial cell transport disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, is not generally associated with malformations of the central nervous system (CNS). This report describes two African-American children who died at less than 2 years of age with known Chiari I malformations and were found, unexpectedly at autopsy, to have the classic pancreatic and respiratory changes of CF. Both patients had suffered from failure to thrive that had been attributed to their CNS malformations. One child also had recurrent pneumonia and died with Pseudomonas sepsis. Mutational analysis for > 70 common CFTR mutations identified a single delta F508 mutation in one patient and a single 3120 + 1G to A mutation in the other. Their second CFTR mutations were not identified. The association of CF with Chiari I malformation is not likely to be purely coincidental, as the probability of such an occurrence in African-Americans is greater than one in 7,500,000 patients. It is possible that the CFTR gene may play a previously unrecognized role in CNS development. Alternatively, this CNS abnormality may be acquired due to the metabolic and electrolyte imbalances that characteristically occur in CF.

Advanced Chiari malformation Type I with marked spinal cord atrophy

2005 ◽  
Vol 3 (4) ◽  
pp. 324-327 ◽  
Author(s):  
Yoshiro Ito ◽  
Koji Tsuboi ◽  
Hiroyoshi Akutsu ◽  
Satoshi Ihara ◽  
Akira Matsumura
Keyword(s):  
Spinal Cord ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Type I ◽  
Foramen Magnum Decompression ◽  
Spinal Cord Atrophy ◽  
Chiari Malformation Type I ◽  
Content Type ◽  
Neurological Improvement ◽  
Chiari I

✓ The authors discuss the results obtained in patients who underwent foramen magnum decompression for longstanding advanced Chiari I malformation in which marked spinal cord atrophy was present. This 50-year-old woman presented with progressive quadriparesis and sensory disorders. Magnetic resonance imaging revealed the descent of cerebellar tonsils and medulla associated with remarkable C1—L2 spinal cord atrophy. After a C-1 laminectomy—based foramen magnum decompression, arachnoid dissection and duraplasty were undertaken. These procedures resulted in remarkable neurological improvement, even after 40 years of clinical progression. Spinal cord atrophy may be caused by chronic pressure of entrapped cerebrospinal fluid in the spinal canal.

scholarly journals Surgical treatment of Arnold-Chiari malformation type I in an adult patient

2008 ◽  
Vol 65 (8) ◽  
pp. 648-652
Author(s):  
Marko Markovic ◽  
Iva Berisavac ◽  
Vladimir Bojovic ◽  
Bojan Kostic ◽  
Vuk Djulejic
Keyword(s):  
Cranial Nerves ◽  
Clinical Signs ◽  
Foramen Magnum ◽  
Clinical Image ◽  
Posterior Arch ◽  
Type I ◽  
Chiari Malformation Type I ◽  
Truncal Ataxia ◽  
Dural Graft ◽  
Cerebellar Tonsils

Background. Herniation of the cerebellar tonsils through the foramen magnum into the cervical spinal canal with obliteration of the cerebellomedullary cistern is the primary feature of Arnold-Chiari type I malformation (ACM I). It is considered to be congenital malformation, although there have been reported cases of an acquired form. Case report. We presented a female patient, 45-year old, with ACM I without syringomyelia as a rare and unusual clinical image, as well as the effect of decompressive surgery in the treatment of this malformation. The patient was admitted to the Department of Neurosurgery with clinical signs of truncal ataxia worsening during the last six years. Moderate quadriparesis with predominant lower extremity involvement and the signs of the cranial nerves damages occured during the last seven months before admission, with progressive clinical course up to the date of admission. Neurosurgical treatment that included suboccipital medial craniectomy with resection of posterior arch C1 vertebrae and C2 laminectomy resulted in a significant clinical improvement and a much better quality of life. Conclusion. Posterior craniovertebral decompression with microsurgical reduction of the cerebellar tonsils and placement of an artificial dural graft is a treatment of choice in severe forms of ACM I without syringomyelia. .

scholarly journals Elucidating the pathophysiology of syringomyelia

1999 ◽  
Vol 7 (2) ◽  
pp. E1 ◽  
Author(s):  
John D. Heiss ◽  
Nicholas Patronas ◽  
Hetty L. DeVroom ◽  
Thomas Shawker ◽  
Robert Ennis ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Pulse Pressure ◽  
Subarachnoid Space ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Imaging Studies ◽  
Cine Mr ◽  
Spinal Subarachnoid Space ◽  
Chiari I ◽  
Cerebellar Tonsils

Object Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Chiari I malformation of the cerebellar tonsils. Determination of the pathophysiological mechanisms underlying the progression of syringomyelia associated with the Chiari I malformation should improve strategies to halt progression of myelopathy. Methods The authors prospectively studied 20 adult patients with both Chiari I malformation and symptomatic syringomyelia. Testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging; evaluation of the syrinx and cerebrospinal fluid (CSF) velocity and flow by using phase-contrast cine MR imaging; and evaluation of lumbar and cervical subarachnoid pressure at rest, during the Valsalva maneuver, during jugular compression, and following removal of CSF (CSF compliance measurement). During surgery, cardiac-gated ultrasonography and pressure measurements were obtained from the intracranial, cervical subarachnoid, and lumbar intrathecal spaces and syrinx. Six months after surgery, clinical examinations, MR imaging studies, and CSF pressure recordings were repeated. Clinical examinations and MR imaging studies were repeated annually. For comparison, 18 healthy volunteers underwent T1-weighted MR imaging, cine MR imaging, and cervical and lumbar subarachnoid pressure testing. Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. In patients, CSF velocity at the foramen magnum was increased, but CSF flow was reduced. Transmission of intracranial pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was partially obstructed. Spinal CSF compliance was reduced, whereas cervical subarachnoid pressure and pulse pressure were increased. Syrinx fluid flowed inferiorly during systole and superiorly during diastole on cine MR imaging. At surgery, the cerebellar tonsils abruptly descended during systole and ascended during diastole, and the upper pole of the syrinx contracted in a manner synchronous with tonsillar descent and with the peak systolic cervical subarachnoid pressure wave. Following surgery, the diameter of the CSF passages at the foramen magnum increased compared with preoperative values, and the maximum flow rate of CSF across the foramen magnum during systole increased. Transmission of pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was normal and cervical subarachnoid mean pressuree and pulse pressure decreased to normal. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape. Conclusions The progression of syringomyelia associated with Chiari I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Chiari I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-1 laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures.

Sleep Apnea and the Chiari I Malformation: Case Report

Neurosurgery ◽  
1988 ◽  
Vol 23 (4) ◽  
pp. 508-510 ◽  
Author(s):  
Philip Levitt ◽  
Martin A. Cohn
Keyword(s):  
Case Report ◽  
Sleep Apnea ◽  
Chiari I Malformation ◽  
Type I ◽  
Ambulatory Patient ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Chiari I ◽  
Chiari Type I

Abstract The authors report the case of an ambulatory patient who presented with sleep apnea episodes of the central and obstructive types and an associated Chiari Type I malformation. The central episodes stopped and the obstructive episodes decreased markedly after decompression. The improvement in the so-called obstructive episodes was unexpected and indicates that they may have been on a central basis.

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