scholarly journals Syringomyelia with Chiari I Malformation Presenting as Hip Charcot Arthropathy: A Case Report and Literature Review

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Roya Memarpour ◽  
Basheer Tashtoush ◽  
Lydia Issac ◽  
Fernando Gonzalez-Ibarra
Keyword(s):  
Articular Surface ◽  
Imaging Study ◽  
Chiari I Malformation ◽  
Type I ◽  
Charcot Arthropathy ◽  
Charcot Neuroarthropathy ◽  
Chiari Type I Malformation ◽  
Upper And Lower Extremities ◽  
Surface Destruction ◽  
The Right

Neuroarthropathy (neuropathic osteoarthropathy), also known as Charcot joint, is a condition characterized by a progressive articular surface destruction in the setting of impaired nociceptive and proprioceptive innervation of the involved joint. It is seen most commonly in the foot and ankle secondary to peripheral neuropathy associated with diabetes mellitus. Cases of hip (Charcot) neuroarthropathy are rare and almost exclusively reported in patients with neurosyphilis (tabes dorsalis). We report a case of a 36-year-old man who presented to the emergency department complaining of right hip pain. On physical examination, pain and thermal sensory deficits were noted in the upper torso with a cape-like distribution, as well as signs of an upper motor neuron lesion in the left upper and lower extremities. A magnetic resonance imaging study (MRI) of the right hip showed evidence of early articular surface destruction and periarticular edema consistent with hip Charcot arthropathy. An MRI of the spine revealed an Arnold-Chiari type I malformation with extensive syringohydromyelia of the cervical and thoracic spine.

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.

*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>

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.

scholarly journals Outcome of Posterior Fossa Decompression for Chiari Type I Malformation with Syringomyelia

2019 ◽  
Vol 16 (1) ◽  
pp. 35-38
Author(s):  
Sagar Koirala ◽  
Suresh Bishokarma ◽  
Dinesh Nath Gongal ◽  
Henry Thomas Marsh
Keyword(s):  
Posterior Fossa ◽  
Chiari I Malformation ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Tertiary Center ◽  
Clinical And Radiological Outcome ◽  
Chiari I ◽  
Chiari Type I

There are various treatment approaches for treating Chiari type I malformation with syringomyelia. Despite various choices, consensus for one particular approach is lacking. The objective of this study is to find out the clinical and radiological outcome of standard posterior fossa decompression incorporating removal of C1 arch with lax duroplasty in such cases. A retrospective study based on data acquired from a single tertiary center were analyzed. All cases who underwent posterior fossa decompression incorporating removal of C1 arch with lax duroplasty over a period of five years were included and their clinical and radiological progress were recorded during OPD follow up at 6 months. Out of 21 cases, occipital headache with nape of neck pain was the predominant complaint accounting to 71% followed by sensory symptoms and motor weakness, 61% and 33% respectively. Pain resolved in 93%, weakness in 71% and sensory symptoms in 69% of the cases. Only one patient developed hydrocephalus requiring shunting. Radiological improvement of syringomyelia were documented in 76.1% of the patients. There was no mortality. Posterior fossa decompression incorporating removal of C1 arch and lax duroplasty is a safe approach with good outcome in patients with Chiari I malformation with syringomyelia.

scholarly journals Chiari type I malformation with cervicothoracic syringomyelia subterfuge as flail arm syndrome

2017 ◽  
Vol 9 (3) ◽  
Author(s):  
Zhi Gang  Lan ◽  
Seidu A. Richard ◽  
Jiagang Liu ◽  
Chao You
Keyword(s):  
Neuromuscular Disorders ◽  
Neurological Complications ◽  
Type I ◽  
Chiari Type ◽  
Chiari Type I Malformation ◽  
Detection And Diagnosis ◽  
Magnetic Resonance Imaging Mri ◽  
The Right ◽  
Chiari Type I

Chiari type I malformation with cervicothoracic syringomyelia although very common in clinical practice usually in children can progress slowly and mimic muscular dystrophies in adulthood. We present a rare adult case of Chiari type I malformation with cervicothoracic syringomyelia subterfuge as Flail arm syndrome. A 44- year-old man was diagnosed with congenital type I Chiari malformation with cervicothoracic syringomyelia about 21 years ago without surgery. His health status deteriorated over the years until 21 days prior to presentation when he had severe pain in the right knee. In his upper limbs, he had bilateral corresponding severe weakness of 0/5 proximal strength and 0/5 strength in his distal muscles. Magnetic resonance imaging (MRI) revealed an enlargement of the spinal cord from C1-C4 level with a mass that appeared hypo-dense on T1 and hyperdense on T2. Syringomyelia is a potentially serious neurologic condition that can mimic other neuromuscular disorders. Early detection and diagnosis with MRI is crucial to avoid irreversible neurological complications. We suggest that whether asymptomatic or symptomatic, decompressive surgery should be carried out to allow for free flow of cerebrospinal fluid thereby improving the quality of life for the patient.

Chiari I malformation associated with premature unilateral closure of the posterior intraoccipital synchondrosis in a preterm infant

2013 ◽  
Vol 11 (6) ◽  
pp. 658-660 ◽  
Author(s):  
Aikaterini Kanavaki ◽  
Benoit Jenny ◽  
Sylviane Hanquinet
Keyword(s):  
Preterm Infant ◽  
Brain Mri ◽  
Chiari I Malformation ◽  
Occipital Bone ◽  
Secondary Development ◽  
Type I ◽  
Brain Malformation ◽  
Chiari Malformation Type I ◽  
Progressive Development ◽  
The Right

The authors report a case of a preterm infant at 29 weeks of gestation who gradually developed a Chiari malformation Type I (CM-I) with hydrocephalus due to a premature unilateral fusion of the posterior intraoccipital synchondrosis. Brain ultrasonography results in the 1st week of life were normal. Follow-up ultrasonography showed progressive development of triventricular hydrocephalus. Brain MRI demonstrated the presence of a CM-I and a deformation of the occipital bone. A complementary CT scan was obtained, showing a closure of the right posterior intraoccipital synchondrosis, resulting in a deformation of the posterior cranial fossa. This case shows the close relationship between a malformation of the skull base and the secondary development of a brain malformation. The authors discuss the anatomy of the occipital bone and suggest a probable theory for the premature closure of this synchondrosis and the consequent development of a CM-I. The originality of this case lies in the observation of the natural history of a brain malformation in a preterm infant.

Syringomyelia extending to the basal ganglia

1989 ◽  
Vol 71 (4) ◽  
pp. 616-617 ◽  
Author(s):  
Satoshi Okada ◽  
Yoshio Nakagawa ◽  
Kimiyoshi Hirakawa
Keyword(s):  
Magnetic Resonance Imaging ◽  
Pyramidal Tract ◽  
Type I ◽  
Sensory Disturbance ◽  
Resonance Imaging ◽  
Content Type ◽  
Chiari Type I Malformation ◽  
The Right ◽  
Chiari Type I ◽  
Fine Print

✓ A 10-year-old girl was admitted to the hospital with complaints of progressive right hemiparesis and sensory disturbance. Magnetic resonance imaging revealed a Chiari Type I malformation and syringomyelia from T-10 to C-1. The syrinx extended from the medulla to the right putamen along the pyramidal tract.

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.

scholarly journals Correlation of a new hydrodynamic index with other effective indexes in Chiari I malformation patients with different associations

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Seifollah Gholampour ◽  
Hanie Gholampour
Keyword(s):  
Cfd Simulation ◽  
Clinical Symptoms ◽  
Fluid Dynamic ◽  
Chiari I Malformation ◽  
The Other ◽  
Type I ◽  
Csf Pressure ◽  
Chiari Type I Malformation ◽  
Headache Symptom ◽  
Relationship Of

Abstract This study aimed to find a new CSF hydrodynamic index to assess Chiari type I malformation (CM-I) patients’ conditions and examine the relationship of this new index with morphometric and volumetric changes in these patients and their clinical symptoms. To this end, 58 CM-I patients in four groups and 20 healthy subjects underwent PC-MRI. Ten morphometric and three volumetric parameters were calculated. The CSF hydrodynamic parameters were also analyzed through computational fluid dynamic (CFD) simulation. The maximum CSF pressure was identified as a new hydrodynamic parameter to assess the CM-I patients’ conditions. This parameter was similar in patients with the same symptoms regardless of the group to which they belonged. The result showed a weak correlation between the maximum CSF pressure and the morphometric parameters in the patients. Among the volumetric parameters, PCF volume had the highest correlation with the maximum CSF pressure, which its value being higher in patients with CM-I/SM/scoliosis (R2 = 65.6%, P = 0.0022) than in the other patients. PCF volume was the more relevant volumetric parameter to assess the patients’ symptoms. The values of PCF volume were greater in patients that headache symptom was more obvious than other symptoms, as compared to the other patients.

scholarly journals Severe Cerebellar Degeneration and Chiari I Malformation - Speculative pathophysiology based on a systematic review

2020 ◽  
Vol 66 (3) ◽  
pp. 375-379
Author(s):  
Andrei Fernandes Joaquim
Keyword(s):  
Cerebellar Atrophy ◽  
Chiari I Malformation ◽  
Venous Hypertension ◽  
Cerebellar Degeneration ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Small Vessels ◽  
Chiari Type I Malformation ◽  
Pubmed Database ◽  
Key Terms

SUMMARY BACKGROUND Symptomatic Chiari Type I Malformation (CM) is treated with posterior fossa decompression with or without duroplasty. We have noticed some cases with concomitant severe cerebellar ataxia due to cerebellar atrophy. The aim of this study is to review the literature of CM associated with severe cerebellar atrophy and discuss its potential physiopathology. METHODS A systematic literature review in the Pubmed Database was performed using the following key-terms: “cerebellar atrophy Chiari”, and “cerebellar degeneration Chiari”. Articles reporting the presence of cerebellar degeneration/atrophy associated with CM were included. RESULTS We found only six studies directly discussing the association of cerebellar atrophy with CM, with a total of seven cases. We added one case of our own practice for additional discussion. Only speculative causes were described to justify cerebellar atrophy. The potential causes of cerebellar atrophy were diffuse cerebellar ischemia from chronic compression of small vessels (the most mentioned speculative cause), chronic raised intracranial pressure due to CSF block, chronic venous hypertension, and association with platybasia with ventral compression of the brainstem resulting in injury of the inferior olivary nuclei leading to mutual trophic effects in the cerebellum. Additionally, it is not impossible to rule out a degenerative cause for cerebellar atrophy without a causative reason. CONCLUSIONS Severe cerebellar atrophy is found in some patients with CM. Although chronic ischemia due to compression is the most presumed cause, other etiologies were proposed. The real reasons for cerebellar degeneration are not known. Further studies are necessary.

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