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Journal articles on the topic 'Arnold chiari syndrome'

Author: Grafiati

Published: 4 June 2025

Last updated: 23 June 2025

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1

Nowaczyk, Natalia, and Jolanta Góral-Półrola. "Neuropsychological diagnosis of a female patient with Arnold-Chiari malformation type I." Acta Neuropsychologica 21, no. 4 (2023): 457–78. http://dx.doi.org/10.5604/01.3001.0054.1734.

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Arnold–Chiari I malformation is a congenital malformation of the hindbrain characterized by displacement of the cerebellar tonsils into the foramen magnum, pressure on the fourth ventricle, and decreased fluid flow to the basal cisterns. It is a mild form of the neuro-cranio-vertebral syndrome (Arnold–Chiari syndrome) because in many cases it is asymptomatic or has few clinical signs and symptoms of mild severity.The case study presented in the article concerns a 16.5-year-old patient with Arnold–Chiari I malformation following surgery. Based on the neuropsychological diagnosis, clinical symptoms that might result from the course of structural and functional changes in the hindbrain in Arnold–Chiari I syndrome were described and explained. The following were observed: selective difficulties in planning and concentration (maintaining the direction of action) and a weakening of visual perception (described by the patient as a subjective state of derealization).The results of the neuropsychological diagnosis point to certain practical implications for the further therapeutic treatment of patients with a mild form of Arnold–Chiari I malformation, especially as there is a risk of clinical symptoms changing over time due to the posibility of progressive neurodegenerative changes in the hindbrain.

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2

Pociecha, Agnieszka, Marta Kozikowska, Bożena Kmak, et al. "Arnold-Chiari Syndrome – a review." Journal of Education, Health and Sport 68 (May 15, 2024): 49398. http://dx.doi.org/10.12775/jehs.2024.68.49398.

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Arnold Chiari syndrome, also known as Chiari malformation, is a group of deformations of the posterior cranial fossa and hindbrain, consisting in the lowering of the cerebellum or its tonsils towards the occipital foramen. This leads to a reduction in the volume reserve within the foramen magnum, pressure on surrounding structures and obstruction of the flow of cerebrospinal fluid. The process is most often asymptomatic. Symptoms include headaches, neck pain, but also fainting, sinus bradycardia, coordination disorders and many others. Chiari malformation is often diagnosed incidentally. The basis of diagnosis is the detection of characteristic morphological features in magnetic resonance imaging. Surgical treatment is successfully used among patients with severe Arnold-Chiari syndrome and progression of clinical symptoms. Materials and methods Literature included in the PubMed, BioMed Central and Polish Medical Platform databases searched by means of the words such as Gamma Knife, CyberKnife, radiosurgery, stereotactic radiotherapy. Sources quoted in selected works were also used. Summary Arnold-Chiari malformation is often discovered incidentally. If the features observed on MRI suggest the above diagnosis, the patient should be referred to a neurologist. After excluding other possible causes of the imaged morphological features and performing a dedicated magnetic resonance imaging examination, the neurosurgeon decides whether to qualify for the procedure. In a selected group of patients, significant improvement can be expected after surgical treatment.

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3

Tasdemir, Nebahat, Orhan Tacar, Ayda Demirant, and Serhan Tasdemir. "Arnold-Chiari Syndrome (Type I)." Neuroembryology and Aging 3, no. 3 (2004): 149–51. http://dx.doi.org/10.1159/000094671.

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4

S.Aithmadouch, K.larbiouassou, A.laaraj, and R.abikassem. "A Case Report on Arnold Chiari Type III: Constellation of Disorders, from Diagnosis to Treatment." Asian Journal of Advanced Research and Reports 18, no. 12 (2024): 297–301. https://doi.org/10.9734/ajarr/2024/v18i12826.

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Arnold-Chiari type III is a malformation characterised by protrusion of the brainstem, cerebellum and lower part of the brain due to a cranial malformation. It is a rare congenital malformation in which the brain protrudes through a fissure in the skull. It occurs during pregnancy when the neural tube of the foetus does not close completely. The Chiari malformation type III, although described in the literature, remains rare, complicating our understanding of their common pathophysiological mechanisms and their management. We report the case of a female infant, admitted for seizure with hypotonia and dysmophic syndrome. An MRI was performed showing encephalomeningocele leading for Arnold Chiari type III diagnosis. The aim of this article is to understand and investigate the association between Arnold-Chiari type III malformation and encephalocele in order to develop more effective therapeutic strategies.

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5

Young, Stephanie, Marc G. Reyes, and I. Livingstone Uzoaru. "Arnold-Chiari Malformation in Jarcho-Levin Syndrome." Journal of Child Neurology 4, no. 3 (1989): 229–30. http://dx.doi.org/10.1177/088307388900400318.

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6

Voltisa, Lama. "Coexistence of Psychiatric Symptoms and Chiari Type I Malformation - A Case Report." Medicus 1 (October 16, 2018): 35–43. https://doi.org/10.5281/zenodo.7611606.

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Chiari type I malformation has been described infrequently in association with defined psychiatric syndromes. Method: There is a limited literature about obsessions in comorbidity with Chiari malformation. It is described a case of an adolescent with obsessive compulsive disorder and Chiari I malformation and it is reviewed the literature regarding Chiari I malformation and psychiatric disorders. The child came to the attention of child psychiatrist at the age of 7 years old when he manifested developmental delay and various kinds of behavioral symptoms. He was followed up at the age of fifteen when he developed obsessions and upon MRI was identified a Chiari malformation type I. Discussion: This paper discusses the likely under recognized co-occurrence of Chiari malformation and psychiatric symptoms. Currently available data from case reports associate Chiari I malformation with a variability of psychiatric symptoms: autism spectrum disorder, bipolar disorder, seizures, developmental delay, generalized anxiety disorder, panic attacks, Tourette’s syndrome, OCD, ADHD, cognitive disorder NOS and psychosis. It is concluded that mixed psychiatric symptoms and developmental delay might be a more common finding in comorbidity with Arnold Chiari type I malformation.

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7

Belykh, N. A., A. M. Khrulyova, I. V. Piznyur, and G. F. Raeva. "CLINICAL CASE OF ARNOLD-CHIARI SYNDROME IN COMBINATION WITH DEXTROCARDIA IN A CHILD." Euroasian Health Journal, no. 5 (December 17, 2024): 115–21. https://doi.org/10.54890/1694-8882-2024-5-115.

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Arnold-Chiari syndrome is a rare congenital disease characterized by a decrease in the volume of the cranial cavity and a change in the anatomy of the posterior cranial fossa. Arnold-Chiari syndrome has several types (from 1 to 4), each of which is characterized by a different degree of compression of the brain and hydrocephalus, and its new forms have recently been added (type 0 and 1.5). the quality of life of patients. The disease is most common in children and young people. According to experts, its prevalence ranges from 3.2 to 8.4 cases per 100,000 people worldwide. The incidence and severity of Arnold-Chiari syndrome have a statistically significant tendency to increase in the female sex. The etiology of Arnold-Chiari syndrome has not yet been fully studied, but it is assumed that genetic factors and effects on the fetus during pregnancy may play a dominant role in its development. Clinically, the syndrome is manifested by headache, vomiting, swallowing disorders, various motor disorders, impaired coordination and long-lasting Babinsky reflex, weakness and numbness in the extremities, sleep problems and other symptoms. Patients may also develop hydrocephalus, which increases the risk of serious complications and requires surgical interventions. The article presents data on the prevalence, clinical manifestations and treatment of Arnold-Chiari syndrome, as well as our own clinical observation of the course of this disease, which is combined with dextrocardia and heterotaxia – the mirror position of internal organs in a 10-year-old patient. Arnold-Chiari syndrome today refers to severe congenital diseases of the nervous system that are difficult to diagnose at an early age, therefore it is important for a pediatrician to understand the mechanism of occurrence of this disease in order to be able not only to eliminate, but also to prevent the undesirable consequences of this disease. Синдром Арнольда-Киари - представляет собой редкое врожденное заболевание, характеризующееся снижением объема черепной коробке и изменением анатомии задней черепной ямки. Синдром Арнольда-Киари имеет несколько типов (от 1 до 4), каждый из которых характеризуется различной степенью сдавливания мозга и гидроцефалией, также недавно были добавлены его новые формы (0 и 1,5 тип). на качество жизни пациентов. Заболевание наиболее часто встречается у детей и молодых людей. По оценкам экспертов, его распространенность составляет от 3,2 до 8,4 случаев на 100 000 населения по всему миру. Встречаемость и тяжесть синдром Арнольда-Киари имеют статистически значимую тенденцию к увеличению у женского пола. Этиология синдрома Арнольда-Киари до сих пор не полностью изучена, но предполагается, что генетические факторы и воздействие на плод во время беременности могут играть главенствующую роль в его развитии. Клинически синдром проявляется головной болью, рвотой, нарушением глотания, различными двигательными нарушениями, нарушением координации и длительно сохраняющимся рефлексом Бабинского, слабостью и онемением в конечностях, проблемы со сном и другими симптомами. У пациентов также может развиться гидроцефалия, что увеличивает риск возникновения серьезных осложнений и требует оперативных вмешательств. В статье приведены данные в отношении распространенности, клинических проявлений и лечения синдром Арнольда-Киари, а также собственное клиническое наблюдение течения данного заболевания который сочетается с декстрокардией и гетеротаксией – зеркальным положением внутренних органов у пациента 10 лет. Синдром Арнольда-Киари на сегодняшний день относится к тяжелым врожденным заболеваниям нервной системы, трудно диагностируемом в раннем возрасте, поэтому педиатру важно понимать механизм возникновения данного заболевания, чтобы уметь не только устранить, но и предотвратить нежелательные последствия данного заболевания.

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8

Chang, Y.-T., F.-J. Tsai, W.-C. Shen, H.-C. Lin, C.-T. Peng, and C.-H. Tsai. "Antley-Bixler syndrome associated with Arnold-Chiari malformation." Acta Paediatrica 89, no. 6 (2000): 737. http://dx.doi.org/10.1111/j.1651-2227.2000.tb00376.x.

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9

Lam, B. "Arnold-Chiari malformation presenting as sleep apnea syndrome." Sleep Medicine 1, no. 2 (2000): 139–44. http://dx.doi.org/10.1016/s1389-9457(99)00004-0.

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10

Hrishi, AjayP, ShilpaV Nagmoti, and Manikandan Sethuraman. "Gitelman syndrome with Arnold–Chiari malformation for neurosurgery." Neurology India 67, no. 3 (2019): 898. http://dx.doi.org/10.4103/0028-3886.263189.

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11

Jalal, Malik Liaqat Ali, Muhammad Shaukat Farooq, Atta-ur-Rehman Khan, Muhammad Yousaf, Muhammad Rizwan, and Saima Faiz. "Management and Complications of Arnold Chiari Hydrocephalus at Tertiary Health Care Center." International Journal of Frontier Sciences 4, no. 1 (2020): 44–46. https://doi.org/10.5281/zenodo.3613504.

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<strong>ABSTRACT</strong> <strong>Objective: </strong>to investigate the role of CT and MRI in diagnosis of Arnold chiari malformation of hydrocephalus and its treatment with VP shunt along with complications. <strong>Study Design:</strong> Prospective study <strong>Place and duration:</strong> Department of Neurosurgery, DG Khan Medical College from May 4, 2018 to May 4, 2019. <strong>Methodology:</strong> Fifty patients of congenital hydrocephalus who were not treated previously were selected. Detailed history about disease and clinical examination of patients was performed. Follow ups were done at neurosurgery OPD. SPSS software for data analysis was used and mean ± SD, frequency and percentages were calculated for variables. P value ≤0.05 was considered as significant. <strong>Results:</strong> Treatment in case of congenital hydrocephalus as VP shunt, intra-aneurysmal coiling, excision of meningomyelocele with VP shunt and suboccipital craniectomy + upper cervical laminectomy (scucl) were observed as 10%, 6%, 8% and 8% respectively.  Twelve percent of patients were not treated. <strong>Conclusion: </strong>CT and MRI are the main diagnostic tools for diagnosis of Arnold chiari malformation and VP shunt is the treatment of choice. Among complications of VP shunt infection of shunt and shunt block are the main complications.

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12

Huber, Aric, Matthew Driben, and Eduardo Espiridion. "Arnold-Chiari Malformation-I Borderline Personality Disorder." Transformative Medicine 2, no. 2 (2023): 25–28. http://dx.doi.org/10.54299/tmed/wgdh6653.

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Introduction: Arnold-Chiari Malformation I (AM-I) is a congenital anomaly that manifests with cerebellar dysfunction. There is a displacement of cerebellar tonsils into the foramen magnum. Several mood disorders, personality disorders, and intellectual disabilities are associated with AM-I. Borderline personality disorder (BPD) is characterized by symptoms of mood lability, impulsivity, extreme efforts of abandonment, splitting and dysfunctional relationships. Case Description: The patient is an early aged adult with a past medical history of AM-I, hypothyroidism, Wolff-Parkinson-White syndrome, and diabetes mellitus type II. The patient was admitted to the hospital after ingesting foreign bodies. He/she presented with mood lability, sad mood, anhedonia, insomnia, panic attacks, ruminative worries, feelings of emptiness, and recurrent suicidal gestures and threats. The patient was eventually diagnosed with borderline personality disorder. This case suggests a possible connection between AM-I and BPD. Discussion: Emerging from contemporary research involving the cerebellum, it is important to acknowledge that the current definition of control of fine motor and balance is inadequate. Symptoms associated with AM-I and BPD may be better explained by Cerebellar Cognitive Affective Syndrome (CCAS), a condition that ties the cerebellum with the higher cognitive functioning in the brain.

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13

Lama, Voltisa. "Coexistence of Psychiatric Symptoms and Chiari Type I." MEDICUS 1, no. 1 (2022): 35–43. https://doi.org/10.58944/mdbt7215.

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Introduction: Chiari type I malformation has been described infrequently in association with defined psychiatric syndromes. Method: There is a limited literature about obsessions in comorbidity with Chiari malformation. It is described a case of an adolescent with obsessive compulsive disorder and Chiari I malformation and it is reviewed the literature regarding Chiari I malformation and psychiatric disorders. The child came to the attention of child psychiatrist at the age of 7 years old when he manifested developmental delay and various kinds of behavioral symptoms. He was followed up at the age of fifteen when he developed obsessions and upon MRI was identified a Chiari malformation type I. Discussion: This paper discusses the likely under recognized co-occurrence of Chiari malformation and psychiatric symptoms. Currently available data from case reports associate Chiari I malformation with a variability of psychiatric symptoms: autism spectrum disorder, bipolar disorder, seizures, developmental delay, generalized anxiety disorder, panic attacks, Tourette’s syndrome, OCD, ADHD, cognitive disorder NOS and psychosis. It is concluded that mixed psychiatric symptoms and developmental delay might be a more common finding in comorbidity with Arnold Chiari type I malformation.

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14

Jacome, D. "Blepharoclonus and Arnold—Chiari malformation (Acta Neurol. Scand. — 2001. — Aug. —104(2).— P. 113—117: англ.)". Neurology Bulletin XXXIII, № 3-4 (2001): 101. http://dx.doi.org/10.17816/nb80918.

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Clonic blepharospasm - high-amplitude contractions of the circular muscle of the eye. This syndrome occurs in some diseases, such as traumatic brain injury. In this work, clinical observations with Arnold-Chiari malformation, in which clonic blepharospasm is shown for the first time.

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do Amaral, Maria Stella Arantes, Daniela Soares Fonseca, Antonio Carlos dos Santos, et al. "Cochlear Implant in a Patient with Arnold-Chiari Syndrome." Case Reports in Clinical Medicine 07, no. 02 (2018): 162–67. http://dx.doi.org/10.4236/crcm.2018.72014.

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Valdés, F., F. J. Vadillo, and A. Martínez. "Klippel-Trénaunay Syndrome and Arnold-Chiari Type I Malformation." Actas Dermo-Sifiliográficas (English Edition) 98, no. 6 (2007): 441–42. http://dx.doi.org/10.1016/s1578-2190(07)70482-1.

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Seliverstov, Yu A., Yu A. Shpilyukova, and S. N. Illarioshkin. "Are Some Eponyms in Neurology Used Correctly?" Russian neurological journal 25, no. 3 (2020): 45–50. http://dx.doi.org/10.30629/2658-7947-2020-25-3-45-50.

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The article analyzes the origin and competence of using several eponyms widely known in neurology. It is shown that some of them are not used correctly. So, the alternates “Jakob–Creutzfeldt disease” and “Arnold– Chiari malformation” are more correct. The eponym “Steele–Richardson–Olszewski syndrome” should not be used as a synonym for progressive supranuclear palsy syndrome. The historical aspects and correct variants of the use of a number of other neurological eponyms are highlighted in the article.

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Viana, Graça Maria de Castro, João Arnaud Diniz Neto, Igor de Sousa Furtado, José Roberto Binda Júnior, Bárbara Neiva Tanaka, and Maria do Desterro Soares Brandão Nascimento. "Association of HTLV-I with Arnold Chiari syndrome and syringomyelia." Brazilian Journal of Infectious Diseases 12, no. 6 (2008): 536–37. http://dx.doi.org/10.1590/s1413-86702008000600018.

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Pincherle, Alessandro, and Serge Bolyn. "Cerebellar syndrome after occipital nerve block: A case report." Cephalalgia 40, no. 10 (2020): 1123–26. http://dx.doi.org/10.1177/0333102420929015.

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Background Occipital nerve blocks are commonly used in the treatment of different types of refractory headaches. The procedure is considered safe, and serious complications have rarely been described. Case presentation We report a serious complication of occipital nerve blockade secondary to the penetration of local anesthetic and non-steroidal anti-inflammatory drugs into the posterior fossa in a patient affected by type I Arnold Chiari malformation. Conclusions This case reminds that a proper injection technique is mandatory to avoid potentially severe complications when performing occipital nerve blocks.

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Babintseva, Anastasiya, Yu Yu Khodzinska, І. V. Lastivka, О. І. Yurkiv, A. I. Roshka, and S. І. Basistyi. "ARNOLD-CHIARI MALFORMATION: LITERATURE REVIEW AND CLINICAL CASE IN SIBLINGS." Neonatology, surgery and perinatal medicine 11, no. 1(39) (2021): 58–64. http://dx.doi.org/10.24061/2413-4260.xi.1.39.2021.8.

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Arnold-Chiari malformation is a defectof the cervical-medullar transition characterized bydisplacement of the cerebellar tonsils and in a numberof cases when the stem and IV ventricle extend into theforamen magnum. There are four main types of pathology,and type II is found most often.The article presents a clinical case of type II ArnoldChiari malformation in siblings. The newborn girl born afterVII pregnancy and VII physiological delivery in the termof 39-40 weeks was under observation. US examination inthe terms of 20-21 and 34 weeks of gestation diagnoseda congenital developmental defect of the central nervoussystem characterized by a “lemon”-like shape of the brain,displacement of the brain structures in the portion of theforamen magnum, ventriculomegaly, a defect of the lumbarsacral portion with formation of hernia sac, and dropsy ofamnion. The family couple refused from interruption ofpregnancy and medical-genetic examination.The basic diagnosis of the child was congenitaldevelopmental defect of the central nervous system(type II Arnold-Chiari malformation: rachischisis andhydrocephalus) including complications such as inferiortorpid paraplegia and dysfunction of the pelvic organs.Investigation of hereditary anamnesis foundcompromised heredity on the mother’s side (her mother’ssibling has Down’s syndrome), and IV child in the familyis disabled from birth due to a congenital developmentaldefect of the central nervous system – type II Arnold-Chiarimalformation.The family couple refused from a comprehensivemedical-genetic consultation during the previous andcurrent pregnancies, and magnetic-resonance imaging ofthe child.

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Ponomarchuk, V., N. Naritsyna, N. Konovalova, T. Serebrina, and V. Reshetniak. "A case of combination of Eales disease and Arnold Chiari syndrome." Oftalmologicheskii Zhurnal 59, no. 1 (2016): 65–68. http://dx.doi.org/10.31288/oftalmolzh201616568.

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22

Barnet, Ann B., Ira P. Weiss, and Catherine Shaer. "EVOKED POTENTIALS IN INFANT BRAINSTEM SYNDROME ASSOCIATED WITH ARNOLD-CHIARI MALFORMATION." Developmental Medicine & Child Neurology 35, no. 1 (2008): 42–48. http://dx.doi.org/10.1111/j.1469-8749.1993.tb11550.x.

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23

Pastor Escartín, Felix, Vicent Quilis Quesada, Pau Capilla Guasch, et al. "Chiari I - Idiopathic Intracranial Hypertension Association After Failed Posterior Fossa Surgery. Case Report." JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA 30, no. 3 (2019): 252–60. http://dx.doi.org/10.22290/jbnc.v30i3.1852.

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Objectives: Several papers have been published relating the Idiopathic Intracranial Hypertension Syndrome (HTII) to the Arnold Chiari type I malformation (AC1M). Both entities have clinical and demographic similarities, a poorly defined etiology and, sometimes common therapeutic posibilities. A correlation between both entities has been suggested, especially in a subgroup of patients in whom posterior fossa decompression surgery fails. With regard to a case, we reviewed the literature and proposed our hypothesis about the origin of Chiari-HTII syndrome and its therapeutic possibilities. Case presentation: A 41year-old patient with mild obesity, menstrual abnormalities and empty Sella Turcicae, was operated on with an AC1M associating basilar impression and syringomyelia causing all together a centromedullary syndrome. After posterior fossa decompression surgery and successful arthrodesis, she improved in the immediate postoperative period. Nevertheless, she soon developed symptoms of intracranial hypertension (ICH), and showed increased opening pressure in lumbar puncture compatible with HTII syndrome. A ventriculoperitoneal shunt (VPS) was implanted with clinical improvement and 12 months later the syringomyelia was absent on in the magnetic resonance (MRI). Conclusion: The Chiari I-HTII syndrome is described as the coexistence of ICH symptoms after failed posterior fossa surgery, in patients with no flow MRI anomalies, and increased opening pressure at the lumbar puncture. In our experience, both entities seem to overlap in a common syndrome and must be taken into account, especially in patients with atypical onset of symptoms or patients in whom conservative treatment fails.

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Di Genova, Chiara, Socrates Charitos, Gabriella Ba, and Caterina Adele Viganò. "Case Report: Atypical psychotic onset of type I Arnold-Chiari malformation." F1000Research 4 (September 28, 2015): 915. http://dx.doi.org/10.12688/f1000research.6975.1.

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Introduction: We report a case of type I Arnold-Chiari malformation that is very peculiar because of its particular onset especially characterized by psychiatric symptoms. These symptoms were so prevailing that, for fifteen years, they masked the neurological aspects and the patient was treated with high doses of psychotropic drugs without any benefit. If the Arnold-Chiari malformation had been diagnosed before the development of severe hydro-syringomyelia, the patient could have underwent decompressive neurosurgery which may have improved her quality of life. It is worthwhile to highlight that psychotic symptoms may be caused by this congenital malformation, that typically has an aspecific onset. Therefore it’s important to consider an eventual organic etiology while challenging a resistant clinical picture with unusual presentation.Case description: A 51-year-old woman reported neurological symptoms consisting of headaches, blurred vision, diplopia, tinnitus, vertigo and psychiatric symptoms including obsessive ideas about the fear of killing her son, auditory and visual pseudo-hallucinations. The symptoms had developed suddenly at the age of 35 years and persisted thereafter. She underwent multiple hospitalizations in psychiatric units and was treated with a variety of psychopharmacological approaches without substantial improvement. We performed a brain MRI that identified a type I Arnold-Chiari malformation. We assessed psychiatric symptoms using the Structured Clinical Interview for DSM IV Axis I Disorders, the Brief Psychiatric Rating Scale, and the Yale-Brown Obsessive-Compulsive Scale. A tailored psychopharmacological therapy led to a partial improvement in mood and anxiety but not in hallucinations.Discussion: We want to highlight how important is, in everyday psychiatric clinical practice, not to focus only on psychiatric aspects but consider the patient globally, because in this case psychiatric problems were the onset presentation of a rare neurological syndrome.

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Van den Bergh, Raymond. "Headache caused by craniospinal pressure dissociation in the Arnold-Chiari-syringomyelia syndrome." Journal of Neurology 239, no. 5 (1992): 263–66. http://dx.doi.org/10.1007/bf00810349.

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26

Mimura, T., S. Asajima, Y. Saruhashi, and Y. Matsusue. "A case of Arnold–Chiari syndrome with flaccid paralysis and huge syringomyelia." Spinal Cord 42, no. 9 (2004): 541–44. http://dx.doi.org/10.1038/sj.sc.3101607.

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27

Holder-Espinasse, Muriel, and Robin M. Winter. "Type 1 Arnold???Chiari malformation and Noonan syndrome. A new diagnostic feature?" Clinical Dysmorphology 12, no. 4 (2003): 275. http://dx.doi.org/10.1097/00019605-200310000-00013.

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VIDOR, ARTHUR CASTILHOS, ARTHUR REGINATO CORBELLINI, LARISSA CASTILHOS VIDOR, et al. "REPORT OF DENTAL ALTERATIONS IN AN ARNOLD-CHIARI TYPE 2 SYNDROME PATIENT." Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology 129, no. 1 (2020): e100. http://dx.doi.org/10.1016/j.oooo.2019.06.421.

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29

Grazzi, Licia, and Frank Andrasik. "Headaches and Arnold-Chiari Syndrome: When to Suspect and How to Investigate." Current Pain and Headache Reports 16, no. 4 (2012): 350–53. http://dx.doi.org/10.1007/s11916-012-0270-2.

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30

Dadali, E. L., T. V. Markova, and O. P. Ryzhkova. "Aymé–Gripp syndrome in a Russian patient with a newly detected mutation in the MAF gene." Neuromuscular Diseases 11, no. 3 (2021): 64–68. http://dx.doi.org/10.17650/2222-8721-2021-11-3-64-68.

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Aymé–Gripp syndrome is a rare autosomal dominant syndrome caused by mutations in the MAF gene and is characterized by a pronounced phenotypic polymorphism. The core of clinical signs consists of congenital cataracts, sensorineural hearing loss, specific dysmorphic facial features and intellectual disabilities. With varying frequency, patients have: radioulnar synostosis, Arnold–Chiari malformation, aseptic pericarditis, dental anomaly and osteoarthritis. The article presents the clinical and genetic characteristics of the first Russian patient with Aymé–Gripp syndrome caused by a newly identified mutation s.173C>A (p.Thr58Asn NM_005360.4) in a heterozygous state in the MAF gene. The influence of the lo calization and type of amino acid substitutions in the protein product of the gene on the severity and specificity of the clinical manifestations of the syndrome is discussed.

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Rodríguez-Blanque, Raquel, Cristina Almazán-Soto, Beatriz Piqueras-Sola, et al. "Chiari Syndrome: Advances in Epidemiology and Pathogenesis: A Systematic Review." Journal of Clinical Medicine 12, no. 20 (2023): 6694. http://dx.doi.org/10.3390/jcm12206694.

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Arnold Chiari syndrome is a rare congenital disease of unknown prevalence and whose origin is still under study. It is encompassed within the posterior cranial malformations, showing a wide spectrum of symptomatology that can range from severe headache, dizziness, and paresthesia to complete asymptomatology. It is for this reason that early diagnosis of the disease is difficult, and it is usually diagnosed in adolescence. Treatment is based on remodeling and decompression of the malformed posterior cranial fossa, although the risk of residual symptoms after surgery is high. The aim of this review is to update all the existing information on this pathology by means of an exhaustive analysis covering all the scientific literature produced in the last 5 years. In addition, it has been carried out following the PRISMA model and registered in PROSPERO with code CRD42023394490. One of the main conclusions based on the results obtained in this review is that the origin of the syndrome could have a genetic basis and that the treatment of choice is the decompression of the posterior cerebral fossa.

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Alvarez, D., I. Requena, M. Arias, L. Valdes, I. Pereiro, and R. De la Torre. "Acute respiratory failure as the first sign of Arnold-Chiari malformation associated with syringomyelia." European Respiratory Journal 8, no. 4 (1995): 661–63. http://dx.doi.org/10.1183/09031936.95.08040661.

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We report a rare case of acute respiratory failure in a previously asymptomatic patient showing clinical signs of inferior cranial nerve palsy together with weakness and muscular atrophy of the upper limbs. Magnetic resonance imaging revealed Arnold-Chiari malformation associated with platybasia, basilar impression, syringomyelia and Klippel-Feil syndrome. Episodes of apnoea required tracheostomy and recurred upon tentative closure of the tracheostome, but remitted upon decompression of the posterior fossa. This case involved both obstructive mechanisms and dysfunction of the respiratory centre. Patients with respiratory failure not explained by pulmonary pathology should be checked for underlying neurological disease.

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Cakmakkaya, Ozlem S., Guner Kaya, Fatis Altintas, Mefkur Bakan, and Abdullah Yildirim. "Anesthetic management of a child with Arnold-Chiari malformation and Klippel-Feil syndrome." Pediatric Anesthesia 16, no. 3 (2006): 355–56. http://dx.doi.org/10.1111/j.1460-9592.2005.01795.x.

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Ramesh, Chandana, and Dr Meenakshi Parthasarathi. "A rare case of Arnold-Chiari syndrome in a primigravida and its outcome." International Journal of Obstetrics and Gynaecology 5, no. 1 (2023): 05–06. http://dx.doi.org/10.33545/26648334.2023.v5.i1a.30.

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Tisano, Adriana, Angelo Alito, Rita Ragonese de Gregorio, et al. "The Benefits of Cognitive Therapeutic Exercise in Symptomatic Arnold–Chiari Syndrome Type I: A Case Report on Gait, Balance, and Pain Management." Journal of Clinical Medicine 13, no. 18 (2024): 5502. http://dx.doi.org/10.3390/jcm13185502.

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Background: Chiari malformation is a rare congenital condition in which the cerebellar tonsils herniate through the foramen magnum, causing symptoms related to compression of the surrounding structures. Rehabilitation plays a key role in the pre- and post-operative management of the syndrome, as it can improve strength, range of motion, motor coordination, pain management, and performance of activities of daily living. Methods: This article presents the case of a 43-year-old woman with Chiari malformation 1B who underwent resection of the filum terminale. She presented as an outpatient at the University Hospital “G. Martino” in Messina, complaining of difficulty walking, balance problems, lumbar pain, and heaviness in the lower limbs. Following a multidisciplinary assessment, she underwent an 11-month rehabilitation protocol based on cognitive therapeutic exercise. Results: The patient achieved significant improvements in pain, mental confusion, and quality of life after treatment and at the 12-month follow-up. Conclusions: The results of this study highlight the significant benefits of cognitive therapeutic exercises in Chiari malformation, with improvements in several key areas, including quality of life, pain management, and ability to perform activities of daily living.

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Araújo, Daniel Lopes, Maxsuel Pereira do Nascimento, Maria Clara Teles de Souza, et al. "Anatomoclinical correlation and main radiological findings in a patient affected by Arnold Chiari's malformation: a review." Research, Society and Development 10, no. 4 (2021): e1201048130. http://dx.doi.org/10.33448/rsd-v10i4.8130.

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Chiari's malformation is within the spectrum of congenital abnormalities of the central nervous system (CNS). It is characterized by a complex deformity of the posterior fossa associated almost always with a malformation of the spine. Chiari malformation is still a subject of little evidence in literature, where new studies on this parameter may trigger influences for researchers in the field. The objectives of this study were to describe the main alterations found in the nervous system in individuals with Chiari malformation. It is a literature review study. The following descriptors were used for the search: Chiari malformation, Posterior Pit and Craniovertebral Junction (together and separately). In the selection criteria, full articles were chosen for the period: 2016 - 2019, in the languages: Portuguese and English. The searches were made in the databases: Scielo, Pubmed and Google academic. The articles were selected first by title, then by abstract, and finally by full reading. Based on the established criteria, 3 articles were selected that contemplate the theme. The literature shows that the most suggestive brain alterations of this pathology are cerebellar abnormalities such as caudal displacement of the bridge, IV ventricle and bulb; medullary torsion; IV ventricle in abnormal shape and hypoplasia of the cerebellum tent and mesencephalic ceiling. It is noticeable that Chiari's malformation can cause alterations that can vary from lighter to more severe. New studies are needed on this subject, as well as solutions to improve the quality of life of patients affected by this syndrome.

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Santalova, G. V., E. A. Tuturova, O. V. Sluzhaeva, Yu V. Serezhkina, and A. V. Sofina. "Multifaceted autonomic dysfunction syndrome: A case of combined Kimmerle`s anomaly and Chiari malformation." Complex Issues of Cardiovascular Diseases 12, no. 1 (2023): 129–34. http://dx.doi.org/10.17802/2306-1278-2023-12-1-129-134.

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Highlights. Autonomic dysfunction syndrome is an urgent issue in modern medicine, however, it should be an exceptional diagnosis, established after a comprehensive diagnostic study, as many diseases can often appear as this syndrome. The presented case describes the patient with a combined pathology of Kimmerle`s anomaly and Chiari malformation, who was diagnosed with autonomic dysfunction long time ago.Abstract. Autonomic dysfunction syndrome (ADS) is a topical issue of modern medicine. Differential diagnosis of this syndrome is often a difficult task for a practicing physician, because an organic disease of various etiologies can be hidden under the guise of autonomic dysfunction. The article analyzes a clinical case of a patient with a combined pathology of Kimmerle`s and Arnold-Chiari anomalies, which were not diagnosed for a long time due to the fact that clinical symptoms (syncope and presyncope conditions, dizziness, pain in the occipital region, aggravated by physical exertion and emotional stress, rises in blood pressure up to 160 mm Hg) are identical to those in ADS. After prolonged treatment of ADS without any positive effect, the child was sent to the Research Clinical Institute of Pediatrics and Pediatric Surgery named after academician Yuri Veltischev. Having received surgical treatment, the patient had no complaints for 6 months. The presented clinical case confirms that the diagnosis of ADS should be an exceptional diagnosis, established only after a complex and comprehensive examination.

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P., Salini Raani J., Ashwin Rao, Madhan Balu, and Rashmi Rao. "Diastematomyelia differences in management of diastemetomyelia with associated abnormalities versus isolated diastemetomyelia: a case series." International Journal of Reproduction, Contraception, Obstetrics and Gynecology 9, no. 11 (2020): 4688. http://dx.doi.org/10.18203/2320-1770.ijrcog20204834.

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Diastematomyelia (DM), also known as split cord malformation (SCM) is a type of spinal dysraphism. It is a very rare congenital spinal anomaly characterized by clefting of the spinal cord due to a partial or complete bony or fibrous septum within the spinal canal with splaying of the posterior spinal elements resulting in localized division of the spinal cord into two parts on either side of the septum which typically reunite below the cleft. The pathology was first described by Cruvelhier in 1853. About 1-3 per 1000 live birth, is the estimated incidence of spinal dysraphism and neural tube defects (NTD) occurs more commonly in females (55-70%). Prenatal diagnosis of DM is possible by ultrasonography (USG). The clinical significance of DM is that it may manifest as an isolated abnormality or in association with other spinal abnormalities such as spina bifida, Arnold-Chiari malformation, hemivertebra, butterfly vertebra, kyphoscoliosis or part of Jarcho-Levin syndrome. The management of pregnancy with a foetus diagnosed with DM antenatally, differs based on whether the foetus has an isolated DM with intact skin or DM with more serious associated anomalies. We present two cases of Foetal DM both diagnosed by antenatal USG, Case 1 was diagnosed at 16 weeks gestation age (GA) with DM associated with Type II Arnold-Chiari malformation, hydrocephalus and case 2 was diagnosed with isolated DM at 19 weeks 2 days GA.

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Al-Zamil, M., and N. G. Kulikova. "Phenocopy of Amyotrophic Lateral Sclerosis in Patients with Chiari 1 Malformation Associated Syringomyelia: Brief Literature Review." Personalized Psychiatry and Neurology 3, no. 2 (2023): 25–31. http://dx.doi.org/10.52667/2712-9179-2023-3-2-25-31.

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Arnold-Chiari malformation or Chiari malformation (CM1) is the name of a group of deformities of the posterior fossa and hindbrain (cerebellum, pons and medulla oblongata). The pathogenetic basis of this disease is associated with herniation of the cerebellar tonsils through the foramen magnum. CM1 is classified as a rare disease. CM1 can present with a wide variety of symptoms, also non-specific, with consequent controversies on diagnosis and surgical decision-making, particularly in asymptomatic or minimally symptomatic. Syringomyelia (Syr), hydrocephalus, craniocervical instability, encephalocele, scoliosis, spina bifida and spinal dysraphism are the most common comorbidities that may present at the time of diagnosis or develop secondarily. Most attention has been paid to syringomyelia complicated by CM1 (CM1-related Syr). Formation of single or multiple fluid-filled cavities in the spinal cord and/or bulb as a result of pulse changes in intracranial pressure associated with disruption of normal cerebrospinal fluid circulation due to morphological abnormalities of the brain at the magnum level. This condition can be complicated by a rarer disease caused by the development of damage to the anterior horns of the spinal cord - amyotrophic sclerosis (ALS syndrome). In this brief literature review we are trying to demonstrate the mean pathogenic basis of amyotrophic lateral sclerosis in patients with chiari 1 malformation associated syringomyelia.

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Xinyu, Guo, Zhu Na, and Hao Dingjun. "Rare Hereditary Klippel-Feil Syndrome and Arnold-Chiari Malformation Caused by Cervical Spondylotic Myelopathy." World Neurosurgery 125 (May 2019): 126–28. http://dx.doi.org/10.1016/j.wneu.2018.12.101.

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Ireland, P. D., D. Mickelsen, T. G. Rodenhouse, R. S. Bakos, and B. Goldstein. "Evaluation of the Autonomic Cardiovascular Response in Arnold-Chiari Deformities and Cough Syncope Syndrome." Archives of Neurology 53, no. 6 (1996): 526–31. http://dx.doi.org/10.1001/archneur.1996.00550060068018.

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Covantev, Serghei, Rasul Uzdenov, Kseniya Zabudskaya, and Olga Belic. "Posterior midline cleft of the atlas – a crucial anatomical variation in vertebral fractures." Iberoamerican Journal of Medicine 3, no. 3 (2021): 284–87. http://dx.doi.org/10.53986/ibjm.2021.0045.

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The anatomy of the first vertebra, namely atlas, has significant clinical implications. Atlas is situated between the occipital bone and the second cervical vertebra (axis) and is one of the main points of head movement. Most congenital anomalies of the vertebra are diagnosed incidentally during imaging investigations and can be associated with cervical spine anomalies. The neurological symptoms may include weakness in the four limbs, acute neurologic deficits such as transient quadriparesis, paraparesis, Lhermitte's sign, chronic neck pain, and headache. This anomaly is also commonly seen in gonadal dysgenesis, Klippel-Feil syndrome, Arnold-Chiari malformations, and Turner and Down syndrome. Unlike other variations, which arise due to disturbances of ossification posterior midline clefts of the atlas, are different since they are a developmental failure of chondrogenesis. We therefore present an anatomical case and analysis of the literature about posterior arch clefts of atlas.

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Serghei, Covantev, Uzdenov Rasul, Zabudskaya Kseniya, and Belic Olga. "Posterior midline cleft of the atlas – a crucial anatomical variation in vertebral fractures." Iberoamerican Journal of Medicine 3, no. 3 (2021): 284–87. https://doi.org/10.5281/zenodo.5034577.

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The anatomy of the first vertebra, namely atlas, has significant clinical implications. Atlas is situated between the occipital bone and the second cervical vertebra (axis) and is one of the main points of head movement. Most congenital anomalies of the vertebra are diagnosed incidentally during imaging investigations and can be associated with cervical spine anomalies. The neurological symptoms may include weakness in the four limbs, acute neurologic deficits such as transient quadriparesis, paraparesis, Lhermitte's sign, chronic neck pain, and headache. This anomaly is also commonly seen in gonadal dysgenesis, Klippel-Feil syndrome, Arnold-Chiari malformations, and Turner and Down syndrome. Unlike other variations, which arise due to disturbances of ossification posterior midline clefts of the atlas, are different since they are a developmental failure of chondrogenesis. We therefore present an anatomical case and analysis of the literature about posterior arch clefts of atlas.

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44

Das, Joydeep, Krishnendu Choudhury, Shweta Kothari, and NavneetJeevan Nayek. "Co-existence of russell–silver syndrome with arnold–chiari type 1: A new syndrome or just an association." Journal of the Pediatrics Association of India 9, no. 4 (2020): 160. http://dx.doi.org/10.4103/jpai.jpai_20_21.

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Pistone, G., M. Malaguarnera, M. Motta, I. Vecchio, R. Raffaele, and L. Rampello. "Dementia due to acute hyponatremic encephalopathy in an elderly patient with Arnold-Chiari-I syndrome." Archives of Gerontology and Geriatrics 35 (January 2002): 279–82. http://dx.doi.org/10.1016/s0167-4943(02)00143-7.

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Hung, Po-Cheng, Huei-Shyong Wang, and Tai-Ngar Lui. "Coexistence of oto-palato-digital syndrome type II and Arnold–Chiari I malformation in an infant." Brain and Development 21, no. 7 (1999): 488–90. http://dx.doi.org/10.1016/s0387-7604(99)00044-3.

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47

Nowakowski, Przemek. "TCTAP C-198 Patient with Arnold-Chiari Syndrome and Cervical, Subclavian and VCS Occlusion Endovascular Treatment." Journal of the American College of Cardiology 73, no. 15 (2019): S259—S260. http://dx.doi.org/10.1016/j.jacc.2019.03.404.

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Tsvetovsky, Sergey Borisovich, Vyacheslav Vladimirovich Stupak, Aleksandr Borisovich Dmitriev, Galina Vladimirovna Tomash, and Irina Alekseyevna Gribacheva. "ELECTRONEUROPHYSIOLOGICAL PARAMETERS OF THE BRAIN IN PATIENTS WITH CHIARI MALFORMATION BEFORE AND AFTER SURGERY." Hirurgiâ pozvonočnika, no. 2 (June 22, 2006): 055–61. http://dx.doi.org/10.14531/ss2006.2.55-61.

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Objective. The impartial instrument assessment of a nature and degree of cephalic and cervical spinal cord functional abnormalities and dynamics of their changes in patients with Chiari malformation before and after surgery. Material and Methods. Visual evoked potentials (VEP), somatosensory (SSEP) and brainstem auditory evoked potentials (BAEP), and motor responses to the transcranial magnetic stimulation (TMS) were registered in 28 patients preoperatively and in 12–30 days postoperatively. Results. Initial VEP and SSEP showed deviations from normal values caused by hydrocephalus and increased intracranial pressure. In majority of patients a latent time of VEP P100 was abnormally short. Lateral asymmetries of P100 delay and amplitude, and VEP shape changes were also observed. P100 delays increase and VEP asymmetry were observed when foci of vascular dysmetabolic genesis and dystrophic changes were present. SSEP had changes in N30 cortical components. Initial N20 components were radically changed only in patients with cervical syringomyelia. The same patients had the worst M-responses to the TMS. BAEP were registered with decreased V component and increased interpeak IV–V interval. Conclusion. Arnold – Chiari syndrome is reflected in various deviations of electrophysiological parameters characterizing a functional condition of the central nervous system. Postoperative dynamics showed prevalence of positive shifts and normalization of electrophisiological parameters

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Tsvetovsky, Sergey Borisovich, Vyacheslav Vladimirovich Stupak, Aleksandr Borisovich Dmitriev, Galina Vladimirovna Tomash, and Irina Alekseyevna Gribacheva. "ELECTRONEUROPHYSIOLOGICAL PARAMETERS OF THE BRAIN IN PATIENTS WITH CHIARI MALFORMATION BEFORE AND AFTER SURGERY." Hirurgiâ pozvonočnika, no. 3 (September 12, 2006): 076–83. http://dx.doi.org/10.14531/ss2006.3.76-83.

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Objective. The impartial instrument assessment of a nature and degree of cephalic and cervical spinal cord functional abnormalities and dynamics of their changes in patients with Chiari malformation before and after surgery. Material and Methods. Visual evoked potentials (VEP), somatosensory (SSEP) and brainstem auditory evoked potentials (BAEP), and motor responses to the transcranial magnetic stimulation (TMS) were registered in 28 patients preoperatively and in 12–30 days postoperatively. Results. Initial VEP and SSEP showed deviations from normal values caused by hydrocephalus and increased intracranial pressure. In majority of patients a latent time of VEP P100 was abnormally short. Lateral asymmetries of P100 delay and amplitude, and VEP shape changes were also observed. P100 delays increase and VEP asymmetry were observed when foci of vascular dysmetabolic genesis and dystrophic changes were present. SSEP had changes in N30 cortical components. Initial N20 components were radically changed only in patients with cervical syringomyelia. The same patients had the worst M-responses to the TMS. BAEP were registered with decreased V component and increased interpeak IV–V interval. Conclusion. Arnold – Chiari syndrome is reflected in various deviations of electrophysiological parameters characterizing a functional condition of the central nervous system. Postoperative dynamics showed prevalence of positive shifts and normalization of electrophisiological parameters

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Jacome, DE. "Headache in Ehlers—Danlos Syndrome." Cephalalgia 19, no. 9 (1999): 791–96. http://dx.doi.org/10.1046/j.1468-2982.1999.1909791.x.

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Objective: Ehlers—Danlos Syndrome (EDS) is a complex hereditary connective tissue disorder with neurologic manifestations that include cerebrovascular disorders and chronic pain. The clinical data collected on 18 patients with EDS and chronic headaches is reported. Procedure: Clinical history, neurologic examination, computerized tomography of the head, magnetic resonance imaging (MRI) of the brain, and electroencephalogram (EEG). Headaches were classified according to the International Headache Society and the patients were followed by the author for a minimum of 2 years. Findings: Four patients had migraine with aura, four had migraine without aura, four had tension headaches, four had a combination of migraine and tension headaches, and two had post-traumatic headaches. Nine patients exhibited blepharoclonus but none had history of seizures and their EEGs were normal, ruling out eye closure epilepsy. Although one patient had a small right frontal angioma, a second had Arnold Chiari malformation type I, and a third had an old stroke, headaches did not clinically correlate with their central nervous system (CNS) lesions. Conclusion: Chronic recurrent headaches may constitute the neurologic presentation of EDS in the absence of structural, congenital, or acquired CNS lesions that correlate with their symptoms. Individuals with EDS may be prone to migraine due to an inherent disorder of cerebrovascular reactivity or cortical excitability. Additional studies are needed to elucidate the pathogenesis of headaches in EDS.

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