Factitious disorder in a patient with Arnold-Chiari malformation: A case report

IntroductionThe factitious disorder is characterized by simulation or exaggeration by the subject of his/her physical or psychological symptoms to take a sick role. This disorder may be associated with a real disease, used to simulate other symptoms and receive treatment. Our case is represented by a 49-year-old man, affected by Arnold Chiari Malformation Type I (ACM-I), a structural defect in the cerebellum with extension of the cerebellar tonsils into the foramen magnum, without involving the brain stem. The patient had three surgical operations involving the complete resolution of the disease. However, the subject reported a worsening of all symptoms, with the outcome of a severe self-limitation, with admission to a residential care and taken over by the multidisciplinary equipe of the district. Symptoms, which mainly consisted in pain, motor functional impairment, headaches, slurred speech were not evaluated due to the ACM-I. This diagnosis was confirmed when there was the complete resolution of the symptoms in a date pointed by the patient.MethodsTo make an appropriate differential diagnosis, in addition to the neurological examination, the subject had psychiatric interviews and completed an MMPI evaluation.ResultsNeurological examinations revealed no residual outcome of the ACM-I. At MMPI there were not significant peaks. Regarding to the attitude towards this test and its validity, it appears an obvious attempt to lie from the patient, which tries to present himself in a favourable unrealistically way.ConclusionThis case shows that appropriate diagnosis can help health services in a better management of their resources in such situations.Disclosure of interestThe authors have not supplied their declaration of competing interest.

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Foramen magnum decompression for the treatment of Arnold Chiari malformation type I with associated syringomyelia in an elderly patient

Spinal Cord ◽

10.1038/sj.sc.3101675 ◽

2004 ◽

Vol 43 (4) ◽

pp. 249-251 ◽

Cited By ~ 6

Author(s):

I Takigami ◽

K Miyamoto ◽

H Kodama ◽

H Hosoe ◽

S Tanimoto ◽

...

Keyword(s):

Elderly Patient ◽

Chiari Malformation ◽

Foramen Magnum ◽

Type I ◽

Foramen Magnum Decompression ◽

Chiari Malformation Type I ◽

Arnold Chiari Malformation

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Prognostic significance of C1–C2 facet malalignment after surgical decompression in adult Chiari malformation type I: a pilot study based on the Chicago Chiari Outcome Scale

Journal of Neurosurgery Spine ◽

10.3171/2020.6.spine20544 ◽

2020 ◽

pp. 1-7

Author(s):

Michael Lumintang Loe ◽

Tito Vivas-Buitrago ◽

Ricardo A. Domingo ◽

Johan Heemskerk ◽

Shashwat Tripathi ◽

...

Keyword(s):

Pilot Study ◽

Chiari Malformation ◽

Prognostic Significance ◽

Foramen Magnum ◽

Bivariate Analysis ◽

Type I ◽

Foramen Magnum Decompression ◽

Swallowing Function ◽

Chiari Malformation Type I

OBJECTIVEThe authors assessed the prognostic significance of various clinical and radiographic characteristics, including C1–C2 facet malalignment, in terms of surgical outcomes after foramen magnum decompression of adult Chiari malformation type I.METHODSThe electronic medical records of 273 symptomatic patients with Chiari malformation type I who were treated with foramen magnum decompression, C1 laminectomy, and duraplasty at Mayo Clinic were retrospectively reviewed. Preoperative and postoperative Neurological Scoring System scores were compared using the Friedman test. Bivariate analysis was conducted to identify the preoperative variables that correlated with the patient Chicago Chiari Outcome Scale (CCOS) scores. Multiple linear regression analysis was subsequently performed using the variables with p < 0.05 on the bivariate analysis to check for independent associations with the outcome measures. Statistical software SPSS version 25.0 was used for the data analysis. Significance was defined as p < 0.05 for all analyses.RESULTSFifty-two adult patients with preoperative clinical and radiological data and a minimum follow-up of 12 months were included. Motor deficits, syrinx, and C1–C2 facet malalignment were found to have significant negative associations with the CCOS score at the 1- to 3-month follow-up (p < 0.05), while at the 9- to 12-month follow-up only swallowing function and C1–C2 facet malalignment were significantly associated with the CCOS score (p < 0.05). Multivariate analysis showed that syrinx presence and C1–C2 facet malalignment were independently associated with the CCOS score at the 1- to 3-month follow-up. Swallowing function and C1–C2 facet malalignment were found to be independently associated with the CCOS score at the 9- to 12-month follow-up.CONCLUSIONSThe observed results in this pilot study suggest a significant negative correlation between C1–C2 facet malalignment and clinical outcomes evaluated by the CCOS score at 1–3 months and 9–12 months postoperatively. Prospective studies are needed to further validate the prognostic value of C1–C2 facet malalignment and the potential role of atlantoaxial fixation as part of the treatment.

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Standard and cardiac-gated phase-contrast magnetic resonance imaging in the clinical course of patients with Chiari malformation Type I

Neurosurgical FOCUS ◽

10.3171/2011.7.focus11105 ◽

2011 ◽

Vol 31 (3) ◽

pp. E5 ◽

Cited By ~ 19

Author(s):

Uwe Max Mauer ◽

Andreas Gottschalk ◽

Carolin Mueller ◽

Linda Weselek ◽

Ulrich Kunz ◽

...

Keyword(s):

Mr Imaging ◽

Chiari Malformation ◽

Phase Contrast ◽

Foramen Magnum ◽

Central Canal ◽

Type I ◽

Csf Flow ◽

Chiari Malformation Type I ◽

Cerebrospinal Fluid Flow ◽

Contrast Magnetic Resonance

Object The causal treatment of Chiari malformation Type I (CM-I) consists of removing the obstruction of CSF flow at the level of the foramen magnum. Cerebrospinal fluid flow can be visualized using dynamic phase-contrast MR imaging. Because there is only a paucity of studies evaluating CSF dynamics in the region of the spinal canal on the basis of preoperative and postoperative measurements, the authors investigated the clinical usefulness of cardiacgated phase-contrast MR imaging in patients with CM-I. Methods Ninety patients with CM-I underwent preoperative MR imaging of CSF pulsation. Syringomyelia was present in 59 patients and absent in 31 patients. Phase-contrast MR imaging of the entire CNS was used to investigate 22 patients with CM-I before surgery and after a mean postoperative period of 12 months (median 12 months, range 3–33 months). In addition to the dynamic studies, absolute flow velocities, the extension of the syrinx, and tonsillar descent were also measured. Results The changes in pulsation were highly significant in the region of the (enlarged) cistern (p = 0.0005). Maximum and minimum velocities (the pulsation amplitude) increased considerably in the region where the syrinx was largest in diameter. The changes of pulsation in these patients were significant in the subarachnoid space in all spinal segments but not in the syrinx itself and in the central canal. Conclusions The demonstration of CSF flow pulsation can contribute to assessments of surgical outcomes. The results presented here, however, raise doubts about current theories on the pathogenesis of syringomyelia.

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A Case of Chiari Malformation Type I : Improvement of Syringomyelia and Scoliosis by Foramen Magnum Decompression Alone

Japanese Journal of Neurosurgery ◽

10.7887/jcns.8.727 ◽

1999 ◽

Vol 8 (11) ◽

pp. 727-731

Author(s):

Tetsuya Kubota ◽

Kenichi Nishiyama ◽

Akira Tamura ◽

Kouichi Kawasaki ◽

Hiroshi Masuda ◽

...

Keyword(s):

Chiari Malformation ◽

Foramen Magnum ◽

Type I ◽

Foramen Magnum Decompression ◽

Chiari Malformation Type I

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Trzyipółletni chłopiec z malformacją Chiariego typu I – opis przypadku

Nowa Pediatria ◽

10.25121/np.2017.21.3.87 ◽

2017 ◽

Vol 21 (3) ◽

Author(s):

Anna Worch ◽

Małgorzata Wielopolska

Keyword(s):

Chiari Malformation ◽

Foramen Magnum ◽

Common Type ◽

Type I ◽

Chiari Malformation Type I ◽

Chiari Type I Malformation ◽

Large Head ◽

Exacerbation Of Symptoms ◽

Chiari Type I ◽

Cerebellar Tonsils

Chiari malformation is the most common structural defect of the posterior fossa and cerebellum, consisting of displacements of hindbrain inclination through the foramen magnum, often associated with syringomyelia. In the most common type I of this disease, only the cerebellar tonsils are descended. Chiari type I malformation may remain asymptomatic in early childhood, however a number of unspecific symptoms, such as: neck pains and headaches, vertigos, balance and sight disorders, may strengthen with age. Because of unspecificity and various exacerbation of symptoms the disease is often misdiagnosed or undiagnosed. We describe a case of a 3.5-year old boy with whom his mother visited a pediatrician because of a chronic cough. During the examination mother of the patient informed that all infections start with vomiting and the patient is not able to stand up from horizontal position without turning first to the side and then to the abdomen. During the examination, a large head circumference was noticed (56 cm, > 97 percentile). MRI showed elongated cerebellar tonsils descended below the foramen magnum (left 14 mm, right 11 mm). Chiari malformation type I with associated syringomyelia was diagnosed. The patient was reffered to neurosurgery consultation.

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Arnold-Chiari Malformation Type I Associated with Syringomyelia and Atlantoaxial Instability: A Case Report

Ewha Medical Journal ◽

10.12771/emj.1998.21.4.279 ◽

1998 ◽

Vol 21 (4) ◽

pp. 279

Author(s):

Eun Jong Kim ◽

Myung Shin Kim ◽

Ju Young Lee ◽

Jin Won Whang ◽

Tae Sik Yoon

Keyword(s):

Case Report ◽

Chiari Malformation ◽

Atlantoaxial Instability ◽

Type I ◽

Chiari Malformation Type I ◽

Arnold Chiari Malformation

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Osteopetrosis with Arnold Chiari malformation type I and brain stem compression

The Indian Journal of Pediatrics ◽

10.1007/s12098-007-0070-6 ◽

2007 ◽

Vol 74 (4) ◽

pp. 412-415 ◽

Cited By ~ 18

Author(s):

M. L. Kulkarni ◽

S. N. Marakkanavar ◽

S. Sushanth ◽

N. Pradeep ◽

C. Ashok ◽

...

Keyword(s):

Brain Stem ◽

Chiari Malformation ◽

Type I ◽

Chiari Malformation Type I ◽

Arnold Chiari Malformation

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Arnold-Chiari Malformation Type I Appearing after Tonsillectomy

Anesthesiology ◽

10.1097/00000542-198707000-00025 ◽

1987 ◽

Vol 67 (1) ◽

pp. 120-121 ◽

Cited By ~ 8

Author(s):

Mai-Li Dong

Keyword(s):

Chiari Malformation ◽

Type I ◽

Chiari Malformation Type I ◽

Arnold Chiari Malformation

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Surgical outcomes in adult patients with syringomyelia associated with Chiari malformation Type I: the relationship between scoliosis and neurological findings

Journal of Neurosurgery Spine ◽

10.3171/spi.2007.6.3.216 ◽

2007 ◽

Vol 6 (3) ◽

pp. 216-221 ◽

Cited By ~ 24

Author(s):

Atsushi Ono ◽

Futoshi Suetsuna ◽

Kazumasa Ueyama ◽

Toru Yokoyama ◽

Shuichi Aburakawa ◽

...

Keyword(s):

Chiari Malformation ◽

Clinical Status ◽

Japanese Orthopaedic Association ◽

Foramen Magnum ◽

Neurological Dysfunction ◽

Type I ◽

Chiari Malformation Type I ◽

Group A ◽

The Mean ◽

Group B

Object The clinical characteristics of pediatric scoliosis associated with syringomyelia have been reported in previous studies, but scoliosis associated with syringomyelia in adults is rarely treated, and there is a paucity of detailed studies. In the present study of adult syringomyelia associated with Chiari malformation Type I, the authors investigated the relationships among the syrinx, scoliosis, and neurological data. Methods The population was composed of 27 patients (≥ 20 years of age) who underwent foramen magnum decompression for the treatment of syringomyelia. The patients were divided into two groups: those with scoliosis of 10° or more (Group A) and those without scoliosis (Group B). The authors assessed the length of the syrinx, duration of morbidity, and clinical status before and after surgery based on the Japanese Orthopaedic Association (JOA) Scale. There were 15 cases in Group A and 12 in Group B. The mean length of the syrinx was 12.8 vertebral bodies (VBs) in Group A and 7.2 VBs in Group B. The mean duration of morbidity was 14.2 years in Group A and 6.8 years in Group B. The mean preoperative JOA score was 10.1 in Group A and 14.4 in Group B, whereas the mean postoperative JOA scores were 11.9 and 15.8, respectively. There were significant differences between Groups A and B in length of the syrinx, duration of morbidity, and pre- and postoperative JOA scores. Conclusions In patients with syringomyelia and scoliosis the syringes spanned a greater number of VBs, the duration of morbidity was greater, neurological dysfunction was more severe, and surgical results were poorer. Scoliosis could be a predicting factor of the prognosis in patients with syringomyelia and Chiari malformation Type I.

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