Impacted cisterna magna without syringomyelia associated with spastic paraparesis: case report

Gonçalves da Silva, José Alberto; Costa, Maria do Desterro Leiros da; Holanda, Maurus Marques de Almeida; Melo, Luiz Ricardo Santiago; Araújo, Antônio Fernandes Almeida de; Viana, André Pachelli Bezerra

doi:10.1590/S0004-282X2006000400030

Abstracts

We report on a 49 year old man with impacted cisterna magna without the presence of syringohydromyelie (SM). The clinical picture was characterized by spastic paraparesis. Magnetic resonance imaging depicted a cisterna magna filled by the cerebellar tonsils. Six months after osteodural-neural decompression of the posterior fossa there was resolution of neurological symptoms and signs with the exception of hyperactive patellar and Achilles reflexes.

tight cisterna magna; impacted cisterna magna; Chiari malformation; posterior fossa decompression; spastic paraparesis

Hans Chiari (1891, 1895) descreveu 4 tipos de anomalias cerebelares que, ulteriormente, foram denominadas de malformação de Chiari (MC). Iskandar et al. (1998) relataram 5 casos de hidrosiringomielia (SM) sem herniação rombencefálica, nos quais as tonsilas cerebelares preenchiam a cisterna magna. Todos os casos melhoraram após a descompressão da fossa posterior com redução do tamanho da cavidade siringomiélica. Relatamos o caso de paciente de 49 anos com cisterna magna impactada sem a presença de siringo-hidromielia (SM). O quadro clínico caracterizava-se por paraparesia espástica. A ressonância magnética evidenciou a cisterna magna preenchida pelas tonsilas cerebelares. Seis meses após a descompressão osteodural-neural da fossa posterior, houve resolução dos sintomas e sinais neurológicos, com exceção da hiperatividade dos reflexos patelares e aquileus.

cisterna magna impactada; malformação de Chiari; descompressão da fossa posterior; paraparesia espástica

Impacted cisterna magna without syringomyelia associated with spastic paraparesis: case report

Cisterna magna impactada sem siringomielia associada com paraparesia espástica: relato de caso

José Alberto Gonçalves da Silva^I; Maria do Desterro Leiros da Costa^II; Maurus Marques de Almeida Holanda^I; Luiz Ricardo Santiago Melo^I; Antônio Fernandes Almeida de Araújo^I; André Pachelli Bezerra Viana^III

Neurosurgical Unit Hospital Unimed, João Pessoa PB, Brazil

^INeurosurgeon

^IINeurologist

^IIINeuroanesthesist

ABSTRACT

We report on a 49 year old man with impacted cisterna magna without the presence of syringohydromyelie (SM). The clinical picture was characterized by spastic paraparesis. Magnetic resonance imaging depicted a cisterna magna filled by the cerebellar tonsils. Six months after osteodural-neural decompression of the posterior fossa there was resolution of neurological symptoms and signs with the exception of hyperactive patellar and Achilles reflexes.

Key words: tight cisterna magna, impacted cisterna magna, Chiari malformation, posterior fossa decompression, spastic paraparesis.

RESUMO

Hans Chiari (1891, 1895) descreveu 4 tipos de anomalias cerebelares que, ulteriormente, foram denominadas de malformação de Chiari (MC). Iskandar et al. (1998) relataram 5 casos de hidrosiringomielia (SM) sem herniação rombencefálica, nos quais as tonsilas cerebelares preenchiam a cisterna magna. Todos os casos melhoraram após a descompressão da fossa posterior com redução do tamanho da cavidade siringomiélica. Relatamos o caso de paciente de 49 anos com cisterna magna impactada sem a presença de siringo-hidromielia (SM). O quadro clínico caracterizava-se por paraparesia espástica. A ressonância magnética evidenciou a cisterna magna preenchida pelas tonsilas cerebelares. Seis meses após a descompressão osteodural-neural da fossa posterior, houve resolução dos sintomas e sinais neurológicos, com exceção da hiperatividade dos reflexos patelares e aquileus.

Palavras-chave: cisterna magna impactada, malformação de Chiari, descompressão da fossa posterior, paraparesia espástica.

Hans Chiari^1,2 described four types of cerebellar anomalies (CM). Type I characterized by downward displacement of the cerebellar tonsils and the medial portions of the inferior cerebellar lobes which accompanied the medulla into the cervical spinal canal. Type II showed downward displacement of portions of the cerebellum (1891), and portions of the inferior vermis (1895), pons, medulla oblonga and, at least, part of lengthened fourth ventricle, which reached the disc C 4-C 5, into the enlarged cervical spinal canal. In type III, the hydrocephalic cerebellum, pons and medulla oblonga were inside a cervical meningocele (hydroencephaloceles cerebellaris cervicalis), through a spina bifida of the first three cervical vertebrae. In type IV, there was hipoplasia of the cerebellum without herniation of cerebellar structures into the spinal canal. Iskandar et al.³ (1998) related five cases of syringohidromyelia (SM), in which the cisterna magna was filled by the cerebellar tonsils. After decompression of the posterior fossa, there was clinical improvement, as well as marked reduction in the size of the syrinx in all cases. The authors admitted that this dramatic response to decompression indicates that this entity has a Chiari-like pathophysiology.

The present publication is based on the rareness of the clinical picture characterized by spastic paraparesis, hypopallesthesia of the lower limbs and sexual impotency associated with impacted cisterna magna without SM. We did not find a similar report in the consulted literature (Medline and Lilacs).

CASE

A 49-year old male with a five-year history, of progressive loss of muscular strength of the lower limbs causing severe gait difficulty, sexual impotency and paraesthesia on the feet, occasionally ascending up to the knees. Neurological exam showed spastic paraparesis, marked hyperactive patellar and Achilles reflexes, bilateral Hoffmann and Babinski signs, inexhaustible knee and ankle clonus associated with diminished pallesthesia of the lower limbs. The patient was infected by hepatitis C virus (HCV) and since 1999 has been receiving interferon therapy. Magnetic resonance imaging (MRI) depicted a cisterna magna filled by the cerebellar tonsils (Fig 1).

The osteodural-neural decompression of the posterior fossa using the Gonçalves da Silva technique, was carried out with the patient in sitting position. A large craniectomy of the posterior fossa was performed and after the dural opening in Y format the following abnormalities were observed (Fig 2): the cerebellar tonsils filled the cisterna magna without herniation into the spinal canal, the fourth ventricle and the foramen of Magendie were compressed by the cerebellar tonsils and we could identify only the left posterior inferior cerebellar artery (PICA). After dissection of the arachnoid membrane, we performed intrapial aspiration of the cerebellar tonsils and made a large opening of the fourth ventricle, and sutured the residual pial sac upwards to the dura-mater in cranial lateral position.

The postoperative MRI depicted the large created cisterna magna and also that the cerebellar tonsils did not compress the fourth ventricle and the foramen of Magendie anymore (Fig 3). Six months after posterior fossa decompression the disappearance of neurological symptoms and signs was observed, with the exception of the hyperactive patellar and Achilles reflexes and amelioration of sexual impotency.

This study was approved by the appropriate Bioethics Research Commitee.

DISCUSSION

Five cases of impacted cisterna magna without hindbrain herniation were related by Iskandar et al.³, nevertheless these cases were accompanied by SM. All cases improved after posterior fossa decompression with reduction in the size of the syrinx. The dramatic response to decompression indicates that this entity has a Chiari like pathophysiology. Kyoshima et al.⁴ related four similar cases with improvement in symptoms and a reduction in syrinx size in three patients, and a reduction in ventricle size in two. The authors named "tight cisterna magna", the impacted cisterna magna by the cerebellar tonsils, and called the description according to Iskandar et al. ³ "Chiari "0" malformation".

According to Williams⁵, the cerebellar tonsils herniation may compress brainstem structures and contribute to bulbar and a long tract dysfunction.

In a similar way the impactation of the cerebellar tonsils in the cisterna magna, without herniation into the cervical spinal canal causes disturbances in the CSF flow at the foramen magnum and can develop neurological symptomatology by compression of the brainstem. In the present case during the operation the fourth ventricle, brainstem and the foramen of Magendie were compressed by the impacted cerebellar tonsils. Probably this pathophysiology could explain the neurological symptoms and signs of this patient.

Spastic tetraparesis and paraparesis are frequently described in patients with basilar impression (BI) and/or CM and SM^6-17, in cases of cervical spondilotic myelopathy¹⁸, constrictive arachnoiditis¹⁹, among others.

SM is absent in the present case, despite an evident obstruction to the CSF flow in the posterior fossa. This finding lead us to hypothesize wether in the future a SM would develop, since obstruction to the CSF flow in the posterior fossa is considered the condition for it is development.

Regarding the HCV, some authors described neurological complications like seizures, hemiparesis, hemianopsia, and urinary retention²⁰, vasculitic neuropathy²¹, neuropsychiatric symptoms associated with chronic HCV²². Highleyman²³ suggested that HCV coinfection adversely affects neuropsychological function in patients with HIV but does not seem to contribute to peripheral sensory neuropathy. The disappearance of the clinical symptomatology after the posterior fossa decompression excludes the HCV as part of the pathophysiology of this case.

More studies should be carried out regarding the impacted cisterna magna to elucidate its pathophysiology and the correct diagnosis for the surgical treatment. The study of CSF flow is a very important method for the diagnosis of the tight cisterna magna but unfortunately this exam is not at our dispose.

Acknowledgements - This paper is dedicated with thanks to Prof. Dr. med h.c. Mario Brock

Received 8 December 2005, received in final form 7 March 2006. Accepted 24 April 2006.

Dr. José Alberto Gonçalves da Silva - Avenida Minas Gerais 1150 - 58030-092 João Pessoa PB - Brasil.

1. Chiari H.Über Veränderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch med Wschr 1891;17:1172-1175.
2. Chiari H. Über Veränderungen des Kleinhirns, des Pons und der Medulla Oblongata in Folge von congenitaler Hydrocephalie dês Grosshirns. Dtsch Akd Wiss 1895;63:71-85.
3. Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ. The resolutions of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 1998; 89:212-216.
4. Kyoshima K, Kuroyanagi T, OYA F, Kamijo Y, El-Noamany H, Kobayashi S. Syringomyelia without hindbrain herniation: tight cisterna magna. J Neurosurg (Spine 2) 2002;96:239-249.
5. Williams B. Surgery for hindbrain related syringomyelia. In Advanced and Technical Standards in Neurosurgery. Wien, New York: Springer, 20:107-164.
6. Arruda JAM. Tratamento da siringomielia associada à malformação de Chiari: análise de 30 casos. Tese. São Paulo, 1996.
7. Arruda JAM. Resultados do tratamento cirúrgico da siringomielia associada à malformação de Chiari: análise de 60 casos operados. Tese. São Paulo, 2001.
8. Arruda JAM, Costa CMC, Tella OI Jr. Results of the treatment of syringomyelia associated with Chiari malformation: analysis of 60 cases. Arq Neuropsiquiatr 2004;62:237-244.
9. Caetano de Barros M. Contribuição ao estudo da impressão basilar associada à malformação de Arnold-Chiari. Tese, Recife,1959.
10. Caetano de Barros M, Farias W, Ataíde L, Lins S. Basilar impression and Arnold-Chiari malformation: a study of 66 cases. J Neurol Neurosurg Psychiatr 1968;31: 596-605.
11. Canelas HM, Zaclis J, Tenuto RA. Contribuição ao estudo das malformações occípito-cervicais, particularmente da impressão basilar. Arq Neuropsiquiatr 1952;10:407-476.
12. Canelas HM, Zaclis J, Tenuto RA, Cruz OR. Malformações occípito-cervicais: a propósito de vinte novos casos. Arq Neuropsiquiatr 1956;14:1-26.
13. Carneiro GS Filho. Tratamento cirúrgico-circunferencial da invaginação basilar. Tese. Recife, 2001.
14. Gonçalves da Silva JA. Resultados do tratamento cirúrgico da impressão basilar e malformação de Arnold-Chiari: estudo de 72 casos. Tese. João Pessoa, 1977.
15. Gonçalves da Silva JA, Basilar impression and Arnold-Chiari malformation: surgical findings in 209 cases. Neurochirurgia 1992;35:189-195.
16. Gonçalves da Silva JA, Holanda MMA. Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients. Arq Neuropsiquiatr 2003;61:368-375.
17. Taricco MA. Tratamento cirúrgico da siringomielia asociada à malformação de Chiari do tipo I. Tese, São Paulo, 1994.
18. Pereira CAB. Arcocristectomia múltipla com foraminotomia: contribuição ao tratamento da mielopatia cervical espondilótica. Tese. São Paulo, 1991.
19. Gonçalves da Silva JA, Taricco MA, Brito JCF, Neves VD, Farias RL. Aracnoidite constrictiva causada por pantopaque resultando em siringomielia e paraparesia: relato de caso. Arq Neuropsiquiatr 2001;59: 619-622.
20. Sacconi S, Salviati L, Merelli E. Acute disseminated encephalomyelitis associated with hepatitis C virus infection. Arch Neurol 2001;58:1679-1681.
21. Heckmann JG, Kayser C, Heuss D, Manger B, Blum HE, Neundorfer. Neurological manifestations of chronic hepatitis C. J Neurol 1999; 246:486-491.
22. Dieperink E, Willenbring M, Ho SB. Neuropsychiatric symptoms associated with hepatitis C and interferon alpha: a review. Am J Psychiatry 2000;157:867-876.
23. Highleyman L. Focus on hepatitis: HCV and neuropsychological impairment. JIAPAC Monthly 2004;10:133.

Publication Dates

Publication in this collection
28 Sept 2006
Date of issue
Sept 2006

History

Received
08 Dec 2005
Reviewed
07 Mar 2006
Accepted
24 Apr 2006

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Chiari H.Über Veränderungen des Kleinhirns infolge von Hydrocephalie des Grosshirns. Dtsch med Wschr 1891;17:1172-1175.

[2] 2. Chiari H. Über Veränderungen des Kleinhirns, des Pons und der Medulla Oblongata in Folge von congenitaler Hydrocephalie dês Grosshirns. Dtsch Akd Wiss 1895;63:71-85.

[3] 3. Iskandar BJ, Hedlund GL, Grabb PA, Oakes WJ. The resolutions of syringohydromyelia without hindbrain herniation after posterior fossa decompression. J Neurosurg 1998; 89:212-216.

[4] 4. Kyoshima K, Kuroyanagi T, OYA F, Kamijo Y, El-Noamany H, Kobayashi S. Syringomyelia without hindbrain herniation: tight cisterna magna. J Neurosurg (Spine 2) 2002;96:239-249.

[5] 5. Williams B. Surgery for hindbrain related syringomyelia. In Advanced and Technical Standards in Neurosurgery. Wien, New York: Springer, 20:107-164.

[6] 6. Arruda JAM. Tratamento da siringomielia associada à malformação de Chiari: análise de 30 casos. Tese. São Paulo, 1996.

[7] 7. Arruda JAM. Resultados do tratamento cirúrgico da siringomielia associada à malformação de Chiari: análise de 60 casos operados. Tese. São Paulo, 2001.

[8] 8. Arruda JAM, Costa CMC, Tella OI Jr. Results of the treatment of syringomyelia associated with Chiari malformation: analysis of 60 cases. Arq Neuropsiquiatr 2004;62:237-244.

[9] 9. Caetano de Barros M. Contribuição ao estudo da impressão basilar associada à malformação de Arnold-Chiari. Tese, Recife,1959.

[10] 10. Caetano de Barros M, Farias W, Ataíde L, Lins S. Basilar impression and Arnold-Chiari malformation: a study of 66 cases. J Neurol Neurosurg Psychiatr 1968;31: 596-605.

[11] 11. Canelas HM, Zaclis J, Tenuto RA. Contribuição ao estudo das malformações occípito-cervicais, particularmente da impressão basilar. Arq Neuropsiquiatr 1952;10:407-476.

[12] 12. Canelas HM, Zaclis J, Tenuto RA, Cruz OR. Malformações occípito-cervicais: a propósito de vinte novos casos. Arq Neuropsiquiatr 1956;14:1-26.

[13] 13. Carneiro GS Filho. Tratamento cirúrgico-circunferencial da invaginação basilar. Tese. Recife, 2001.

[14] 14. Gonçalves da Silva JA. Resultados do tratamento cirúrgico da impressão basilar e malformação de Arnold-Chiari: estudo de 72 casos. Tese. João Pessoa, 1977.

[15] 15. Gonçalves da Silva JA, Basilar impression and Arnold-Chiari malformation: surgical findings in 209 cases. Neurochirurgia 1992;35:189-195.

[16] 16. Gonçalves da Silva JA, Holanda MMA. Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients. Arq Neuropsiquiatr 2003;61:368-375.

[17] 17. Taricco MA. Tratamento cirúrgico da siringomielia asociada à malformação de Chiari do tipo I. Tese, São Paulo, 1994.

[18] 18. Pereira CAB. Arcocristectomia múltipla com foraminotomia: contribuição ao tratamento da mielopatia cervical espondilótica. Tese. São Paulo, 1991.

[19] 19. Gonçalves da Silva JA, Taricco MA, Brito JCF, Neves VD, Farias RL. Aracnoidite constrictiva causada por pantopaque resultando em siringomielia e paraparesia: relato de caso. Arq Neuropsiquiatr 2001;59: 619-622.

[20] 20. Sacconi S, Salviati L, Merelli E. Acute disseminated encephalomyelitis associated with hepatitis C virus infection. Arch Neurol 2001;58:1679-1681.

[21] 21. Heckmann JG, Kayser C, Heuss D, Manger B, Blum HE, Neundorfer. Neurological manifestations of chronic hepatitis C. J Neurol 1999; 246:486-491.

[22] 22. Dieperink E, Willenbring M, Ho SB. Neuropsychiatric symptoms associated with hepatitis C and interferon alpha: a review. Am J Psychiatry 2000;157:867-876.

[23] 23. Highleyman L. Focus on hepatitis: HCV and neuropsychological impairment. JIAPAC Monthly 2004;10:133.