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.

Chiari Type I Malformation Presenting as Hemifacial Spasm: Case Report

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

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

scholarly journals 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.

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 Chiari Type I malformation yielded to the diagnosis of Crouzon syndrome

2014 ◽  
Vol 5 (01) ◽  
pp. 81-83 ◽  
Author(s):  
Aydin Canpolat ◽  
Mehmet Osman Akçakaya ◽  
Emre Altunrende ◽  
Harun Mehmet Özlü ◽  
Hakan Duman ◽  
...  
Keyword(s):  
Posterior Fossa ◽  
Clinical Symptoms ◽  
Careful Examination ◽  
Posterior Fossa Decompression ◽  
Type I ◽  
Crouzon Syndrome ◽  
Late Adolescent ◽  
Chiari Malformation Type I ◽  
Chiari Type I Malformation ◽  
Syndromic Craniosynostosis

ABSTRACTChiari malformation Type I (CM-I) related to syndromic craniosynostosis in pediatric patients has been well-studied. The surgical management consists of cranial vault remodeling with or without posterior fossa decompression. There were also cases, in whom CM-I was diagnosed prior to the craniosynostosis in early childhood. We present a 16-year-old boy who admitted with symptoms related to CM-I. With careful examination and further genetic investigations, a diagnosis of Crouzon syndrome was made, of which the patient and his family was unaware before. The patient underwent surgery for posterior fossa decompression and followed-up for Crouzon’s syndrome. To our knowledge, this is the only case report indicating a late adolescent diagnosis of Crouzon syndrome through clinical symptoms of an associated CM-I.

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