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Abstract
Cerebellar mutism (CM) is a rare neurological condition characterized by lack of speech
due to cerebellar lesions. CM is often reported in children. We describe a rare case
of CM after spontaneous cerebellar hemorrhage. The patient showed mutism, irritability,
decreased spontaneous movements and oropharyngeal apraxia. Diffusion tensor imaging
revealed significant volume reduction of medial frontal projection fibers from the
corpus callosum. In Tracts Constrained by UnderLying Anatomy (TRACULA) analysis, forceps
major and minor and bilateral cingulum-angular bundles were not visualized. Cerebello-frontal
pathway reconstructed from the FMRIB Software Library showed continuity of fibers,
with decreased number of fibers on qualitative analysis. These results suggest that
cerebello-frontal disconnection may be a neuroanatomical mechanism of CM. Damage of
brain network between occipital lobe, cingulate and cerebellum caused by hemorrhage
may also have role in the mechanism of CM in our case.
We present data on the intellectual, language and executive functions of 26 children who had undergone surgery for the removal of cerebellar hemisphere or vermal tumours. The children with right cerebellar tumours presented with disturbances of auditory sequential memory and language processing, whereas those with left cerebellar tumours showed deficits on tests of spatial and visual sequential memory. The vermal lesions led to two profiles: (i) post-surgical mutism, which evolved into speech disorders or language disturbances similar to agrammatism; and (ii) behavioural disturbances ranging from irritability to behaviours reminiscent of autism. These data are consistent with the recently acknowledged role of the cerebellum as a modulator of mental and social functions, and suggest that this role is operative early in childhood.
The complication of the posterior fossa surgery is seldom described in the literature. The purposes of this retrospective study are to draw attention to the potential complications associated with posterior fossa surgery and to critically review the predisposing factors that might influence the complication rate. We undertook a 10-year (1992-2002) retrospective study of all posterior fossa surgery performed at LSUHSC. A total of 500 patients were obtained from the operation database, and they were categorized into 5 groups based on the surgical approaches: (1) cerebellopontine angle lesion, (2) microvascular decompression for facial pain and spasm, (3) cerebellar lesions, (4) Chiari I decompression, and (5) petroclival lesions. Data collected for analysis included patient demographics, pathological characteristics of the lesions, and the postoperative complications that occurred as unexpected and undesirable events that prolonged hospital stay and may require surgical/medical intervention. Of the 500 patients reviewed, 220 (44%) patients had tumor resections at the cerebellopontine angle; 110 (22%) patients had microvascular decompression for trigeminal neuralgia and hemifacial spasm; 86 (17.2%) patients had cerebellar lesions; 60 (12%) patients had Chiari I decompression; and 24 (4.8%) patients required transpetrosal approaches for petroclival lesions. The overall complication rate in our study was 31.8%, affecting 159 patients. Cerebrospinal fluid leaks were the most frequently encountered, presenting in 65 (13%) patients followed by meningitis in 46 (9.2%) patients, wound infection in 35 (7%) patients, and CN palsies in 24 (4.8%) patients. Other complications that were observed to develop almost exclusively in patients undergoing cerebellar parenchymal tumor resection included cerebellar edema in 25 (5%) patients, hydrocephalus in 23 (4.6%) patients, cerebellar hematoma in 15 (3%) patients, and cerebellar mutism in 6 (1.2%) patients. The overall mortality rate related to surgery was 2.6% occurring in 13 patients. Posterior fossa surgery involves greater morbidity and mortality and has a wider variety of complications than surgery in the supratentorial compartment. These complications may be avoided by careful perioperative planning, strict adherence to aseptic technique, meticulous microsurgical dissection, proper wound closure, and the judicious use of prophylactic agent. A thorough understanding of the patient's history, neurological findings, imaging studies, operative anatomy, as well as all potential adverse events associated with the procedure is also essential to minimize complications.
The Posterior Fossa Syndrome (PFS) may occur in children after resection of cerebellar tumours. The most common feature is mutism, but also oropharyngeal dyspraxia, emotional lability and neuropsychiatric symptoms occur. We analysed the spectrum of behavioural abnormalities, speech and language characteristics during PFS.
Publisher:
Korean Academy of Rehabilitation Medicine
ISSN
(Print):
2234-0645
ISSN
(Electronic):
2234-0653
Publication date
(Print):
December
2017
Publication date
(Electronic):
28
December
2017
Volume: 41
Issue: 6
Pages: 1076-1081
Affiliations
[1
]Department of Physical Medicine and Rehabilitation, Korea University Anam Hospital,
Korea University College of Medicine, Seoul, Korea.
[2
]Department of Biomedical Sciences, Korea University Anam Hospital, Korea University
College of Medicine, Seoul, Korea.
[3
]Department of Rehabilitation Medicine, Bundang Jesaeng General Hospital, Seongnam,
Korea.
[4
]Brain Convergence Research Center, Korea University Anam Hospital, Korea University
College of Medicine, Seoul, Korea.
Author notes
Corresponding author: Sung-Bom Pyun. Department of Physical Medicine and Rehabilitation,
Korea University Anam Hospital, Korea University College of Medicine, 73 Inchon-ro,
Seongbuk-gu, Seoul 02841, Korea. Tel: +82-2-920-6483, Fax: +82-2-929-9951,
rmpyun@
123456korea.ac.kr
This is an open-access article distributed under the terms of the Creative Commons
Attribution Non-Commercial License (
http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted noncommercial use, distribution, and reproduction in
any medium, provided the original work is properly cited.
History
Date
received
: 20
February
2017
Date
accepted
: 22
May
2017
Funding
Funded by: National Research Foundation of Korea, CrossRef http://dx.doi.org/10.13039/501100003725;
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