related to movement.9 It is imperative to keep under consideration that although much convincing evidence supporting a modulatory role of cerebellum in cognition and affect has amassed, a few clinical observations fail to reiterate this concept. Most notable of such studies has been the work of Richter et al on patients with chronic cerebellar infarction.10 They demonstrated an absence of cognitive deficits in 21 adult patients with chronic cerebellar infarctions both in territory of PICA and SCA based on an objective bed-side mental examination.10 However, this simply may imply that such deficits with cerebellar stroke are transient and likely to improve over a course of weeks to months.
the repertoire of clinical evidence favouring the notion.
Though we did not subject our patient to formal neuropsychological testing, clinically prominent symptoms/signs and a clear temporal relation to the stroke suggest this being post stroke cerebellar cognitive affective syndrome. We, therefore, advocate a potential role of cerebellum in regulation of cognition and behaviour in humans, based upon our clinical observation of transient cognitive and affective impairments in a 30 year old female after isolated cerebellar infarction. To the best of our knowledge this is the first reported case from Pakistan and the neighboring countries. This case substantially adds to
References 1.
Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain. 1998;121:561-79.
2.
Levisohn L, Cronin-Golomb A, Schmahmann JD. Neuropsychological consequences of cerebellar tumor resection in children: cerebellar cognitive affective syndrome in a pediatric population. Brain. 2000;123:1041-50
3.
Riva D, Giorgi C. The cerebellum contributes to higher functions during development. Evidence from a series of children surgically treated for posterior fossa tumors. Brain 2000;123:1051-61
4.
Paulus KS, Magnano I, Conti M, Galistu P, D'Onofrio M, Satta W, et al. Pure post-stroke cerebellar cognitive affective syndrome: a case report. Neurol Sci. 2004;25:220-24.
5.
Exner C, Weniger G, Irle E. Cerebellar lesions in the PICA but not SCA territory impair cognition. Neurology. 2004;63:2132-35.
6.
Hokkanen LS, Kauranen V, Roine RO, Salonen O, Kotila M. Subtle cognitive deficits after cerebellar infarcts. Eur J Neurol. 2006;13:161-70.
7.
Botez MI, Gravel J, Attig E, Vézina JL. Reversible chronic cerebellar ataxia after phenytoin intoxication: Possible role of cerebellum in cognitive thought. Neurology. 1985;35:1152-57.
8.
Abel CG, Stein G, Pereyra S, Nano G, Arakaki T, Garreto N, et al. Neuropsychological study of 12 patients with pure degenerative cerebellar disease. Rev Neurol. 2005;40:465-72.
9.
Bugalho P, Correa B, Viana-Baptista M. Role of the cerebellum in cognitive and behavioural control: scientific basis and investigation models. Acta Med Port. 2006;19:257-67.
10.
Richter S, Aslan B, Gerwig M, Wilhelm H, Kramer S, Todica O, et al. Patients with chronic focal cerebellar lesions show no cognitive abnormalities in a bedside test. Neurocase. 2007;13:25-36.
Letter to the Editor Sudden unexpected death in a young adult with Chiari I malformation Madam, Chiari I malformation is a dysraphic congenital disorder, frequently associated with other malformations of the same kind, including syringomyelia, Klippel-Feil syndrome and tethered cord.1,2 Chiari I malformation can be a cause of sudden unexpected death in a group of patients.3 We report an extremely rare case of Chiari I malformation who developed posterior fossa pneumocephalus after surgery resulting in fatal outcome. Thirty year gentleman presented with loss of sensation over right half of body since 1 month, headache and giddiness of same duration. There was no history of loss of consciousness or trauma. General and systemic examination was normal. Higher mental functions were normal. Neurological examination revealed paralysis of the 9th, 10th and 12th cranial nerves; distal paresis, muscular atrophy and areflexia of the upper limbs; abolition of left abdominal cutaneous reflexes and hyper-reflexia in the lower limbs. Sensory examination revealed loss of position and vibration sense in lower limbs and loss of pain and temperature over upper chest. Laboratory tests and the chest radiograph were normal. X-ray cervical spine was normal. Magnetic resonance imaging (MRI) (figure-1A) showed a Vol. 58, No. 7, July 2008
large tonsillar herniation (Chiari I malformation), with a syringomyelic cavity extending from cervical cord to the T 8 vertebra. He underwent suboccipital craniectomy (foramen magnum decompression), C1 arch excision, C2 laminectomy and duroplasty. He was apparently alright in immediate post-operative period. On third day he developed slowly progressive headache followed by deterioration in sensorium. There was no fever or meningeal signs. On
Figure-1. (A) Sagittal cranio-cervical magnetic resonance imaging (MRI) section T1W image showing herniated cerebellar tonsils (up to C2 level) with an extensive syringomyelia extended from the lower medulla to the level of the T8 vertebra (not seen in the figure) and (B). Postoperative CT scan showing pneumocephalus in the posterior fossa
417
repeat CT scan there was no evidence of hydrocephalus but it showed pneumocephalus in the posterior fossa with compression of surrounding structures (Figure-1B). He suddenly deteriorated and developed cardio-respiratory arrest with fatal outcome. Tension pneumocephalus after posterior fossa surgery though uncommon but is a well known complication.4,5 The literature shows that air may enter or diffuse through multiple eroded or thinned bone areas during surgery.3 It may manifest as deterioration of consciousness with or without localizing signs, severe restlessness, generalized convulsions or focal neurological deficits.4 Posterior fossa pneumocephalus can be a cause of sudden cardiac arrest in immediate post-operative period.4 Lethal brainstem compression may accompany even relatively minor trauma associated with chronic cerebellar tonsillar herniation in patients with Chiari I malformation.3,4 In the present case pneumocephalus aggravated already compromised brain stem dysfunction and resulted in fatal outcome.
Amit Agrawal Department of Surgery, Datta Meghe Institute of Medical Sciences, Sawangi (Meghe), Wardha (India).
References 1.
Paul KS, Lye RH, Strang FA, Dutton AJ. Arnold-Chiari malformation: review of 71 cases. J Neurosurg 1983; 58: 183-187.
2.
Dyste GN, Menezes AH, Van Gilder JC. Symptomatic Chiari malformations: an analysis of presentation, management and long-term outcome. J Neurosurg 1989; 71:159-168.
3.
Rickert CH, Grabellus F, Varchmin-Schultheiss K, Stoss H, Paulus W. Sudden unexpected death in young adults with chronic hydrocephalus. Int J Legal Med 2001; 114: 331-7.
4.
Dash HH, Bithal PK, Muley, Vishnoi N, Saini SS, Tandon PN. Tension pneumocephalus following posterior fossa surgery in sitting position. J anaesth Clin Pharmacol 1990:6;207-12
5.
Hernandez-Palazon J, Martinez-Lage JF, de la Rosa-Carrillo VN, Tortosa JA, Lopez F, Poza M. Anesthetic technique and development of pneumocephalus after posterior fossa surgery in the sitting position. Neurocirugia (Astur) 2003; 14: 216-21.
Retraction of Publication Journal of Pakistan Medical Association J Pak Med Assoc. 2001; 51: 243 - 7
Retraction of original article in whole Title: Profile of Female Breast Lesions in Saudi Arabia Authors: Ibrahim Mansoor Institution: King Abdul Aziz University Hospital, Jeddah, Saudi Arabia Published in JPMA July 2001
Announcement The 6th International Symposium of Urology, Nephrology and Transplantation, organized by the Sindh Institute of Urology, and Transplantation (SIUT), will be held from 23rd to 26th October, 2008 in Karachi. Besides plenary Sessions, Symposia and free papers, workshops will be held on 'Robotics in Urology', Renal histopathology, clinical Ethics and Molecular Techniques in Laboratory Diagnosis. Last date for Abstract submission: 31st July, 2008. Last date for registration: 31st August, 2008. For detail information visit the website: www.siut.org Email:
[email protected];
[email protected]
418
J Pak Med Assoc
