2013年4月4日 星期四

SUMMARY REPORT on MRI-T2 Imaging of Chen Shui Bian's brain on Jan. 16, 2013


SUMMARY REPORT
on MRI-T2 Imaging of Chen Shui Bian's brain on Jan. 16, 2013
by
Samuel M. Chou, M.D., Ph.D., Prof.

Clinical history
As provided by Prof. S-S. Chen (陳順勝教授) who was requested as a neurologist to see Chen Shui Bian (CSB) in Sept. of 2012, for the first time, during the past 4 years' imprisonment. Therefore the onset of his slowly developing neurological disease especially at its onset is difficult to assess. 
           This 62 year old man developed progressively worsened neurological symptoms and signs since about 6 months earlier. The neurological deficits have steadily and step-wisely worsened and now displaying tremors in the right hand, and stuttering for brief wording. For this reason, it was decided to take MRI every 3 months apart.

Neurological finding since Sept. 2012
a)      Speech impediments: stuttering speech, nominal aphasia (disturbance in formulation and comprehension of language), partial expressive aphasia (loss of ability to produce language), however echolalia was possible.
b)     Emotional disturbance: extensive depression, mask-like expressionless face (a common description of Parkinson’s facies)
c)      Cerebellar signs: F-N-F (finger-nose-finger, implying upper extremity superior cerebellar defect, right side) + K-H-T (knee-heel-toe, implying lower limb cerebellar defect bilaterally), Romberg's sign (implying cerebellar or posterior spinal column deficit).
d)     Memory disturbance; poor recent memory, especially for dealing with the daily life.
e)      Cognitive disorders or early signs of dementia.
f)       Abnormal reflexes 

Motor systems: unsteady or ataxic gait, Extrapyramidal signs: cogwheel rigidity of Rt arm, Tremor: resting and action, Rt hand, with weakness, Bradykinesia, Mask-like expressionless face resembling Parkinsonism.

Sensory system: double stimulation test showed neglect of right side, agraphesthesia.

More recently, CSB has started to display urinary incontinence once; cognitive disorders and wide-based gait, they began to slowly progressed and developed as so-called Adam's triad of Normal Pressure Hydrocephalus (NPH): (1) urinary Incontinence; (2) cognitive disorders and or early dementia; and (3) wide-based gait disturbance.

Cognitive disorders or Dementia: in the form of apathy, dullness and forgetfulness, frontal-lobish, or subcortical in type which resemble Parkinson's and can be distinguished from the cortical, or Alzheimer type. Also with its hydrocephalic changes with the MRI findings.

Urinary urgency: in the form of spastic hypereflexic to lack of concern so called frontal- lobe incontinence.

Gait disturbance: occur most frequently from the early stages characterizes by broad-base gait may change to short shuffling gait resembling that in Parkinson's disease.

MRI Findings (MRI-T2 and Voxel MRI) 
Multiple cystic lesions can be identified. The largest are located at Sylvian fissures, infraorbital, infratemporal fossa, interhemispherical sulcus. Some of them appear ruptured forming hygroma. The gyri are flattened and shortened over the convexity, or atrophic with fibro-gliosis over the under surfaces of the brain. Topographically the largest cystic lesions appear associated with Limbic system. 
           The clinical findings and diagnosis of NPH may lead to brain imaging, either CT or MRI, followed by lumbar puncture for measurement of the CSF pressure, the evidence for trauma or tumor. 

Pathogenesis
The pathogenesis for the hygroma formation is not fully understood although the strategy for the treatment for the ventriculoperitoneal shunt to drain the excess CSF is the same. However, the prognosis depends heavily on clinical measurements of the clinical "triad", the cortical sulci, gyri and periventricular lucency.

Diagnosis
Normal Pressure Hydrocephalus (NPH) or NPH-like syndrome secondary to multiple large subdural arachnoidal cysts (some probably ruptured), with resultant pan-subdural hygromas (the largest in the Sylvian fissures, bilateral and symmetrical) compressing the major components of the Limbic system (including Ammon’s horns, amygdaloid nuclei, hippocampal, orbitofrontal, anterior cingulate and insular cortices and hypothalamus). Comparing the September 2012 to December 2012 brain MRI, the findings have worsened, correlating with the patient’s worsening neurologic symptoms some of which did not exist before September 2012.

Concerns over human rights for CSB
The conditions of continuous light exposure and sleep deprivation which CSB has been subjected to for a number of years has caused severe, probably irreversible damage to his brain as evidenced in the recent detailed medical evaluation. I concur with Nicole Bieske, spokeswoman for Amnesty International Australia, whose statement regarding sleep deprivation is as follows: "We are fundamentally opposed to cruel, inhumane and degrading treatment of any person". Sleep deprivation, Ms. Bieske said, was considered cruel but if used for prolonged periods of time, it was torture. "At the very least it is cruel, inhumane and degrading. If used for prolonged periods of time it is torture". It has been confirmed that CSB was subjected to continuous light exposure coupled with sleep deprivation for a prolonged period of time in addition to internationally unacceptable physical condition of the cell in which he was incarcerated.
           In a real sense, based on international standards, CSB was subjected to torture for a prolonged period of time preceding his transfer to the current hospital in which he now is receiving care.

Recommendations
Trauma resulting from continuous light exposure and sleep deprivation cannot be dismissed and in fact are the causes of the neurological abnormalities. Detrimental effects such as those experience by CSB have been widely substantiated and confirmed in numerous clinical and animal studies in the last few decades and must be considered direct consequences of confirmed prison conditions imposed on the former president.
           Based on established guidelines set by the Mayo Clinic, American Autonomic Society, the American Academy of Neurology, and other professional organizations, the most effective treatment for CSB is IMMEDIATE HOME CARE with supervising medical professionals and management by rehabilitation specialists in a home environment surrounded by family and loved ones. This home environment will be an important factor in halting further deterioration and with intensive therapy help begin the slow and long process towards alleviating the symptoms.
           The critical nature of his illness cannot be overemphasized and must be addressed immediately.

References
1) Adams RD, Fisher CM, Haskim S. et al. Symptomatic occult hydrocephalus with "normal" cerebrospinal fluid pressure. a treatable syndrome. N Eng J Med 1965;273: 117-126.
2) Chou S, Gutmann L. Deteriorating parkinsonism and subdural hematomas. Neurology 2001; 57:1295
3) Tamburrini G, Caldarelli M, Massimi L, Santini P. Subdural hygroma: an unwanted result of Sylvian arachnoid cyst marsupialization. Childs Nerv Syst. 2003;19(3):159-165
4) Albuquerque FC, Giannotta SL. Arachnoid cyst rupture producing subdural and intracranial hypertension: case report. Neurosurgery 1997:41(4):951-956
5) Copinschi G. Metabolic and endocrine effects of sleep deprivation. Essent Psychopharmacol 2005; 6(6):341-347
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7) Everson CA, Szabo A. Recurrent restriction of sleep and inadequate recuperation induce both adaptive and pathological changes. Am J Physiol Regul Integr Comp Physiol 2009; 297(5):R1430-40.



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