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Cognitive deterioration, Cortical blindness , Fine movement prob

Neurological Medical Report
Name Dr. Faten Nofal
Age 61 yrs Gender Female
Height 170 cm Weight 112Kg
Address Alexandria – Egypt
Profession Prof. of industrial Hygiene & occupational health

Complaints
- Fine Motor Movement Problems - Coordination of arms, hands, fingers , since 2 years
- Diminution of Vision(Field of vision is very limited ), since 1.5 year
- Cognitive functions deterioration, since 1.5 year
- Remarkable tremors, weakness & stiffness in Left hand , since 2 years
to a lesser extent in the Right hand , since 6 months
Her left palm is folded
- Inability to drive , since 2 years
- Loss of hand skills , since 1.5 years
- Inability to dress , since 1.5 years
- Coordination Difficulties , since 1.5 years
- Fatigue , since 1.5 years
- Facial recognition difficulties , since 1.5 year
- Concentration Problems , since 1.5 years
- Word Finding Difficulties, since 1.5 years
- Difficulty Retaining Information , since 1.5 years
- Difficulties to handle mathematical calculations , since 1.5 years
- Depression , since 1.5years
- Frequency Urination , since 3 months
- Reasoning, memory, speech & judgment are not affected.

Course of illness has showed rapidly progression during the last year specially in her vision

Illness history & progression
The condition started in 2008 by:
Hypothyroid manifestation
Investigations revealed hashimoto disease
Treated e’ thyroid extract
Thyroid profile within normal with treatment.
& Developed psychomotor retardation
Diagnosed by consultant neurologist as early parkinsonian features
Received sinemet with no apparent improvement
Over the last 2 years
- certain high cortical functions and cognitive abilities became gradually affected including visuospatial disorientation, recent amnesia , motoric apraxia , constructional apraxia , visual field defect as evidenced by perimetry visual field defect deteriorated significantly over the last year with impact on daily life activities.
- She has no hallucinations or psychotic features , no confusional state.
- Her condition is cognitively stable scoring around 24-25 on MMSE.
- She has been put on Exelon since June 2010, 10 mg patch form and Ebixa 20mg/d. Pramipexol added with a close of 3mg / d.





Examination
- The patient is fully conscious (15 GCS) , cooperative
- reactive facial expressions
- Cranial domain intact (apart from visual field defect )
Attached Neuro ophthalmology report
- Patient shows hypertonia, in the whole range of movement highlighting rigidity and mild dysphagia, a clumsiness in movements and psychomotor retardation,
- In the lower limbs no motoric deficit, coordination intact
- no signs of cerebellar dysfunction,
- Deep reflexes increased bilaterally no pathological reflexes could be ellicited .
- Brain stem reflexes intact
- Mild Several apraxia ( constructional , oculomotor, verbal , limb-Kinetic )
- Non cortical sensations intact
- Cortical sensation (Astereognosis Agraphesthesia )

Medical History
The patient is diabetic with good control on oral hypoglycemic(AMARYL M )
Controlled Hypertension ( X-Forge 5mg)
Normal range lipid profile on Anti-Hyper cholestrolemic drug(Crestor 10mg)
Hashimoto Thyroditis controlled on 100mg euthyrox
Though anti- thyroid Antibody titre is high, no signs suggesting hashimoto encephalopathy
Current treatments
- Exelone patch 10mg
- Cipralex 10 mg /day
- Lucidril 500mg 2/day
- Uripan 1mg 2/day
- Ramixol 1mg ½ * 2 /day
- Aggrex 75mg 2/day
- Ebixa 10mg . 2/day

Investigations:
- MRI - Brain
mild peripheral cerebral involutional changes, partial empty sella
- Brain . CT scan & CT cerebral angiography
Normal
- Pituitary profile
Normal

test Result Reference range for age
TSH 8.140 Uiu/ml 0.4-8.9
Anti thyroid peroxidase Ab. 383 IU/ml up to 35
Anti-nuclear Ab 40 up to 160
Anti(ds)DNA 15.8 IU/ml <30
CRP 9.1 mg/l Up to 6
ESR 1st hr
2nd hr 34 mm
81 Less than 20
HCV Abs -ve
HBs Ag -ve

- Renal, hepatic, blood picture show no abnormalities
- Provisionally -----------→ Dementia induced by hypothyroidism

Family History
The patient’s mother had Alzheimer disease onset at 78 yrs old

Neuro Ophthalmology report
Vision R: 6/60 L: 6/60
Pupil : rounded, regular reactive ( directly & consensually), less on left side
EOM: acting in all directions ( mild interruption of pursuit movements)
• No visual agnosia ,
• can identify objects
• Can identify & name colors
• left hemineglect
• mild alexia with mild agraphia
Field : now constricted field (quadrantic field)
Automated field :
27/1/2010 Left homonymous hemianopia with sparing of the macula in the right eye ( suggestive of RT accipital cortex affection)
25/6/2010 ↑ density of LT homonymous hemianopia & starting of right homonymous hemianopia ( suggestive of increase affection of RT visual occipital cortex & stent of affection of LT occipital cortex)
Flurescein angiography 27/1/2010:
Bilateral normal fluorogram.
MRI: 2/11/2010 Suggestive of RT accipital cortical atrophy with milder LT
Picture suggestive of : Bilateral cortical blindness affection of both occipital visual cortex non in the right side
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replied November 13th, 2012
Did you ever get an answer? If not, perhaps it is worth further investigation of autoimmune ("Hashimoto's") encephalopathy / SREAT as at least part of the profile?

Much of what you describe is experienced by such patients, even without hallucinations, etc. You can hear our stories in the Hashimoto's encephalopathy group on facebook, or within the Encephalitis Global pages at inspire.com, among many other places.

Hoping you've found answers long before now and wishing you well
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replied November 13th, 2012
Note: seemingly 'age appropriate' cognition and speech may be still exist in autoimmune encephalopathy patients who started with very high cognitive performance.

Those starting with very high performance can fall through more than half the bell curve of intelligence before they will appear to be noticeably 'below normal', and impairments will depend on the part of the brain affected. It can be helpful to consider relative decline.

Also, many countries look for anti alpha enolase antibodies to investigate for "Hashimoto's" encephalopathy. Perhaps you can investigate whether this test is available in Egypt, or can be coordinated through a foreign institution?
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