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From: TSS ()
Subject: Of Illusions, Hallucinations and Creutzfeldt-Jakob Disease (Heidenhain's Variant)
Date: March 3, 2005 at 1:43 pm PST

-------- Original Message --------
Subject: Of Illusions, Hallucinations and Creutzfeldt-Jakob Disease (Heidenhain's Variant)
Date: Thu, 03 Mar 2005 15:46:32 -0600
From: "Terry S. Singeltary Sr."
To: Bovine Spongiform Encephalopathy


J Neuropsychiatry Clin Neurosci 17:124-126, February 2005
© 2005 American Psychiatric Press, Inc.

------------------------------------------------------------------------


Letter


Of Illusions, Hallucinations and Creutzfeldt-Jakob Disease
(Heidenhain's Variant)

Harpal K. Brar, M.D., Vaishnavi Vaddigiri, M.D. and Angela Scicutella,
M.D., Ph.D., Long Island Jewish Medical Center, Department of Geriatric
Psychiatry, 75-59 263rd Street, Glen Oaks, NY 11004

SIR: Sporadic CreutzfeldtJakob disease (CJD), a rare progressive
neurodegenerative disorder whose classic features include dementia,
ataxia, and myoclonus can initially present with nonspecific psychiatric
symptomatology such as fatigue, anxiety or a change in personality in
about one third of cases, sometimes leading to erroneous diagnoses of
depression or psychosis, as has been described in single patient
reports. 1
In
contrast to the above psychiatric symptoms, the presence of visual
perceptual abnormalities such as illusions and hallucinations observed
at the onset of a patient's clinical course is usually more likely to be
viewed as indicative of a medical, ophthalmologic or neurologic illness
rather than of psychiatric etiology.2
We
present a case of an elderly female with an initial presentation notable
for the acute manifestation of visual illusions followed by visual
hallucinations, but whose complicated medical course led to a variety of
psychiatric diagnoses prior to her ultimate diagnosis of CJD.

Case Report
The patient is a 75-year-old female with a past medical history
significant for coronary artery bypass surgery, hypertension, arthritis,
noninsulin dependent diabetes and hypercholesterolemia. Personal and
family history of psychiatric illness, substance abuse, dementia or
cognitive decline was denied. She was functioning well as a housewife
until a few weeks prior to admission when she became more anxious,
presumably over a home reorganization project. Two days prior to
admission, she began to experience visual distortions which were
described as changes in the furniture : the china closet was tilted, the
table had shrunk and the chair legs had been cut off. As the patient was
sleeping poorly and becoming more agitated, the family brought her to a
local emergency room where her vital signs were stable, and her physical
and neurological examinations were unremarkable. On mental status
examination she was alert, with fluent coherent speech and no evidence
of fluctuation in consciousness. She denied psychotic symptoms, and
cognitively her exam was significant only for not knowing the exact date
and missing one item out of three in recall. A complete blood count,
electrolyte panel, chest x-ray, electrocardiogram and brain computed
tomography (CT) were all unremarkable. Her urinalysis revealed white
blood cells (1025) and moderate leucocyte esterase, but this was not
treated pending culture results. The patient was admitted to the
medicine service with a diagnosis of acute change in mental status.
Further evaluation the next day included an ophthalmologic consult who
addressed the patient's blurred vision and diagnosed blepharitis. An
electroencephalogram (EEG) revealed mild to moderate generalized
slowing. Subsequently, the patient had complaints of foot pain and was
found to have a right fifth metatarsal fracture of unclear etiology,
which was casted. She received oxycodone with acetaminophen for pain
relief and then began experiencing visual hallucinations that included a
substance oozing from the ceiling and bugs crawling in the room. The
psychotic symptoms were attributed to the pain medication, so it was
discontinued and haloperidol was prescribed. Magnetic resonance imaging
(MRI) of the brain done on the fourth day of admission revealed only
mild atrophy and angiopathic disease. Over the next 3 days, the
patient's condition worsened as the persistence of visual
hallucinations, including bugs and butterflies, was now accompanied by
disorientation to time and place.

Quetiapine was then substituted for haloperidol and a second psychiatric
evaluation was requested. It was concluded that the patient was in
delirium of unclear etiology, but it was recommended that reversible
causes of dementia be excluded by checking lyme titers, B12, folate, and
rapid plasma reagent, all of which were found to be within normal
limits. With no clinical improvement, the urinalysis was repeated and
revealed the presence of infection which was treated with levofloxacin.
Additionally, the neurology service prescribed donepezil for a
presumptive diagnosis of dementia with superimposed delirium. On the
thirteenth day of hospitalization she was transferred to an inpatient
psychiatric facility at another institution for further treatment of
psychosis.

At the psychiatric facility, the patient was admitted with a diagnosis
of Alzheimer's disease and was treated with donepezil and low dose
quetiapine as needed, but her status did not improve. Lumbar puncture
was suggested but the family refused. Six days later, the patient became
mute and catatonic, presumably secondary to neuroleptics which were then
discontinued, and a course of low dose lorazepam was prescribed for a
period of 5 days. As this was minimally effective, electroconvulsive
therapy (ECT) was then proposed, but after one treatment she ceased to
eat and drink, necessitating transfer to the medical unit to address
dehydration.

On the medical ward, the patient was diagnosed with a fungal urinary
tract infection which was treated with fluconazole, and for nutrition, a
nasogastric tube was placed. The geriatric neuropsychiatry consultant
called to provide continuity of care, discontinued all psychotropic
medications and reviewed the history with the family again, confirming
that there was no evidence to support that the patient had been
experiencing progressive cognitive decline prior to admission.
Therefore, further evaluation was recommended and included a repeat head
CT, which showed atrophy; an EEG to rule out nonconvulsive status, which
demonstrated diffuse slowing consistent with encephalopathy; and a
repeat MRI to evaluate for a structural lesion to explain her akinetic
mutism, which showed no evidence of acute pathology. Two weeks later
when the patient had demonstrated no clinical improvement, it was
proposed that she be transferred back to the psychiatric unit to
continue ECT for catatonia. Prior to the transfer, the geriatric
neuropsychiatry consult requested a lumbar puncture to rule out causes
of a rapidly progressive dementia. The cerebrospinal fluid (CSF)
demonstrated a nonspecific increase in glucose and protein without
malignant cells. The 14-3-3 protein was sent for assessment, and in the
interim 12 days while awaiting these results, the hospital course was
significant for an unsuccessful trial of methylphenidate for akinetic
mutism, the development of myoclonus, and placement of a percutaneous
endoscopic gastrostomy tube (PEG). When the CSF immunoassay result was
remarkable for an elevated level of the 14-3-3 marker protein, a repeat
EEG was performed, which demonstrated triphasic waves at one
cycle/second consistent with CJD. Ten days later and 2 months after the
patient's initial admission to the first acute care hospital, she was
transferred to a long term facility.

Comment
One of the clinicopathologic subgroups of CJD known as the Heidenhain
variant is characterized by the predominance of visual symptoms that
persist throughout the course of the disease and can include disturbed
perceptions of objects or colors, optical hallucinations, visual field
defects, visual agnosia, or cortical blindness.3

Clinically, our patient's course was highlighted throughout by the
presence of visual symptoms which initially were illusions, specifically
distortions in the shape (metamorphopsia), size (micropsia), and axis
(tilt) of her furniture, then blurred vision, and subsequently complex
hallucinations of insects which is consistent with this Heidenhain
classification. Performance of diagnostic studies such as single photon
emission computed tomography (SPECT) or positron emission tomography
(PET), which may have supported our clinical diagnosis had occipital
lobe hypoperfusion or hypometabolism been demonstrated,4
was
omitted to respect the family's wishes. This case serves as a reminder
that in the absence of alterations in consciousness, cognitive decline,
known impairments in vision, or culprit medications, visual perceptual
distortions (illusions and hallucinations) can be due to lesions
anywhere along the visual pathway from the retina to the cortex5
and
may herald an underlying medical, ophthalmologic, or, in this case,
neurologic etiology, rather than a psychiatric one.

REFERENCES

1. Moellentine CK, Rummans TA: The varied neuropsychiatric
presentations of Creutzfeldt-Jakob disease. Psychosomatics 1999;
40:260263[Free Full Text]

2. Norton JW, Corbett JJ: Visual perceptual abnormalities:
Hallucinations and illusions. Semin Neurol 2000;
20:111121[CrossRef]
[Medline]

3. Mathews D, Unwin DH: Quantitative cerebral blood flow imaging in a
patient with the Heidenhain variant of Creutzfeldt-Jakob disease.
Clin Nucl Med 2001; 26:770773[CrossRef]
[Medline]tss

4. Kropp S, Schulz-Schaeffer WJ, Finkenstaedt M, et al: The
Heidenhain variant of Creutzfeldt-Jakob disease. Arch Neurol 1999;
56:5561[Abstract/Free Full Text]

5. Kolmel HW: Visual illusions and hallucinations, in Balliere's
Clinical Neurology: International Practice and Research, vol 2, no
2: Visual Perceptual Deficits, edited by Kennard C. London,
Bailliere Tindall, 1993, pp 243264


http://neuro.psychiatryonline.org/TSS

TSS




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