Find information on thousands of medical conditions and prescription drugs.

Cone dystrophy

A cone dystrophy is an inherited ocular disorder characterized by the loss of cone cells, the photoreceptors responsible from both central and color vision. more...

Home
Diseases
A
B
C
Angioedema
C syndrome
Cacophobia
Café au lait spot
Calcinosis cutis
Calculi
Campylobacter
Canavan leukodystrophy
Cancer
Candidiasis
Canga's bead symptom
Canine distemper
Carcinoid syndrome
Carcinoma, squamous cell
Carcinophobia
Cardiac arrest
Cardiofaciocutaneous...
Cardiomyopathy
Cardiophobia
Cardiospasm
Carnitine transporter...
Carnitine-acylcarnitine...
Caroli disease
Carotenemia
Carpal tunnel syndrome
Carpenter syndrome
Cartilage-hair hypoplasia
Castleman's disease
Cat-scratch disease
CATCH 22 syndrome
Causalgia
Cayler syndrome
CCHS
CDG syndrome
CDG syndrome type 1A
Celiac sprue
Cenani Lenz syndactylism
Ceramidase deficiency
Cerebellar ataxia
Cerebellar hypoplasia
Cerebral amyloid angiopathy
Cerebral aneurysm
Cerebral cavernous...
Cerebral gigantism
Cerebral palsy
Cerebral thrombosis
Ceroid lipofuscinois,...
Cervical cancer
Chagas disease
Chalazion
Chancroid
Charcot disease
Charcot-Marie-Tooth disease
CHARGE Association
Chediak-Higashi syndrome
Chemodectoma
Cherubism
Chickenpox
Chikungunya
Childhood disintegrative...
Chionophobia
Chlamydia
Chlamydia trachomatis
Cholangiocarcinoma
Cholecystitis
Cholelithiasis
Cholera
Cholestasis
Cholesterol pneumonia
Chondrocalcinosis
Chondrodystrophy
Chondromalacia
Chondrosarcoma
Chorea (disease)
Chorea acanthocytosis
Choriocarcinoma
Chorioretinitis
Choroid plexus cyst
Christmas disease
Chromhidrosis
Chromophobia
Chromosome 15q, partial...
Chromosome 15q, trisomy
Chromosome 22,...
Chronic fatigue immune...
Chronic fatigue syndrome
Chronic granulomatous...
Chronic lymphocytic leukemia
Chronic myelogenous leukemia
Chronic obstructive...
Chronic renal failure
Churg-Strauss syndrome
Ciguatera fish poisoning
Cinchonism
Citrullinemia
Cleft lip
Cleft palate
Climacophobia
Clinophobia
Cloacal exstrophy
Clubfoot
Cluster headache
Coccidioidomycosis
Cockayne's syndrome
Coffin-Lowry syndrome
Colitis
Color blindness
Colorado tick fever
Combined hyperlipidemia,...
Common cold
Common variable...
Compartment syndrome
Conductive hearing loss
Condyloma
Condyloma acuminatum
Cone dystrophy
Congenital adrenal...
Congenital afibrinogenemia
Congenital diaphragmatic...
Congenital erythropoietic...
Congenital facial diplegia
Congenital hypothyroidism
Congenital ichthyosis
Congenital syphilis
Congenital toxoplasmosis
Congestive heart disease
Conjunctivitis
Conn's syndrome
Constitutional growth delay
Conversion disorder
Coprophobia
Coproporhyria
Cor pulmonale
Cor triatriatum
Cornelia de Lange syndrome
Coronary heart disease
Cortical dysplasia
Corticobasal degeneration
Costello syndrome
Costochondritis
Cowpox
Craniodiaphyseal dysplasia
Craniofacial dysostosis
Craniostenosis
Craniosynostosis
CREST syndrome
Cretinism
Creutzfeldt-Jakob disease
Cri du chat
Cri du chat
Crohn's disease
Croup
Crouzon syndrome
Crouzonodermoskeletal...
Crow-Fukase syndrome
Cryoglobulinemia
Cryophobia
Cryptococcosis
Crystallophobia
Cushing's syndrome
Cutaneous larva migrans
Cutis verticis gyrata
Cyclic neutropenia
Cyclic vomiting syndrome
Cystic fibrosis
Cystinosis
Cystinuria
Cytomegalovirus
Dilated cardiomyopathy
Hypertrophic cardiomyopathy
Restrictive cardiomyopathy
D
E
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
X
Y
Z
Medicines

The most common symptoms of cone dystrophy are vision loss (age of onset ranging from the late teens to the sixties), sensitivity to bright lights, and poor color vision. Therefore, patients see better at dusk and have progressive difficulty with daytime vision. Visual acuity usually deteriorates gradually, but it can deteriorate rapidly to 20/200; later, in more severe cases, it drops to counting fingers vision. Color vision testing using color test plates (HRR series) reveals many errors on both red-green and blue-yellow plates.

The pathogenesis of cone dystrophy has yet to be elucidated. It appears that the dystrophy is primary, since subjective and objective abnormalities of cone function are found before ophthalmoscopic changes can be seen. However, the retinal pigment epithelium (RPE) rapidly becomes involved, leading to a retinal dystrophy primarily involving the macula. The histological examination of the eyes of one such patient showed that the outer nuclear layer of cones and rods had disappeared completely, whereas the RPE showed pronounced pigment changes. There was also atrophy of the temporal disc.

The fundus exam via ophthalmoscope is essentially normal early on in cone dystrophy, and definite macular changes usually occur well after visual loss. Fluorescein angiography (FA) is a useful adjunct in the workup of someone suspected to have cone dystrophy, as it may detect early changes in the retina that are too subtle to be seen by ophthalmoscope. For example, FA may reveal areas of hyperfluorescence, indicating that the RPE has lost some of its integrity, allowing the underlying fluorescence from the choroid to be more visible. These early changes are usually not detected during the ophthalmoscopic exam.

The most common type of macular lesion seen during ophthalmoscopic examination has a bull’s-eye appearance and consists of a doughnut-like zone of atrophic pigment epithelium surrounding a central darker area. In another, less frequent form of cone dystrophy there is rather diffuse atrophy of the posterior pole with spotty pigment clumping in the macular area. Rarely, atrophy of the choriocapillaris and larger choroidal vessels is seen in patients at an early stage. The inclusion of fluorescein angiography in the workup of these patients is important since it can help detect many of these characteristic ophthalmoscopic features. In addition to the retinal findings, temporal pallor of the optic disc is commonly observed.

As expected, visual field testing in cone dystrophy usually reveals a central scotoma. In cases with the typical bull’s-eye appearance, there is often relative central sparing.

Because of the wide spectrum of fundus changes and the difficulty in making the diagnosis in the early stages, electroretinography (ERG) remains the best test for making the diagnosis. Abnormal cone function on the ERG is indicated by a reduced single-flash and flicker response when the test is carried out in a well-lit room (photopic ERG). The relative sparing of rod function in cone dystrophy is evidenced by a normal scotopic ERG, i.e. when the test is carried out in the dark. In more severe or longer standing cases, the dystrophy involves a greater proportion of rods with resultant subnormal scotopic records. Since cone dystrophy is hereditary and can be asymptomatic early on in the disease process, ERG is an invaluable tool in the early diagnosis of patients with positive family histories.

Read more at Wikipedia.org


[List your site here Free!]


Computer-controlled acupuncture quantification and separation of specific effects
From Neurological Research, 9/1/99 by Litscher, G

The effects of acupuncture have been described and handed down empirically for centuries but there are few reports based on objective data. The aim of this study was to provide selective evidence of a specific effect of acupuncture on the brain and the eye using a Doppler ultrasound technique. A transcranial Doppler sonography arrangement was developed to monitor blood flow profiles in the supratrochlear and middle cerebral arteries simultaneously and continuously. Two acupuncture schemas were tested in a randomized cross-over study with 13 patients with ophthalmologic diseases. Applying acupuncture

needles to special eye points increased the blood flow velocity in the supratrochlear artery significantly (p

Keywords: Computer-controlled acupuncture (CCA); transcranial Doppler sonography (TCD); supratrochlear artery; middle cerebral artery; brain; eye

INTRODUCTION

Acupuncture has its origin in Traditional Chinese Medicine (TCM) and is a holistic therapeutic principle that transcends the divisions among the specialties and subspecialties of Western medicine. More than 360 acupuncture points are located throughout the human body1. According to TCM, stimulation of these points, usually with needles, should activate 'Qi' or 'life energy'. The effects of acunpuncture have been described and handed down empirically for centuries but there are few objective reports based on objective data. There have been studies showing changes in the microcirculation of the skin detected with laser Doppler flowmetry2 and thermography3. Our group has reported initial evidence and preliminary data on measurable general and specific effects of acupuncture in the brain4-11.

The present report describes a new transcranial Doppler sonographic (TCD) monitoring arrangement that can simultaneously and continuously monitor the blood flow profiles of different cerebral vessels. The aim of the study was to provide selective evidence of a specific effect of acupuncture on brain and eye. Two acupuncture schemas, one to stimulate eye points and one to increase overall cerebral activity, were tested.

MATERIALS AND METHODS

TCD-monitoring arrangement

A bidirectional two-way probe holder was constructed for TCD-monitoring during acupuncture (Figure 1). The monitoring arrangement for simultaneous recording of Doppler sonographic signals in the right supratrochlear artery and right middle cerebral artery is stationary at the root of the nose and the outer auditory canal. The sonographic signals were recorded with a Multi-Dop T system (DWL Electronic Systems GmbH, Sipplingen, Germany). A 2 MHz and a 4 MHz probe were used.

Blood flow profiles in the supratrochlear artery, the inner end branch of the ophthalmic artery, were measured transorbitally with the smallest power value able to detect signals (maximum 20 mW/cm^sup 2^). This vessel is easily isolated and is quite constant without direct scanning of the bulbus with ultrasound. The Doppler probe was lightly positioned at the medial corner of the eye in a slightly medial and parietal direction using the specially constructed holder. Under acoustic control, the angle and position of the probe was adjusted until the greatest possible signal amplitude was reached. Changes in the blood flow velocity in the middle cerebral artery were registered continuously and simultaneously. Blood pressure was measured noninvasively (Cardiocap CC-104, Datex Medical Electronics, Hoevelaken, Holland).

Evaluated parameters

The mean blood flow velocity (vm) as determined from the Doppler frequency spectrum is an important parameter5. In contrast to the maximum value, vm can be determined without interference, even when undesirable signal noise is present.

Four hundred values of vm were averaged in each patient in three phases (A, starting 5 min before acupuncture; B, starting 2 min after inserting the needles; and C, starting 5 min after removal of the needles). The averaged values of the three measured phases were then compared for each patient.

Patients, acupuncture, and procedure

The study protocol was approved by the institutional ethics committee and all patients gave informed consent. Eight women and five males aged 24-63 years from the Department of Ophthalmology of the University in Graz were examined. Two patients had retinitis pigmentosa, two had cone dystrophy, two had glaucoma, two had optical nerve atrophy, two had keratoconjunctivitis, one had iridocyclitis, one had ureitis intermedia and one had endocrine orbitopathy. Each patient was studied twice, once after application of each acupuncture schema A and B (Figure 2). The interval between the two studies was at least two weeks. The choice of the schema for the initial treatment was randomized.

The patients were in a relaxed and comfortable position on a bed in our laboratory. The monitoring arrangement was positioned carefully. After a 10-min resting period, one of the two acupuncture schemes (A, B)1,9-11 was selected in a randomized cross-over study design.

Schema A was chosen to activate blood flow in the supratrochlear artery and schema B to activate blood flow in the middle cerebral artery.

Scheme A (Figure 2, left panel)

UB.2 Zanzhu

Location: In the depression of the medial end of the eyebrow.

Method: Puncture transversely 0.5-0.8 cun.

Ex.3 Yuyao

Location: At the midpoint of the eyebrow. Method: Puncture transversely 0.3-0.5 cun.

SJ.23 Sizhukong

Location: In the depression at the lateral end of the eyebrow.

Method: Puncture transversely 0.5-1 cun.

GB.1 Tongziliao

Location: 0.5 cun lateral to the outer canthus, in the depression on the lateral side of the orbit.

Method: Puncture transversely 0.3-0.5 cun.

SI.6 Yanglao

Location: When the palm faces the chest, the point is in the bony cleft on the radial side of the styloid process of the ulna.

Method: Puncture perpendicularly or obliquely 0.50.8 cun.

GB.37 Guangming

Location: 5 cun above the tip of the external malleolus, at the anterior border of the fibula.

Method: Puncture perpendicularly 1-1.5 cun.

Scheme B (Figure 2, right panel)

CV.6 Qihai

Location: 1.5 cun below the umbilicus. Method: Puncture perpendicularly 1-2 cun.

PC.6 Neiguan

Location: 2 cun above the transverse crease of the wrist, between the tendons of the m. palmaris longus and flexor carpi radialis.

Method: Puncture perpendicularly 0.5-1 cun.

ST.36 Zusanli

Location: 3 cun below Dubi (ST.35), one fingerbreadth from the anterior crest of the tibia.

Method: Puncture perpendicularly 1-2 cun.

SP.6 Sanyinjia

Location: 3 cun directly above the tip of the medial malleolus, on the posterior border of the medial aspect of the tibia.

Method: Puncture perpendicularly 1-1.5 cun.

The points were punctured vertically with sterile, single-use needles (Huan Qui, Suzhou, China) after local disinfection of the skin. Stimulation was achieved by rotation with lifting and thrusting of the needles.

Statistical analysis

The data were tested with analysis of variance (oneway repeated-measure ANOVA) SigmaStat (Jandel Scientific Corp., Erkrath, Germany). The results were given as means + /- standard deviations (x +/- SD). The criterion for significance was defined as p

RESULTS

Figure 2 shows the values of the mean blood flow velocity in the right supratrochlear and right middle cerebral arteries for both acupuncture schemas. Vm in the supratrochlear artery increased significantly with schema A (p

DISCUSSION

Our patients were studied with sophisticated modern technology while undergoing a pre-scientific intervention. We found that applying acupuncture needles to special eye points (scheme A) increased the blood flow velocity in the supratrochlear artery significantly compared to the reference interval before acupuncture. In the middle cerebral artery only a minimal, nonsignificant increase in the blood flow velocity was seen. In contrast, acupuncture of points that are held to increase cerebral blood flow velocity according to TCM4-8 did increase blood flow velocity of the middle cerebral artery while leaving that in the supratrochlear artery unchanged.

In 1987, Aaslid12 pointed out that light exposition induced different flow profiles in the posterior cerebral artery and the superior cerebellar artery measured by TCD. An optical stimulus elicited a 16% increase in blood flow through the posterior cerebral artery but not in the superior cerebellar artery. Cognitive stimulation can elicit 10%-20% changes in the cerebral blood flow velocity during TCD-monitoring of the middle cerebral artery. The extent of these changes depends on the particular process (calculation, memory, speech) as a result of regional differences in cerebral activation2. In our study acupuncture increased blood flow velocity in the supratrochlear artery more than 60%. This change is higher than those described after optical or cognitive stimulation 13.

Modern technology can now objectively measure the effects of acupuncture in the brain4-11. In previous studies we have found that a specific acupuncture schema (B in the present study) can increase blood flow velocity in the middle cerebral artery and oxygen availability in the brain of healthy probands4-11,14 . Similarly, Cho et al.15 have used functional magnetic resonance imaging (MRI) to demonstrate regional effects of acupuncture on cerebral structures of the visual system. Functional MRI showed a local change in the occipital lobe after stimulation of vision-related acupoints on the foot. In the present study we used neurosono raphic techniques to evaluate whether different acupuncture schemas have selective effects on different arteries in the brain. We chose the middle cerebral and supratrochlear arteries because they are well accessible to sonography. The vessels are closely related anatomically and phylogenetically. Nonetheless we were able to discern separate and specific effects of two acupuncture schemas on these two arteries using a system that monitors both arteries synchronously. The results were reproducible in different subjects.

The brain, retina and optical nerve all derive from the ectoderm. Embryologically the ectodermal neural crest forms the cerebral vesicle, which in turn forms the receptive structures of the eye. The corresponding arterial supply for both structures derives from the third pharyngeal arch artery to a common trunk (internal carotid artery) supplying the brain.

Our technique and study design can demonstrate that acupuncture produces specific and reproducible effects on the brain and the eye but cannot explain the underlying mechanism.

REFERENCES

1 Qiu Mao-Liang, ed. Chinese Acupuncture and Moxibustion, Brookline: Churchill Livingstone, 1993

2 Suter B, Kistler A. Beeinflubt die Akupunktur die Hautdurchblutung uber das autonome Nervensystem? Schweiz Med Wochenschr 1994; 124 (Suppl.): 3638

3 Zhang D, Gao H, Wen Z. Research on the acupuncture principles and meridian phenomena by means of infrared thermography. Chen Tzu Yen Chin 1990;15: 319-323

4 Litscher G, Schwarz G, Sandner-Kiesling A, Hadolt I, Eger E, Lenhard H. Transcranial near infrared spectroscopy and Doppler sonography during acupuncture. In: Litscher G, Schwarz G, eds. Transcranial Cerebral Oximetry, Lengerich: Pabst Science Publishers, 1997: pp. 184-198

5 Litscher G, Schwarz G, Sandner-Kiesling A, Hadolt I, Eger E. Effects of acupuncture on the oxygenation of cerebral tissue. Neurol Res 1998; 20 (Suppl. 1 ): 28-32

6 Litscher G, Schwarz G, Sandner-Kiesling A, Hadolt I. Robotic transcranial Doppler sonography probes and acupuncture. Intern J Neurosci 1998; 95:1-15

7 Litscher G, Schwarz G, Sandner-Kiesling A, Hadolt I. Transkranielle Doppler-Sonographie-Robotergesteuerte Sonden zur Quantifizierung des Einflusses der Akupunktur. Biomed Technik 1997; 42: 11 frl 22

8 Litscher G, Schwarz G, Sandner-Kiesling A. Computerkontrollierte Akupunktur. Akupunktur Theorie und Praxis 1998; 3:133-142 9 Litscher G, Yang NH, Wang L, Schwarz G. Quantitative Separation spezifischer Akupunktureffekte von Gehirn und Auge mittels bidirektionaler UltraschalImegkonstruktion. Akupunktur Theorie und Praxis 1998; 4: 212-217

10 Litscher G, Yang NH, Wang L. Ultrasound-controlled acupuncture. Internet J Anesthesiology 1998; 2/4 http://www.ispub.com/ journals/IJA/Vol2N4/acu.htm

11 Litscher G, Wang L, Yang NH, Schwarz G. Ultrasound-monitored effects of acupuncture on brain and eye. Neurol Res 1999; 21: 373-377

12 Aaslid R. Visually evoked dynamic blood flow response of the human cerebral circulation. Stroke 1987;17: 771-775 13 Diehl RR, Berlit P. Funktionelle Dopplersonographie in der Neurologie, Berlin: Springer, 1996

14 Litscher G. A multifunctional helmet for noninvasive neuromonitoring. J Neurosurg Anesthesiol 1998; 10: 116-119 15 Cho ZH, Chung SC, Jones JP, Park JB, Park HJ, Lee HJ, Wong EK, Min BI. New findings of correlation between acupoints and corresponding brain cortices using functional MRI. Proc Natl Acad Sci USA 1998; 95: 2670-2773

G. Litscher*, L. Wang*, N.H. Yangt and G. Schwarz:

*Biomedical Engineering, :Neuroanesthesia and Neurointensive Care Medicine at the Department of Anesthesiology and Critical Care, t Department of Ophthalmology, University of Graz, Austria

Correspondence and reprint requests to: Prof. Dr G. Litscher, Biomedical Engineering at the Department of Anesthesiology and Critical Care, University of Graz, Auenbruggerplatz 29, A-8036 Graz, Austria. Accepted for publication March 1999.

Copyright Forefront Publishing Group Sep 1999
Provided by ProQuest Information and Learning Company. All rights Reserved

Return to Cone dystrophy
Home Contact Resources Exchange Links ebay