felty's and steele syndromes

8/14/2019 Felty's and Steele Syndromes

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FELTY SYNDROMEFELTY SYNDROME

ANDAND

STEELE-RICHARDSON-OLSZEWSKISTEELE-RICHARDSON-OLSZEWSKISYNDROMESYNDROME

(PROGRESSIVE SUPRANUCLEAR PALSY-(PROGRESSIVE SUPRANUCLEAR PALSY-

(PSP))(PSP))


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Syndrome characterized by;-splenomegaly

-chronic rheumatoid arthritis-Leucopenia

there are usually pigmentedthere are usually pigmented

spots on the skin of the lowerspots on the skin of the lower

extremities, and sometimesextremities, and sometimesthere is other evidence ofthere is other evidence of

hypersplenism such ashypersplenism such as

e ty s syn romee y s syn rome


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Causes and risk factorsCauses and risk factors

The cause of Felty's syndrome isThe cause of Felty's syndrome isunknown. It is more common inunknown. It is more common in

people who have had rheumatoidpeople who have had rheumatoid

arthritis for a long time. Peoplearthritis for a long time. Peoplewith this syndrome are at risk ofwith this syndrome are at risk of

infection because they have ainfection because they have a

low white blood cell countlow white blood cell count

Immune-mediated diseaseImmune-mediated disease


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SymptomsSymptoms patients suffer from painful, stiff, andpatients suffer from painful, stiff, and

swollen joints, most commonly in theswollen joints, most commonly in thejoints of the hands, feet, and arms.joints of the hands, feet, and arms. abnormally enlarged spleen andabnormally enlarged spleen and

abnormally low levels of certain whiteabnormally low levels of certain whiteblood cells (neutropenia), anemia,blood cells (neutropenia), anemia,thrombocytopenia and/or vasculitis.thrombocytopenia and/or vasculitis.

maybe fever, weight loss, and/or fatiguemaybe fever, weight loss, and/or fatigue

discoloration of the skin, particularly ofdiscoloration of the skin, particularly ofthe leg (abnormal brown pigmentation),the leg (abnormal brown pigmentation),sores (ulcers) on the lower leg, and/or ansores (ulcers) on the lower leg, and/or an

abnormally large liver (hepatomegaly).abnormally large liver (hepatomegaly).


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ComplicationsComplications

recurrent infection,recurrent infection,hypersplenism causing anaemiahypersplenism causing anaemia

an thrombocytopenia,an thrombocytopenia,

lymphadenopathy,lymphadenopathy,

skin hyperpigmentation &skin hyperpigmentation &

cutaneous ulceration.cutaneous ulceration.


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Diagnostic criteriaDiagnostic criteria

rheumatoid arthritis,rheumatoid arthritis,an enlarged spleen (splenomegaly),an enlarged spleen (splenomegaly),

and an abnormally low white blood count.and an abnormally low white blood count.

It affects less than 1% of patients with rheumatoidIt affects less than 1% of patients with rheumatoid

arthritis.arthritis.


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TreatmentTreatment

control the underlying Rheumatoidcontrol the underlying RheumatoidArthritisArthritis (Immunosuppressive therapy(Immunosuppressive therapy

for RA often improvesfor RA often improves

granulocytopenia and splenomegaly).granulocytopenia and splenomegaly).splenectomysplenectomymay improvemay improve

neutropenia in severe disease.neutropenia in severe disease.

use ofuse ofrituximabrituximab andand leflunomideleflunomide havehavebeen proposed.been proposed.


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ee e- c ar son- szewsee e- c ar son- szewssyndromesyndrome((Progressive supranuclear palsyProgressive supranuclear palsy

((PSPPSP) )) ) A progressive neurological disorder withA progressive neurological disorder with

onset during the sixth decade,onset during the sixth decade,characterized by;characterized by;

supranuclear ophthalmoplegia, especiallysupranuclear ophthalmoplegia, especiallyparalysis of the downward gaze,paralysis of the downward gaze,pseudobulbar palsy,pseudobulbar palsy, dysarthria,dysarthria, dystonic rigidity of the neck and trunk,dystonic rigidity of the neck and trunk, and dementia.and dementia.


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GeneticsGeneticsFewer than 1% of those with PSP have a familyFewer than 1% of those with PSP have a family

member with the same disorder. A variant inmember with the same disorder. A variant inthe gene for tau protein called the H1the gene for tau protein called the H1haplotype, located on chromosome 17, hashaplotype, located on chromosome 17, hasbeen linked to PSP. Nearly all people with PSPbeen linked to PSP. Nearly all people with PSP

received a copy of that variant from eachreceived a copy of that variant from eachparent, but this is true of about two-thirds ofparent, but this is true of about two-thirds ofthe general population. Therefore, the H1the general population. Therefore, the H1haplotype appears to be necessary but nothaplotype appears to be necessary but not

sufficient to cause PSP. Other genes, as well assufficient to cause PSP. Other genes, as well asenvironmental toxins are being investigated asenvironmental toxins are being investigated asother possible contributors to the cause of PSP.other possible contributors to the cause of PSP.


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PathophysiologyPathophysiology

The affected brain cells are bothThe affected brain cells are both

neuronsneurons andand glial cellsglial cells..

The neurons displayThe neurons displayneurofibrillary tangles, which areneurofibrillary tangles, which are

clumps ofclumps oftau proteintau protein, a normal, a normal

part of brain cell's internalpart of brain cell's internal

structural skeleton.structural skeleton.


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PathophysiologyPathophysiology

The principal areas of the brain affected are:The principal areas of the brain affected are: thethe basal gangliabasal ganglia, particularly the, particularly the

subthalamic nucleussubthalamic nucleus,, substantia nigrasubstantia nigra andandglobus pallidusglobus pallidus;;

thethe brainstembrainstem, particularly the portion of the, particularly the portion of the

midbrain where "supranuclear" eye movementmidbrain where "supranuclear" eye movementresides;resides; thethe cerebral cortexcerebral cortex, particularly that of the, particularly that of the

frontal lobesfrontal lobes;;

thethe dentate nucleusdentate nucleus of theof the cerebellumcerebellum;; and theand the spinal cordspinal cord, particularly the area where, particularly the area wheresome control of the bladder and bowel resides.some control of the bladder and bowel resides.
http://en.wikipedia.org/wiki/Basal_gangliahttp://en.wikipedia.org/wiki/Basal_gangliahttp://en.wikipedia.org/wiki/Subthalamic_nucleushttp://en.wikipedia.org/wiki/Subthalamic_nucleushttp://en.wikipedia.org/wiki/Substantia_nigrahttp://en.wikipedia.org/wiki/Substantia_nigrahttp://en.wikipedia.org/wiki/Globus_pallidushttp://en.wikipedia.org/wiki/Globus_pallidushttp://en.wikipedia.org/wiki/Brainstemhttp://en.wikipedia.org/wiki/Brainstemhttp://en.wikipedia.org/wiki/Cerebral_cortexhttp://en.wikipedia.org/wiki/Cerebral_cortexhttp://en.wikipedia.org/wiki/Frontal_lobehttp://en.wikipedia.org/wiki/Frontal_lobehttp://en.wikipedia.org/wiki/Dentate_nucleushttp://en.wikipedia.org/wiki/Dentate_nucleushttp://en.wikipedia.org/wiki/Cerebellumhttp://en.wikipedia.org/wiki/Cerebellumhttp://en.wikipedia.org/wiki/Spinal_cordhttp://en.wikipedia.org/wiki/Spinal_cordhttp://en.wikipedia.org/wiki/Spinal_cordhttp://en.wikipedia.org/wiki/Cerebellumhttp://en.wikipedia.org/wiki/Dentate_nucleushttp://en.wikipedia.org/wiki/Frontal_lobehttp://en.wikipedia.org/wiki/Cerebral_cortexhttp://en.wikipedia.org/wiki/Brainstemhttp://en.wikipedia.org/wiki/Globus_pallidushttp://en.wikipedia.org/wiki/Substantia_nigrahttp://en.wikipedia.org/wiki/Subthalamic_nucleushttp://en.wikipedia.org/wiki/Basal_ganglia


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Symptoms and signsSymptoms and signs

initial symptomsinitial symptoms

-in two-thirds of cases is loss-in two-thirds of cases is loss

of balance and falls.of balance and falls.-changes in personality,-changes in personality,

-general slowing of movement,-general slowing of movement,-and visual symptoms.-and visual symptoms.


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Symptoms and signsSymptoms and signsLater symptomsLater symptoms

-signs are dementia (typically-signs are dementia (typically

including loss of inhibition andincluding loss of inhibition and

ability to organize information),ability to organize information),-slurring of speech,-slurring of speech,

-difficulty swallowing,-difficulty swallowing,

-and difficulty moving the eyes,-and difficulty moving the eyes,

particularly in the vertical direction.particularly in the vertical direction.


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symptoms and signssymptoms and signs

other signs areother signs are-poor eyelid function,-poor eyelid function,

-contracture of the facial muscles,-contracture of the facial muscles,

-a backward tilt of the head with-a backward tilt of the head with

stiffening of the neck muscles,stiffening of the neck muscles,

-sleep disruption,-sleep disruption,

-urinary incontinence and-urinary incontinence and

costipationcostipation


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Symptoms and signsSymptoms and signsCardinal Manifestations:Cardinal Manifestations:

-Supranuclear ophthalmoplegia-Supranuclear ophthalmoplegia

-Neck dystonia-Neck dystonia

-Parkinsonism-Parkinsonism-Pseudobulbar palsy-Pseudobulbar palsy

-Behavioral and Cognitive impairment-Behavioral and Cognitive impairment

-Imbalance and Difficulties walking-Imbalance and Difficulties walking

-Frequent Falls-Frequent Falls


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Differential diagnosisDifferential diagnosis

PSP is frequently misdiagnosedPSP is frequently misdiagnosedasas Parkinson's diseaseParkinson's disease because ofbecause of

the slowed movements and gaitthe slowed movements and gait

difficulty,difficulty, asas Alzheimer's DiseaseAlzheimer's Disease becausebecause

of the behavioral changes.of the behavioral changes. It is one of a number of diseasesIt is one of a number of diseases

collectively referred to ascollectively referred to as

Parkinson plus syndromesParkinson plus syndromes..
http://en.wikipedia.org/wiki/Parkinson%27s_diseasehttp://en.wikipedia.org/wiki/Parkinson%27s_diseasehttp://en.wikipedia.org/wiki/Alzheimer%27s_Diseasehttp://en.wikipedia.org/wiki/Alzheimer%27s_Diseasehttp://en.wikipedia.org/wiki/Parkinson_plus_syndromehttp://en.wikipedia.org/wiki/Parkinson_plus_syndromehttp://en.wikipedia.org/wiki/Parkinson_plus_syndromehttp://en.wikipedia.org/wiki/Alzheimer%27s_Diseasehttp://en.wikipedia.org/wiki/Parkinson%27s_disease


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treatment and prognosistreatment and prognosis

There is currently no effectiveThere is currently no effectivetreatment or cure for PSP,treatment or cure for PSP,

although some of the symptomsalthough some of the symptoms

can respond to nonspecificcan respond to nonspecific

measures. The average age atmeasures. The average age at

symptoms onset is 63 andsymptoms onset is 63 andsurvival from that point averagessurvival from that point averages

7 years with a wide variance7 years with a wide variance

felty's and steele syndromes

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