FELTY’ SYNDROME

AND
STEELE-RICHARDSON-OLSZEWSKI
SYNDROME
(PROGRESSIVE SUPRANUCLEAR PALSY-
(PSP))
Felty’s syndrome
Syndrome characterized by;
-splenomegaly
-chronic rheumatoid arthritis
-Leucopenia

there are usually pigmented

spots on the skin of the lower
extremities, and sometimes
there is other evidence of
hypersplenism such as
Causes and risk factors
 The cause of Felty's syndrome is
unknown. It is more common in
people who have had rheumatoid
arthritis for a long time. People
with this syndrome are at risk of
infection because they have a
low white blood cell count
 Immune-mediated disease
Symptoms
 patients suffer from painful, stiff, and
swollen joints, most commonly in the
joints of the hands, feet, and arms.
 abnormally enlarged spleen and

abnormally low levels of certain white

blood cells (neutropenia), anemia,
thrombocytopenia and/or vasculitis.
 maybe fever, weight loss, and/or fatigue

 discoloration of the skin, particularly of

the leg (abnormal brown pigmentation),

sores (ulcers) on the lower leg, and/or an
abnormally large liver (hepatomegaly).
Complications
recurrent infection,
hypersplenism causing anaemia

an thrombocytopenia,
lymphadenopathy,

skin hyperpigmentation &

cutaneous ulceration.
Diagnostic criteria
 rheumatoid arthritis,
 an enlarged spleen (splenomegaly),

 and an abnormally low white blood count.

It affects less than 1% of patients with rheumatoid

arthritis.
 Treatment
 control the underlying Rheumatoid
Arthritis (Immunosuppressive therapy
for RA often improves
granulocytopenia and splenomegaly).
 splenectomy may improve

neutropenia in severe disease.

 use of rituximab and leflunomide have

been proposed.
 Steele-Richardson-Olszewski
syndrome
(Progressive supranuclear palsy
(PSP ) )
 A progressive neurological disorder with
A progressive neurological disorder with
onset during the sixth decade,
characterized by;
 supranuclear ophthalmoplegia, especially

paralysis of the downward gaze,

 pseudobulbar palsy,

 dysarthria,

 dystonic rigidity of the neck and trunk,

 and dementia.
Genetics
Fewer than 1% of those with PSP have a family
member with the same disorder. A variant in
the gene for tau protein called the H1
haplotype, located on chromosome 17, has
been linked to PSP. Nearly all people with PSP
received a copy of that variant from each
parent, but this is true of about two-thirds of
the general population. Therefore, the H1
haplotype appears to be necessary but not
sufficient to cause PSP. Other genes, as well as
environmental toxins are being investigated as
other possible contributors to the cause of PSP.
Pathophysiology

 The affected brain cells are both

neurons and glial cells.
 The neurons display

neurofibrillary tangles, which are

clumps of tau protein, a normal
part of brain cell's internal
structural skeleton.
Pathophysiology
The principal areas of the brain affected are:
 the basal ganglia, particularly the
subthalamic nucleus, substantia nigra and
globus pallidus;
 the brainstem, particularly the portion of the
midbrain where "supranuclear" eye movement
resides;
 the cerebral cortex, particularly that of the
frontal lobes;
 the dentate nucleus of the cerebellum;

 and the spinal cord, particularly the area where

some control of the bladder and bowel resides.
Symptoms and signs
initial symptoms
-in two-thirds of cases is loss
of balance and falls.
-changes in personality,
-general slowing of movement,
-and visual symptoms.
Symptoms and signs
 Later symptoms
-signs are dementia (typically
including loss of inhibition and
ability to organize information),
-slurring of speech,
-difficulty swallowing,
-and difficulty moving the eyes,
particularly in the vertical direction.
symptoms and signs
 other signs are
-poor eyelid function,
-contracture of the facial muscles,
-a backward tilt of the head with
stiffening of the neck muscles,
-sleep disruption,
-urinary incontinence and
costipation
Symptoms and signs
 Cardinal Manifestations:
-Supranuclear ophthalmoplegia
-Neck dystonia
-Parkinsonism
-Pseudobulbar palsy
-Behavioral and Cognitive impairment
-Imbalance and Difficulties walking
-Frequent Falls
Differential diagnosis
 PSP is frequently misdiagnosed
as Parkinson's disease because of
the slowed movements and gait
difficulty,
 as Alzheimer's Disease because

of the behavioral changes.

 It is one of a number of diseases

collectively referred to as
Parkinson plus syndromes.
treatment and prognosis

There is currently no effective

treatment or cure for PSP,
although some of the symptoms
can respond to nonspecific
measures. The average age at
symptoms onset is 63 and
survival from that point averages
7 years with a wide variance