Assignment 5 - Psychology 486/686
Graduate Students
Name: _________________
Identify the following in each of these three cases:
1. Symptoms
case 1 -
case 2 -
case 3 -
2. Diagnostic tests
case 1 -
case 2 -
case 3 -
3. Brain areas affected
case 1 -
case 2 -
case 3 -
4. Differential and Final Diagnoses
case 1 -
case 2 -
case 3 -
5. Treatment
case 1 -
case 2 -
case 3 -
Case 1 - CLINICAL HISTORY: This 43-year-old man presented with a "seizure"
of dizziness, associated with difficulty in walking and performing simple
movements. Four months later the same symptoms were accompanied with
partial, and six months later with generalized epileptic seizures. Upon admission
the patient complained of diffuse headache and some difficulties in finding words.
Neurological and neuropsychological examinations did not reveal any motor,
sensory, cognitive deficits, nor speech disturbances. Electroencephalography
(EEG) showed a dysrythmic region with oppositional-phase waves in the left
fronto-parietal region with a normal background activity, without epileptic activity.
NEUROIMAGING: Cerebral magnetic resonance imaging (MRI) revealed an
extra-axial, parasagittal, tumor of 2.5cm of diameter in the left parietal region
(figure 1). The lesion was iso-intense on T1- and slightly hyper-intense on T2
weighted images, with homogenous contrast enhancement after Gadolinium
administration. The tumor was attached to the dura without deforming the sagittal
sinus but compressing the underlying brain parenchyma. Four-vessels digitally
subtracted angiography showed that tumor to be supplied by a parietal branch of
the left middle meningeal artery (Figure 2).
SURGICAL INTERVENTION: Left sided parietal craniotomy and complete
resection of the well delineated extra-axial tumor was performed. The
postoperative course was uneventful.
HISTOLOGICAL DESCRIPTION: A diffuse, dense follicular lymphoplasmacytic
infiltration dominated the histological picture (Fig 3A and 3B; single arrow). The
small spaces which remained between the extensive lymphoplasmacytic
infiltrations were taken up by round-oval cells with large nuclei containing finely
dispersed chromatin, some with small nuclear vacuoles. These cells showed
morphological similarity to meningothelial cells but morphologically it was difficult
to rule out the possibility of dendritic cells. There was no apparent lobular or
fascicular pattern, whorl formation. A pattern typical for meningothelial
meningioma (region surrounded by arrows on Fig 3B), was rarely detected in the
biopsy specimen. The tumor was richly vascularized.
FINAL DIAGNOSIS: LYMPHOPLASMACYTE-RICH MENINGIOMA
DISCUSSION: The radiological appearance of the parietal, parasaggital,
extra-axial tumor including the vascular supply from the external carotid artery
system as seen on angiography, was indistinguishable from that associated with
meningioma. The histological appearance of the surgically removed tumor was
unusual. An extensive lymphoplasmacytic infiltration obscured the meningothelial
component of the tumor, which required immunohistochemistry to rule out a
lymphoproliferative process. We classified the tumor as a lymphoplasmacytic-rich
meningioma, a rare meningioma variant. The immunohistochemical analysis
enabled us to identify the meningothelial component of the tumor thus to exclude
the diagnosis of Casteman's disease. The biological behavior of these
meningiomas seems to depend on the meningioma component but, as the lesion
may be associated with haematological abnormalities, a close postoperative
follow-up of patients with this type of meningioma is needed.
Case 2 - CLINICAL HISTORY: A previously healthy 6 year-old girl presented to
the emergency department with gradually worsening headache, stiff neck,
nausea and vomiting, and low-grade fever for several days in the early October.
There was no history of trauma, chills, night sweating or upper respiratory tract
infection. Physical and neurological examinations as well as routine laboratory
tests and chest radiograph were within normal limits. Blood culture for
microorganisms was negative.
NEROIMAGING: A head MRI with contrast demonstrated a 2.0 cm ringenhancing lesion in the right parietotemporal region, consistent with abscess
formation (Figure 1. MRI with contrast). CSF Gram stain and microorganism
cultures were negative. The patient was treated empirically with Acyclovir,
Oxacillin and Flagyl. The abscess appeared to have responded to the therapy
based on a repeat imaging study, although the patient's symptoms were not
relieved.
HISTOLOGICAL DESCRIPTION: On CT and MRI, multiple cystic lesions were
noted in both cerebral hemispheres. A brain biopsy was performed showing
multiple foci of microorganisms arranged mostly in perivascular spaces (Figure 3:
H&E and Figure 4: Trichrome.). An immunofluorescence antibody stain was
performed for further classification of the microorganisms. The therapy was then
switched to pentamidine, sulfadiazine, intraconazole and azithromycin. But the
patient's condition kept deteriorating and she died in the late January, 75 days
after admission. The blood and brain tissue cultures remained negative. At
autopsy, the brain sections showed edema, necrosis, tremendous reactive
astrogliosis, acute and chronic inflammation and foci of multinucleated giant cell
reaction. Scant eosinophils were also present. Partially degenerated
microorganisms were focally abundant.
FINAL DIAGNOSIS: GRANULOMATOUS AMEBIC MENINGOENCEPHALITIS
CAUSED BY BALAMUTHIA MANDRILLARIS.
DISCUSSION: Balamuthia mandrillars is a relatively newly described pathogen
related to the free-living ameba superfamily, which also includes Naegleria
fowleri and Acanthamoeba species. These free-living amebas, except for B.
mandrillaris, have been isolated from the soil, fresh water of swimming pool, and
air conditioning unit. They can cause infections of skin, respiratory tract and
brain in both human and animals. Although the route of invasion into the brain is
still unclear, hematogenous spread of amebas through a skin lesion or the
respiratory tract has been postulated. The identification of the organism in tissue
section is the key to make the diagnosis of amebic meningoencephalitis. The
presence or absence of a cyst wall of trophozoites can be used to distinguish
Balamuthia and Acanthamoeba from N. fowleri since the latter does not produce
cyst forms in tissue. There is no specific treatment for B. mandrillaris infections .
Some in vitro studies demonstrated that B. mandrillaris is sensitive to
pentamidine methionate, azithromycin and clarithromycin, but none are
amebicidal at nontoxic concentrations. In fact, the treatment for CNS amebic
infection is in general non-specific and late, mainly due to difficulty of diagnosis.
CASE 3 - CLINICAL HISTORY: A 56-year-old woman, with history of peptic
ulcer and family history for cerebrovascular disease was referred to our Division
of Internal Medicine with headache, asthenia and generalized discomfort. She
reported a cerebrovascular accident manifesting as a right brachial and crural
hyposthenia ten month ago, almost completely receded at observation time; she
also referred recurrent episodes of proximal deep venous thrombosis (DVT) of
lower limbs in the last seven months.
Laboratory findings: In order to identify any thrombophilia, in view of her personal
and familiar history, we tested blood prothrombin time, as INR, activated partial
thromboplastin time, as ratio, fibrinogen, protein C and S, antithrombin III,
activated protein C resistance, anti-cardiolipin antibodies IgG and IgM, lupus
anticoagulant, plasminogen activator inhibitor type 1, d-dimer, gene polimorphism
of clotting factor II and V, gene polymorphism C9774T and G3775A of
apolipoprotein B and gene polymorphism C3932T and C4070T of apolipoprotein
E resulted all in normal range; while gene polimorphism of tetrahydrofolate
reductase and angiotensin converting enzyme revealed heterozigosity for both.
Subsequently, homocysteinemia test revealed mild hyperhomocysteinemia.
Neuroimaging: A magnetic resonance imaging (MRI) scan showed little and
multiple ischemic lesions in particular in left cerebral peduncle (fig 1A), semioval
centres (fig 1B), left pons and midbrain.
Moreover, a vascular ultrasound examination ruled out the presence of
significant stenosis of arterial cerebral vessels and confirmed proximal DVT and
post-thrombotic syndrome of lower limbs.
A peripheral blood smear did not show any finding suggestive for haematological
disorders. A bone marrow biopsy showed a slight hyperplasia of erythrocytic
bone marrow cell line.
Final Diagnosis: THROMBOEMBOLYTIC CEREBRAL ISCHEMIC LESIONS
DUE TO NON-IMMUNE HAEMOLYTIC ANEMIA. In particular, due to the
exclusion of other non-immune haemolityc disorders by means of age and
clinical history we hypothesized paroxysmal nocturnal hemoglobinuria. This
diagnosis was confirmed by an immunophenotypic profile of peripheral blood
cells, showing a 15% of deficient CD59 erythrocytes, and by the presence of
hemosiderinuria.
During her hospitalization two haemotrasfusions were necessary on occasion of
two concurrent haemolytic crises. Following dismission, in order to prevent
further thromboembolic events, the patient began oral anticoagulation therapy
with warfarin. Moreover she was treated with B12 vitamin and folate
supplementation.