Hy 2104

[tommyk]

Hy 2104 :)
A 30-year-old gentleman of the NBA basketball league is brought to the emergency department because of fever, headache, and seizures of abrupt onset.
His temperature is 39.4 C (103 F). The patient is disoriented as to place and time.
Physical examination reveals mild nuchal rigidity.
On admission, laboratory studies show 10,000 neutrophils/mm3 in the peripheral blood, while a lumbar puncture is significant for moderately increased CSF pressure, lymphocytosis (500/mm3), and presence of red blood cells.
Electroencephalographic studies demonstrate bitemporal periodic complexes on a slow background. Neuroimaging shows multifocal hemorrhagic lesions in the temporal lobes. What drug, or what else will you do to help out? What bug?
1- Supportive treatment until CSF culture results are available
2- Cerebral angiographic studies
3- Treatment with acyclovir
4- Treatment with antimycotic agent
5- Treatment with antimicrobial agents
6- Treat with massage therapy
7- Practice Voodoo magic
8- Neuro Surgery Burr holes to relieve pressure





















a) Answer is #3, give acyclovir (there are other choices emerging too). The clinical presentation outlined is consistent with herpes simplex encephalitis, which is usually due to herpes simplex virus 1 (HSV 1). HSV is thought to cause encephalitis following transport to the brain along the trigeminal nerve. This usually occurs in persons harboring the latent viral form in the Gasserian ganglion. Bitemporal hemorrhagic necrosis is characteristic of herpes encephalitis. Most patients develop symptomatology of abrupt onset, with fever, headache, nuchal rigidity, and confusion. Motor and sensory deficits are often observed on physical examination. Treatment should be promptly started whenever there is reasonable suspicion of herpes encephalitis. In fact, acyclovir and similar drugs are highly effective against this form of encephalitis. Herpes encephalitis is usually fatal without treatment case, note the typical CSF changes associated with viral encephalitis. Supportive treatment until CSF culture results are available is not adequate, since often culture results are negative. Furthermore, treatment is effective only if started in the earliest stages. Cerebral angiographic studies are not useful in this case. Treatment with an antimycotic agent is adequate in cases of fungal meningoencephalitis which usually occurs in immunocompromised patients. Cryptococcal meningoencephalitis, for example, is frequent in AIDS patients. Treatment with antimicrobial agents should be immediately started whenever there is clinical and/or laboratory evidence of bacterial meningitis. The CSF in purulent (bacterial) meningitis shows marked neutrophilia, increased protein concentration, and reduced glucose. Wow! Hard!

[tommyk]

Hy 2105

A 9-month-old boy named Mike Tyson, is taken to the emergency room because of high fever.

Breath sounds are diminished in the lungs, and a chest x-ray film shows lobar pneumonia.

Probable streptococcal pneumonia is demonstrated in Gram's stain of sputum and then later confirmed by culture. The child responds to antibiotic therapy. A detailed history is taken during the admission, which reveals that this is the third episode of pneumonia in this young child; the two previous episodes occurred at 6 and 71/2 months of age.

One of the mother's brothers had died of infection at age 9. Immunoglobulin studies demonstrate the following:

IgG
80 mg/dL
[normal 723-1685 mg/dL]
IgA
60 mg/dL
[normal 81-463 mg/dL]
IgM
20 mg/dL
[normal 48-271 mg/dL]

Studies of the lymphocyte population demonstrate normal numbers of T cells and markedly decreased B cells. Which of the following is the most likely diagnosis? (Hint: look at immunoglobins titer)

1-Bruton agammaglobulinemia
2-Common variable immunodeficiency
3-DiGeorge syndrome
4-Transient hypogammaglobulinemia of infancy
5-Wiskott-Aldrich syndrome

























a) This is Bruton agammaglobulinemia (X-linked agammaglobulinemia). It causes low or absent numbers of B cells, leading to a panhypogammaglobulinemia. Cellular immunity is intact. The patients typically develop infections after about six months of age, when maternal antibodies have decreased to low levels. There is a life-long predisposition for recurrent pyogenic infections, particularly of the lungs, sinuses, and bones. While the condition is classically considered X-linked, this can only be proven in about 20% of cases. These patients require life-long immunoglobulin therapy and aggressive antibiotic management when infections do develop.
Common variable immunodeficiency can have some clinical overlap with Bruton's agammaglobulinemia, but is characterized by normal B cell levels and typically presents in the second or third decade of life.
DiGeorge syndrome typically presents with hypocalcemia in infancy.
Transient hypogammaglobulinemia of infancy has clinical overlap with Bruton's agammaglobulinemia, but can be distinguished by the presence of normal B cell numbers.
Wiskott-Aldrich syndrome is an X-linked disorder with eczema, thrombocytopenia, and recurrent infection

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