Interstitial deletion 8p21.3----p23.1 in a 6-year-old girl.

We present a 6-year-old mentally retarded girl. Chromosome analysis showed an interstitial deletion of chromosome 8; 46,XX,del(8) (pter----p23.1::p21.3----qter). The proposita had normal activities of glutathione synthetase reductase (GSR) and factor VII. Parental chromosomes were normal.     

September 2004: a 6-year-old girl with headache and stiff neck

Free-living amebas in the genera Naegleria, Acanthamoeba and Balamuthia are known to cause CNS infections. Here we report a case of fatal granulomatous amebic meningoencephalitis (GAE) caused by Balamuthia mandrillaris in a 6-year-old previously healthy girl who presented with headache and stiff neck. She was treated medically for brain abscess after a CT scan identified a ring-enhancing lesion in the right temporo-parietal area. A brain biopsy showed necrosis and granulomatous inflammation. Subsequently, multiple new lesions appeared in the brain bilaterally. A second brain biopsy revealed viable amebic trophozoites that were most abundant in perivascular spaces, accompanied by neutrophils, macrophages and eosinophils. Immunofluorescence study confirmed the amoeba as Balamuthia mandrillaris. This case demonstrates that making diagnosis of GAE pre-mortem requires a high index of suspicion. Amebic infection should be included in the differential diagnosis of any granulomatous lesion in CNS; and careful search for amebic parasites should be carried out especially when necrosis predominates in the pathological material.     

Fatal parvovirus myocarditis in a 5-year-old girl

Infection with parvovirus B19 is common in children and typically causes mild illness. We report here the case of a 5-year-old girl who died suddenly, 2 weeks after the clinical diagnosis of a parvoviral infection (erythema infectiosum). Microscopic examination of the heart showed severe myocarditis with extensive T-cell and macrophage infiltration. Cultures, serology, and molecular analyses of serum for enteroviridae, adenovirus, influenza, varicella zoster, cytomegalovirus, and herpes simplex viruses were negative. Quantitative polymerase chain reaction (PCR) analysis for parvovirus B19 in peripheral blood, however, showed active infection (91,000 genomes/mL serum; 2.4 genomes/mononuclear cell). Despite the presence of myocarditis, immunostaining for parvoviral surface antigens was negative in the heart. Quantitative PCR analysis of paraffin sections showed that myocardial parvoviral content was significantly less than that of the normal appearing kidney and within the range predicted simply by tissue blood content. Thus, parvovirus B19 infection can be complicated by fatal myocarditis. Because the virus does not appear to have infected the heart, per se, we speculate that myocarditis arose from immunological cross-reaction to epitopes shared between the virus and the myocardium. HUM PATHOL 32:342-345.     

Nuchal fibrocartilaginous pseudotumor in a 10-year-old girl

We report a case of a 10-year-old girl who, 4 years after a trauma to the neck, developed a soft tissue mass in the midline at the C5-6 level. The lesion was not attached to the bone and was composed of fibrocartilaginous tissue, similar to the nuchal fibrocartilaginous pseudotumor, a rare, recently reported condition that has been described in the medical literature in only 4 cases, all of them adults.     

Interstitial deletion 8p21.3 → p23.1 in a 6-year-old girl

We present a 6-year-old mentally retarded girl. Chromosome analysis showed an interstitial deletion of chromosome 8; 46,XX,del(8) (pter → p23.1::p21.3 → qter). The proposita had normal activities of glutathione synthetase reductase (GSR) and factor VII. Parental chromosomes were normal.     

Interidentity amnesia in dissociative identity disorder

Introduction: Patients diagnosed with dissociative identity disorder (DID) usually present with alternative personality states (alters) who take separate control of consciousness. Commonly, one alter will claim they have no awareness of events which took place when another alter was in control. However, some kinds of material are transferred across the alter boundary. Huntjens et al. devised an objective method of demonstrating such transfer.

Methods: In the main study, following Huntjens et al., for three patients, two alters were taught different sets of nouns. The following week, one of the alters was given a recognition memory test including both sets plus distractor words. The patients in the Huntjens experiment responded in the same way to words in both sets.

Results: In the present experiemnt, two of the patients tested had pairs of alters where there was no interference from the material which was presented to the other alter. In one of these cases, there was breakthrough with one pairing of alters, a pattern matched in a subsidiary experiment.

Conclusions: The population of individuals with DID are not homogeneous with respect to the depth of the blocking of episodic material from one alter to another.     

Isolated vaginal myeloid sarcoma in a 16-year-old girl

Involvement of the female genital tract by myeloid sarcoma as the initial presentation is extremely uncommon, especially in the vagina. The lack of specific histologic features and the unusual location can be a diagnostic challenge to both the surgical pathologist and the clinician. The very few reported cases of myeloid sarcoma occurring in the vagina have been exclusively seen in adults. We report a 16-year-old girl who presented with a vaginal mass of 4 weeks duration. The initial clinical impression was a Bartholin cyst vs an abscess. However, because of persistence of the vaginal mass after a full course of antibiotic treatment, a biopsy was performed. Immunohistochemistry supported the diagnosis of myeloid sarcoma. Peripheral blood and bone marrow studies were normal. The patient received 4 cycles of chemotherapy and remained disease free 5 months from therapy completion. The clinical course, diagnostic workup, and differential diagnosis of our patient are discussed. Reported cases of myeloid sarcoma occurring in the vagina are reviewed and summarized.