Tehran University Medical Journal

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Background: Bickerstaff's brainstem encephalitis (BBE) is a very uncommon central nervous system disease with unknown etiology. As it is usually responsive to treatment, the diagnosis this disease is important. It seems There is no reported Bickerstaff's brainstem encephalitis case in Iran.
Case presentation: An 83 year old woman presented with vertigo, ataxia and dysarthria from a week prior to admission. Her T2 weighted MRI revealed high intensity lesions in the pons area. She received treatment with impression of ischemic stroke. After few days, lower extremities became weak symmetrically and deep tendon reflexes depressed. Deep sensory loss was noted too. The EMG-NCV studies showed acute polyneuropathy and in CSF examination increased protein content was noted. The patient improved after IVIG and corticosteroid therapy with impression of Bickerstaff's brainstem encephalitis.
Conclusion: In spite of severe initial presentation, Bickerstaff's brainstem encephalitis has a good prognosis if it diagnosed and treated properly. Although clinical features is too important, but paraclinical work up are necessary to diagnose this syndrome.

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Background: Hydatid disease or echinococcosis is a common parasitic disease of human and bovine, caused by infection with larva of the cestode echinococcus. Liver is the most common organ that is involved in this disease. Pelvic involvement and neurological symptoms, due to mass effect of pelvic involvement, in lower extremities are very uncommon manifestations of the disease.
Case presentation: A forty six year old man was referred to clinic of surgery at Ghaem Hospital, Medical University of Mashhad, Iran. The patient complained about weakness and motor impairment in right lower extremity accompanied by numbness and radicular pain over past two months. Physical examination demonstrated muscular atrophy and reduced muscular strength in right lower extremity. Computed tomography and ultrasonographic studies showed a cystic mass in right side of the pelvic cavity with extention to the sciatic notch and another cystic mass in right gluteal region. Surgical operation revealed a cystic mass deep in pelvic cavity with the extention to the right sciatic notch with compression of nerve roots. The cystic mass was contained of daughter cysts which confirmed the diagnosis of hydatid cyst disease. This diagnosis was confirmed by pathologic assessment.
Conclusion: Although uncommon, but hydatid disease can involve the pelvic cavity and make a pelvic, usually cystic, mass that can make compression on nerve roots and so making neurologic symptoms in lower extremities. So in endemic areas for hydatid disease, such as Iran, pelvic hydatid cysts should be considered as a possible differential diagnosis in patients presenting with the sciatic pain and neurological manifestations in whom a pelvic mass has been found too.

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Background: The Trigemino-cardiac reflex (TCR) has been studied as a phenomenon including bradycardia, arterial hypotension, apnea and gastric hypermotility during manipulation of the peripheral or central parts of the trigeminal nerve.
Case presentation: We report a case of a 26-year-old man undergoing surgery for a skull base extra axial tumor in right petrous bone suspected to metastasis of a previous renal cell carcinoma which had been treated four years ago. The patient presented with continuous and unilateral headache and difficulty in swallowing, sensory neural hearing loss, nasal speech and tongue deviation to left side. He underwent general anesthesia with standard monitoring and total intravenous anesthetic technique. The first episode of sudden onset bradycardia and hypotension related to surgical manipulation was detected intraoperatively in which the heart rate spontaneously returned to normal level once the surgical manipulation stopped. However, it repeated several times by beginning of tumor resection and manipulation in the region of trigeminal nerve. The intensity of bradycardia in subsequent episodes of TCR was relatively crescendo and had no fatigability. Finally, it was treated by administration of a single dose of atropine (0.5mg/IV) and did not happen again.
Conclusion: The risk of TCR should be considered in any neurosurgical intervention involving trigeminal nerve and its branches, especially at the skull base surgeries. The vigilance of the medical team and continuous intraoperative hemodynamic monitoring alerts the surgeons to interrupt surgical maneuvers upon the TCR occurrence, immediately.

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Background: Anemia at the time of birth may cause some problem like asphyxia, heart failure shock or even death in a neonate. Different etiologies can be considered for this problem. Parvovirus B19, as a viral organism, can cause hydrops fetalis and neonatal anemia and consequent complications. We present here a case of newborn infant with severe anemia who had human parvovirus B19 infection.
Case Presentation: A male newborn with gestational age of 36 week was born from a mother with poor prenatal care and history of contact with domestic animal. The neonate was very pale with Apgar score 2 at 1 min and received resuscitation, mechanical ventilation and repeated blood transfusion The hemoglobin level was significantly low. Analysis was made based on the clinical presentations. According to the case history, physical and laboratory findings, neonatal severe anemia induced by parvovirus B19 infection was suggested and Laboratory work up documented his infection with parovirus B19.
Conclusion: Parvovirus B19 (B19 virus) is the smallest single strand linear DNA virus in animal viruses, which is the only strain of parvovirus that is pathogenic in humans. Human parvovirus B19 may cross the placenta and result in fetal infection, morbidity and death. Parvovirus is an uncommon cause of neonatal anemia and hydrops fetalis so this etiology must be considered in differential diagnosis of anemia at birth.

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Background: The anatomy of the palmar vascular arches and their variations, being one of the most challenging anatomical regions for reconstructive surgeon.
Case Presentation: During a routine dissection of a male adult cadaver in dissection hall of zahedan university of medical sciences, a complex, unilateral and rare variation in the pattern of blood supply to the palm of the right hand was observed. The history of the individual and cause of the death is not known. In this cadaver there was an incomplete superficial palmar arterial arch had no contribution from the radial artery. The superficial palmar arch giving only one common palmar digital artery, that supply second interdigital space and then it terminated by giving rise to a common trunk for princeps pollicis and radialis indicis arteries. Absence of the second and third common palmar digital artery with the contiguous sides of the third and forth interdigital spaces supply by the second and third palmar metacarpal arteries from the deep palmar arch respectively. The third palmar metacarpal artery giving rise to a branch which supplies the medial side of the little finger.
Conclusion: Having knowledge of the variations of vascular patterns resulting from a number of developmental errors could provide an important source of information for Anatomists, Radiologist, reconstructive and vascular surgeons.

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Background: Traumatic amputation of upper limb above the level of the elbow, are rare injuries that a surgeon may be faced with a handful of cases during his career. Even with advanced techniques in microvascular surgery, the survival of the transplanted limb that cut off completely from the arm is relatively weak. Case Presentation: Four injured people (patient) in Imam Khomeini Hospital during 1999-2013 in Tehran University of Medical Sciences were treated. Two patients had complete amputation of limb and other patients suffered from complete cutting in artery and vein along with neurological and soft tissue damages. There was not any ischemia or gangrene. However, efficiency of limbs with damages in peripheral nerves was not significant. The main reason was lack of immediate repair of the damaged nerves. In the recent examination, after discharge, the performance of the limb is firstly related to the conditions of the nerve system and secondly it depends on the severity of the injury in each of the muscles. However, none of the patients were dissatisfied of any situation and they were prepared to make any cooperation for improving the condition of their transplanted limb. Conclusion: Transplantation of the amputated limb can be done in spite of limited resources. Any delay in repairing damaged nerves will result great reduction of final organs’ performance. A limb, made from the own body, always take precedence to prosthesis, even when the efficiency is low.

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Background: Twin pregnancy with a fetus and a diploid complete mole is not rare, but, the cases of twin pregnancy with a partial mole are rare. Nowadays, the prevalence of these cases has been increased due to the high rate of assisted reproductive techniques in reproductive medicine. The importance of twin pregnancy with a fetus and a diploid complete mole is mainly due to systemic complications such as hypertension and maternal hemorrhage and the possibility of trophoblastic tumor following delivery. Different studies have reported some results about similar cases, but limited case reports are presented in our country. The aim of this study is to report a case with incomplete molar pregnancy concomitant with a live fetus. Case presentation: A 21 yr old woman (G2 ab1) referred to emergency department of Ghaem University Hospital in Mashhad. She complained of scant vaginal bleeding and spotting and a sonography report of a combined molar and normal pregnancy with 15 weeks gestational aged. During hospitalization, the pregnancy complicated with hypertension and proteinuria. Termination of pregnancy was planned at 17th weeks of gestation due to severe preeclampsia. After evacuation of uterus, during follow up visits, -hCG titer raised. Metastasis evaluation was negative. Pathology reports showed patial mole. Then, three doses of methotrexate (50 mg/m2 intra muscular) was administered and finally, according to the monthly follow up, -hCG level was undetectable. Conclusion: The rate of pregnancy complications such as hypertension, hyperthyroidism, and obstetrics hemorrhage and also the risk of Gestational Trophoblastic Neoplasm (GTN) are increasing in incomplete molar pregnancy. Therefore, early diagnosis and timely treatment of molar pregnancy is very important to reduce maternal morbidity and mortality.

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Background: A variety of endocrine disorders can complicate pregnancy. Diabetes insipidus although uncommon, may have devastating effect on pregnancy outcome, if unrecognized and untreated. The etiology of diabetes insipidus is often unknown, many cases are likely autoimmune, with lymphocytic infiltration of the posterior pituitary gland. Massive polyuria, caused by failure of the renal tubular concentrating mechanism, and dilute urine, with a specific gravity 1.005, are characteristic of diabetes insipidus. The diagnosis of diabetes insipidus relies on the finding of continued polyuria and relative urinary hyposmolarity when water is restricted. Most women require increased doses Desmopressin Acetate during pregnancy because of an increased metabolic clearance rate stimulated by placental Vasopressinase. By this same mechanism, subclinical diabetes insipidus may become symptomatic during pregnancy. Transient diabetes insipidus is associated with acute fatty liver and HELLP syndrome as well as twin gestation. Increased placental Vasopressinase activity, along with insufficient liver degradation in HELLP syndrome and acute fatty liver, may unmask this condition. Diabetes insipidus in pregnancy is rare. The disease results from inadequate or absent antidiuretic hormone (vasopressin) production by the posterior pituitary gland. The increased glomerular filtration rate seen in pregnancy may increase the requirement for antidiuretic hormone. Case presentation: We present a 39 years old woman, gravida3 para3, was admitted to Akbarabadi Teaching Hospital in september 2013. She was admitted due to polyuria, malaise, thirst with slight fever, six days after normal vaginal delivery. The urine volume was 8 lit/day and the specific gravity (S.G.) of the urine was 1.010. The urine osmolarity was lower than the plasma osmolarity. Electrolyte serum examination showed hypernatremia. The patient received 5 µg/day of synthetic vasopressin, in the form of l- deamino-8-Darginine vasopressin (DDAVP). This drug was given as intranasal spray in doses 0.25 mg twice daily. Plasma electrolytes and fluid status monitored carefully with initiation of therapy. DDAVP was used because it was not degraded by vasopressinase. Treatment was continuing, when the symptoms of central diabetes insipidus resolve and urinary concentrating ability was preferred. Maximum urinary osmolality over the next 11 hours was assessed, 730 mosm/kg was considered normal. Conclusion: Close attention to electrolyte and fluid balance is important in the postpartum period. The symptoms of transient vasopressin-resistant diabetes insipidus resolve in few days to a few weeks after vaginal delivery or when hepatic function returns to normal.