A 10-year-old Saudi girl was referred from a local hospital with right side weakness, which was of sudden onset, started in the right lower limb, and 2 days later involved the right upper limb. She had a history of 4 months of pallor, and 2 weeks skin bruising prior to the onset. It was not associated with facial weakness, headache, fever, convulsion, visual disturbance, or loss of consciousness, and no history of preceding head trauma. She had a similar attack of left side weakness 5 months earlier, which resolved spontaneously without medical evaluation at a local hospital. Her mother noticed that she is shorter than her other siblings. Her immunization was completed, and her physical, and cognitive development was normal. Her parents were first degree cousin, and had 6 other siblings. Her physical examination revealed a well looking girl, with pallor and skin bruising on lower limbs, with 2x3cm scattered café au lait spots on the back, and inguinal region. Her weight was 25 kg, below 5th centile for her age, and her height was 118 cm, below the 3rd centile for her age. She had right hemiparesis, there were no hepatosplenomegaly, and the examination of other systems were unremarkable.\nHer investigations showed white blood cell count 2600/mm3 (normal value [NV] - 4000-10500/mm3), neutrophil count 500/mm3 (NV - 3000-5000/mm3), hemoglobin 6.3 g/dl (NV - 12.5-16.1 g/dl), and platelet count 33000/mm3 (NV - 150-400/mm3). Her liver and renal profile were within normal limits. Bone marrow aspiration showed hypocellular marrow. Chromosomal analysis confirmed chromosomal breakage. Brain MRI showed a left frontal focal area of acute ischemic infarction, and an area of right parietal periventricular white matter old infarction, with multiple small white matter lacunar infarcts (). The brain MRA showed poor arterial circulation, and a narrow distal internal carotid artery with puff of smoke sign (arrow) on the right side suggestive of moyamoya disease (). The diagnosis was confirmed as FA, with association of moyamoya disease, presenting with right hemiplegia. She was managed initially by supportive care in the form of a blood transfusion, and physical therapy for hemiplegia, later she underwent partial match related donor stem cell transplantation (SCT), but unfortunately, she died after 3 months from post SCT complications.
A 12-year-old male patient reported to the Department of Pedodontics and Preventive Dentistry with a chief complaint of broken tooth in the upper front tooth region due to trauma, 1 week back. The patient gave a history of prediagnosed case of MMD with hemiplegia of the right side.\nAt the age of 1½ years, the patient suffered from pulmonary tuberculosis and completed anti-tuberculosis treatment for the same. At the age of 4 years, the patient experienced sudden onset of pain and weakness in the right lower limb, which was associated with difficulty in holding objects with the right hand and raising the right arm above the head. The MRI of brain showed focal right frontal infarct with left cerebral atrophy and an attenuated appearance of intracanal arteries suggestive of effects of MMD. Symptomatic medical treatment was provided to the patient. The patient had no past dental treatment exposure. There is no related family history.\nThe patient appeared underweight with a lean body type. The general physical examination revealed hemiplegia of the right side, affecting both upper and lower limbs ().\nThe extraoral examination revealed a symmetrical face with competent lips and normal mouth opening. On intraoral examination, permanent dentition was present with Angle's Class I malocclusion. Ellis Class I fracture was present in the upper right central incisor whereas Ellis Class II fracture was present in the upper left central incisor ().\nThe dental treatment plan focused on providing preventive as well as restorative care to the patient while simultaneously providing proactive counseling to both the parents and the patient. Behavior management was an integral part of our management. Oral hygiene measures were instructed to the patient and modified angled toothbrush with fluoridated toothpaste was prescribed due to the lack of manual dexterity. Along with this, diet modification was done by recommending intake of noncarious dietary food items such as fibrous food, nuts, and fruits. The medical consent was obtained from the pediatrician following which oral prophylaxis and composite restoration of upper central incisors were done ().