Epidural hematoma in Fahr s disease FAHR S DISEASE ACCOMPANIED BY EPIDURAL HEMATOMA: A CASE REPORT

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1 138 FAHR S DISEASE ACCOMPANIED BY EPIDURAL HEMATOMA: A CASE REPORT Kara H 1*, Bayir A 1, Ak A 1, Akinci M 1, Değirmenci S 1, Alp H 1 1. Selcuk University, Faculty of Medicine, Department of Emergency Medicine, Konya, Turkey Correspondence Dr. Hasan Kara. Selcuk University, Faculty of Medicine, Department of Emergency Medicine, Konya, Turkey hasankara42@gmail.com Kara H, Bayir A, Ak A, Akinci M, Değirmenci S, Alp H. Fahr s disease accompanied by epidural hematoma: a case report. ABSTRACT Fahr s disease (FH), also referred as Bilateral Striopallidodentate Calcinosis is a rare disease characterized by the symmetrical calcifications in basal ganglia, thalamus, cerebellar dentate nucleus and cerebral white matter. Intracranial calcifications may occur due to various reasons. Bilateral symmetrical cerebral calcifications are mostly seen in idiopathic, familial or calcium and parathyroid hormone metabolism disorders. Calcifications are usually revealed in computed tomography (CT) taken for other reasons. CT findings of these cases can be mixed with subarachnoid hemorrhage manifestations in the emergency departments. Acute epidural hematomas are common after traumas and mostly require immediate surgical intervention. They usually occur in the temporoparietal area after a linear fracture. However, chronic epidural hematomas do not have clinical symptoms and may progress with spontaneous resorption and calcifications, especially in children. Therefore, the medical history should be well-evaluated and it should be considered that there might be an underlying endocrine problem and it should be confirmed with imaging methods in the patients admitted to emergency departments with head trauma and had cerebral calcifications. In this case report, a patient taken to the emergency department (ED) with head trauma and Fahr s disease accompanied by acute epidural hematoma was evaluated with the computed tomography in the light of the literature. Key words: Fahr s disease, epidural hematoma, cerebral calcification INTRODUCTION Intracranial calcifications may emerge due to various reasons and occur with a wide variety of symptoms. Hypoparathyroidism, pseudohypoparathyroidism, Fahr s disease, Wilson s disease,mitochondrial encephalopathies, neoplasias, anoxia, carbon monoxide and lead poisoning and radiation therapy are the mean causes of calcification 1. Cerebral calcifications are detected with 0.3% and 1.2% on routine computed tomography examinations. The incidence of bilateral basal ganglia and cerebellar calcifications 138

2 139 increases with age, but most of them do not reveal symptoms. Basal ganglia calcifications are usually idiopathic. Congenital, endocrine, metabolic and toxic reasons, infections, hypoxia and iatrogenic causes are in the etiology. Some of the basal ganglia calcifications are physiologic 2. Acute epidural hematomas are the urgent neurosurgery cases that occur after head traumas requiring hospitalization and surgical intervention. However, chronic epidural hematomas are seen as the case reports in the literature and are rather rare 3. Chronic epidural hematomas are often seen in children and usually there is not a clinical finding. The case showing spontaneous regression or calcifying are reported in the literature. In general, epidural hematomas reveal symptoms by showing a mass effect or leading to an increase in intracranial pressure. Especially temporal region hematomas clinically progress noisy, and they have high mortality and morbidity rate 4. Medical history of the patients with cerebral calcification that may be confused with subarachnoid hemorrhage at emergency service should be well-evaluated and it should be considered that there might be an underlying endocrine problem. CASE REPORT A 46 years-old female patient was referred to our clinic with the complaint of head trauma. There was no local or systemic disease in her history and no characteristic in the familial history either. In the physical and neurological examination, the patient was conscious and cooperative. Pupillary, isocoric direct and indirect light reflexes were taken bilaterally. Cranial nerve examinations were intact. Motor and sensory examinations were normal. Deep tendon reflexes were normoactive bilaterally. No pathological reflex was found. In the laboratory examination, total serum level of calcium was determined as 6.9 mg/dl and the level of parathyroid hormone as 9.35 mg/ml. Full blood, liver, kidney and thyroid function tests were normal. Cranial computed tomography of the patient was ordered since the complaint of severe headache was in the foreground. On CT examination, an image conforming with a marked calcification was monitored in two basal ganglia [caudate nucleus, lentiform nucleus, globus pallidus] and thalamus and a bone fracture in left parietoccipital region and adjacent epidural hematoma were observed. Basing on the current clinic manifestation, laboratory outcomes and radiological imaging, the patient was considered as Epidural hematoma accompanied by Fahr s disease (Figures 1,2 and 3). Our case was consulted with the neurosurgery clinic and taken under treatment to follow-up by the related clinic. DISCUSSION Fahr s disease also referred as Symmetric Striopallidodentate Calcinosis was described first in Calcifications are usually observed bilaterally in Fahr s disease, which is also called familial idiopathic basal ganglia calcification. Striopallidodentate calcifications usually accompany calcium-phosphorus metabolism disorders, however, this is a disease in some cases that also progresses with quite widespread calcifications in the brain parenchyma caused by genetic damages even without abnormality in the serum levels of calcium and parathyroid hormone 5. In some studies, it was established that Striopallidodentate calcifications developed with deposition of the elements such as calcium, iron and copper and proteins followed by the necrosis caused by various toxins 139

3 140 Figure 1. White arrows show the basal ganglia calcification, and the black arrow indicates to the fracture in the left parietal bone and adjacent epidural hematoma. Figure 2. White arrows show basal ganglia calcification, while the black arrow indicates epidural hematoma in the left parietal. 140

4 141 Figure 3. White arrows show the calcifications in both the basal ganglia and thalamus, while the black arrow shows air-containing epidural hematoma in the left parietoccipital. and infectious agents in the neuronal tissue. Personality changes, Parkinson-like manifestations and progressive deterioration of mental and cognitive functions are among the most common clinical symptoms. The most important examination method to establish Fahr s disease is computed brain tomography. These cases may be confused with subarachnoid hemorrhage on the computed tomography in emergency departments. Intracranial calcifications are often seen especially in globus pallidus and in putamen, caudate nucleus, internal capsule, dentate nucleus, thalamus and cerebellum 6. Although Fahr s disease was described long ago, it may be overlooked and occasionally confused with intracranial bleedings since it is not encountered very often. Although sporadic and autosomal recessive forms of this disease are reported, it usually reveals an autosomal dominant transmission 7. Acute epidural hematoma is a clinical picture resulting from the accumulation of blood between skull and dura matter following a linear fracture in the temporal or temporoparietal region due to head trauma or a damage to a. meningea media or its branches. Epidural hematomas are typically of biconvex and lenticular character. Epidural hematoma is established in about 3% of the patients with head traumas admitted to the hospital while late epidural hematoma is accounted for 8 10% of all the epidural hematomas 8. Intracranial calcifications most commonly occur in globus pallidi. However, dense calcifications similar to vascular traces may be obseved in putamen, caudate nucleus, internal capsule, dentate nucleus, thalamus, cerebellumand and cerebral white matter 9. Epidural hematomas may spontaneously recover or progress with calcifications. Establishing the existence, localization, distribution and features of the calcifications is crucial in the radiological diagnosis. In the late periods of development, 141

5 142 fibrotic capsule may be calcified and cause to mineralization ossification. Computed tomography is more sensitive in imaging the deposits than Magnetic Resonance Imaging (MRI) 10. Images of the cranial CT in our cases were consistent with the literature. On the CT examination, there were classifications in the caudate nucleus, lentiform nucleus, globus pallidus and thalamus, and there was a view of air-containing epidural hematoma in the left parietoccipital region. Calcifications in the brain exist mostly before the onset of the symptoms and the initiation of the symptoms is usually in the 4th and 6th decades. Although rare, pediatric cases have been reported in the literature 11. The age of our case was between the 4th and 6th decades and it is consistent with the literature. In the affected persons, chorea-athetotic movements occur in their childhood and progressive mental deficiency occur in adulthood. Although severe calcifications can be monitored on the direct radiographs, CT is the most sensitive radiological method for early diagnosis and revealing the small calcifications. On CT examination, calcification areas are monitored hyper densely in the above-mentioned regions. Although MRI examination is a more sensitive method in imaging neurological pathologies such as developmental anomalies, intracranial tumors and demyelinating disease, CT is much more sensitive in viewing the calcified areas 12. Because the pre-diagnosis was head trauma in our patient, we preferred CT examination first for hemorrhage and fracture. Images of the calcifications on MRI are variable, and they are monitored as hypo or hyperintense in T1 weighed images and as hypointense in the gradient weighed images 13. Although clinical features of the disease are variable; progressive mental damage, tremor, chorea, ataxia, dysarthria, convulsion, clinical picture as in Parkinson, neuro-psychiatric behavior and mental function disorders are the most common clinical symptoms of Fahr s disease. Much as our case had epidural hematoma due to head trauma; Fahr s disease progressing with calcification, Wilson s disease, hyperthyroidism, hypothyroidism, tuberous sclerosis, systemic lupus, erythematosus, motor neuron disease, carbon monoxide poisoning, lead poisoning, toxoplasma and infections such as AIDS should be considered in the differential diagnosis of intracranial hemorrhage 14. We regarded our case as Fahr s disease resulted from idiopathic hypocalcemia accompanied by epidural hematoma, basing on the clinical picture, laboratory and imaging outcomes. In the CT scanning of a patient admitted to the emergency department with head trauma, definitive diagnosis between hemorrhage and calcification should be established according to the localization of the lesions and density measurements. CONCLUSION In conclusion, Fahr s disease should be considered in differential diagnosis in the presence of the calcifications in basal ganglia, cerebellum and supratentorial regions established with cranial CT or MRI examination of the patients with head traumas. COMPETING INTERESTS The authors declare that they have no competing interests. 142

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