Occasion Title Purpose

: Case Report : Diagnosis and Management of Meningioma in Frontal Lobe : Discuss about Diagnosis and Management of Meningioma in Frontal Lobe : Thursday / November 29th 2012 : dr. Restu Susanti : Prof. dr. Basjiruddin A, Sp.S (K) : Prof. DR. dr. Darwin Amir, Sp.S(K) : dr. Dherma Putra and dr. Ishlahuddin Ibnu Amin

Day and Date Presentant Advisor Moderator Opponent

ABSTRACT Meningioma is a benign intracranial tumor that can cause focal neurologic and neuropsychological deficits. Induced abnormalities depending on the location of existing lesions. Treatment can be given in the form of operative and radiotherapy. This disease has a good prognosis. Reported cases of women 48 years with progressive headache, weakness of right limbs and neurobehavior function changes. Right hemiparesis and papil edema obtained from physical examination and neurobehavior function changes obtained from the value of mini mental state examination 4. The results of a CT scan with contrast there was the frontal lobe meningioma. The patient was treated with anti-edema and metabolic activators. Then proceed with the action craniotomy and microsurgery as well as anatomical pathology examination of the results of atypical meningioma. After the therapeutic action, no headache, there are motor repair and better neurobehavior function.

INTRODUCTION Meningiomas are tumors arising from the arachnoidal coverings of the brain. Found as the second most common central nervous system tumor, accounting for approximately 20% of all primary adult intracranial tumors. The vast majority of meningiomas occur in patients between 50 and 60 years of age, with a twofold higher incidence in women. The biological behavior of meningiomas is one of continued growth, ultimately leading to compression of neuronal structures. The treatment of choice is surgery, which is

frequently successful in treating these tumors.1,2 Patient complain of increasingly severe headache, right limb hemiparalysis and behavioral disorder.

CASE ILUSTRATION A fourty eight-year-old female patient was admitted to neurology department of Dr.M Djamil hospital Padang on June 26th 2012, with :

THE MAIN COMPLAINT: Increasingly severe headache HISTORY OF PRESENT ILLNESS Patient complain of headache that become increasing intense since last 6 month. This headache very severe felt in all parts of the head and most of all day. Initially her headache is not interfere her daily activities and than gradually her activity be came discrupted as well. Finally she could not work anymore. Patients appear to have a defect in appearance daily in her behavior, she seem depressed and often forgotten of time, place and other people of the former familiar. She even forgot her name itself or something new that just happened. Patient also difficult to do her daily job such as cooking, washing and unable to perform her job as a lecturer. Patient had weakness of the right limb, but still able to walk on her own, but by the way she found it difficult to wear slipper or buttoning her blouse. She appeared with a face that is not symmetrical. Since 3 days ago patient contact worse, coldnot doing communication well. Patient couldnot answer and doing everything people ask to her. PAST ILLNESS HISTORY Never been sick like this before History of hypertension is not known She had ovarian cyst surgery in 2008

HISTORY OF FAMILY ILLNESS No family members were sick like this. SOCIAL ECONOMIC BACKGROUND A lecturer in STIKES. The highest education S1, but since 2 years before is not working anymore. Married but has no children

PHYSICAL EXAMINATION General Condition Awareness Blood Pressure Pulse Temperature Breathing frequency : Moderately ill : Alert : 140/90 mmHg : 82 x/minute, regularly : 36,5o c : 16 x/minute

Internal Examination Eye Lymph nodes Neck Lungs : Conjunctiva was not anemic and sclera was not icteric : No enlargement : JVP 5-2 cm H2O. Bruit carotid (-) :Symmetric static and dynamic, palpation is normal, sonor, vesicular, ronchi (-/-), wheezing (-/-) Heart : Ictus is not visible, palpable 1 finger medial LMCS ICS V, Sinus rhytm, metallic sound, HR = 82X/minutes

Neurological Examination GCS : E4M6V5 There are no signs of nuchael rigidity, neither brudzinski I and II. No kernig sign found. No sign of increasing intracranial pressure. Cranial Nerves Nerve I Nerve II : Normal : Visual actuity and visual field could not be examine.

Opthalmoscophy examination showed: papil side is not clear, hyperemis, cupping (+), aa : vv = 1 : 3, av.crossing (-) Impression: Oedema papil ocular dekstra and sinistra + Fundus hypertension KW Ist grade Nerve III,IV,VI : isocor pupil, 3mm/3mm, ligh reflex movement of the eye balls were normal NV N VII N VIII N IX NX N XI N XII : Corneal reflex +/+ : facial assymmetry, lagoftalmus (-), right nasolabial fold flatter then the left side : Hearing function is normal : vomiting reflex (+) : Symmetrical pharyngeal, uvula is in the middle : normal : Deviation of tongue (+),atrophy (-), fasciculation (-) (+/+), Ortho position,

Motoric function : Right Superior extremities Muscle strength Tone, trophy Inferior extremities Muscle strength Tone, trophy Sensoric function Autonomic function Physiological Reflex : Right Biceps Triceps KPR APR ++ ++ ++ ++ Left ++ ++ ++ ++ Active 4 +4 +4 + Eutonic, eutrophy Active 4 +4 +4 + Eutonic, eutrophy Left Active 555 Eutonic, eutrophy Active 555 Eutonic, eutrophy

: normal exteroceptive and proprioceptive function : normal

Pathological reflex : Hoffman tromner Babinsky group -

Laboratory Finding Hb : 14.1 g/dL WBCs: 10.200 /mm3 Ht: 44 % RBG : 104 mg/dL Ureum: 19 mg/dL Creatinin: 0,7 mg/dL Sodium:139 mmol/L Potassium: 3,7 mmol/L

ECG: Sinus rhythm, HR: 82 x/min, ST elevation (-),ST depression (-), T inverted (-), SV1 + RV5 <35 mm Impression: within normal limits

Regression Reflex: Glabela reflex (+), Sucking reflex (-), Snout (-), palmomental (-), corneomandibular (-), kakitonik (-) MMSE: 4 Orientation: 0 Recal: 1 Registration: 3 Language: 0 Attention and calculation: 0 Construction: 0

MoCA-Ina: can not be perform

DIAGNOSIS Working Diagnosis : Severe headache with right limb hemiparalysis and parese seventh cranial nerve central type Topical Diagnosis Etiology Diagnosis Secondary Diagnosis MANAGEMENT: Diet low salt II, 1800 kcal Patient put on medication of : Asetazolamide 250 mg qid (po) KSR 500 mg bid (po) : Intracranial : Space occupying lesion suspected tumor : Hypertension stage I

FURTHER INVESTIGATION : 1. Complete blood count: cell blood count, platelets, SGOT, SGPT, total

cholesterol, HDL, LDL, triglycerides, ureum, creatinine, uric acid, electrolytes (Na, K, Cl), total protein, albumin, globuline 2. Tumor markers : CEA, AFP 3. Skull x-ray sella tursika centration 4. Chest x ray 5. Brain CT scan with contrast 6. Consult to neurobehavior specialist

FOLLOW UP 2th day of hospitalization Subjective : conscious, contacts have not been adequate, no headache or vomite Objective : cm uncooperative, Bp: 140/90, Pulse rate: 72 x / minute, breath: 18 x / min, temperature: 37 0C Neurologic exam : GCS E4M6V4, signs of increased ICP (-). Paresis of right seventh and twelveth nerves central type. Motoric: tones and trophy was normal, upper and lower extremity strength of right side 4+ 4+ 4+ Laboratory findings Hb: 13,1 g/dL Leucosyte: 11.800 /mm3 Ht: 40,7 % Thrombosyte: 222.000 /mm3 LDL: 144 mg/dl Sodium:145mmol/L Total protein : 8 mg/dl Uric Acid : 7,5 mg/dL Ureum : 27,1 mg/dL Creatinin: 0,7 mg/dL Total cholesterol: 212 mg/dl Triglycerides: 101 mg/dl Potassium: 3,6 mmol/L Cloride:106 mmol/L Albumin: 4,4 mg/dl Globuline:3,6 mg/dl SGOT: 26 mg/dL SGPT: 16 mg/dL AFP: 1,79 IU/ml HDL: 47 mg/dl

Skull X-ray (Sella Tursica Few) :

Visible destruction of the sphenoid of sella anterior and posterior and calcification of the calvaria. Suggested : Mass on the sella turcica Suggesiton : brain CT scan with contras

Chest X-Ray PA position :

Heart and pulmonary within normal lmit

Brain CT scan with contras:

Impression : Hiperdens lesions, inhomogen large, well defined, irregular edges, accompanied by calcification, edema, and midline shift perifokal to the right lateral ventricle with obliteration and III in the left temporoparietal lesions appear fronto extends to sella turcica (Chiasma). Widened sulci narrowed gyri. Ventricular system and sisterna not widen. Pons, both CPA and cerebellum normally. Impression: suggestive of astrositoma high grade DD/ meningioma Assesment: frontal lobe meningioma Management: Diet low salt II, 1800 kcal Patient put on medication of : Asetazolamide 250 mg qid (po) KSR 500 mg bid (po) Planning: consult to neurosurgery spesialist 3th day of hospitalization Subjective : conscious, contacts have not been adequate, no headache or vomite Objective : cm uncooperative, Bp: 120/70, Pulse rate: 78 x / minute, breath: 18 x / min, temperature: 36,9 0C

Neurologic exam : GCS E4M6V4, signs of increased ICP (-). Paresis of right seventh and twelveth nerves central type. Motoric: tones and trophy was normal, upper and lower extremity strength of right side 4+ 4+ 4+ Consultation to neurosurgery specialist recommended that the diagnosis as a meningioma and would be performed: tumor removal (after informed consent) Plan: Tumor removal if the patient and family agree. If agreed preparation of operation: Consult to Internal department and ICU, blood preparations: 4 PRC, 4 WB. Assesment: left frontal meningioma Management: Diet MB RG II 1800 kcal Medication given : Asetazolamide 4 x 250 mg (po) KSR 2 x 500 mg (po)

4th day of hospitalized Subjective : conscious, contacts have not been adequate, no headache or vomite Objective : cm uncooperative, Bp: 130/80, Pulse rate: 82 x / minute, breath: 22x / min, temperature: 36,8 0C Neurologic exam : GCS E4M6V4, signs of increased ICP (-). Paresis of right seventh and twelveth nerves central type. Motoric: tones and trophy was normal, upper and lower extremity strength of right side 4+ 4+ 4+ Assesment: left frontal meningioma Management: Diet low salt II, 1800 kcal Patient put on medication of : Asetazolamide 250 mg qid (po) Informed consent to families Results: Family aggree for tumor removal KSR 500 mg bid (po)

Planning: Consult to internal departement Check the hemostatic physiology Preparation of blood transfussion

Laboratory results: PT: 11.3 seconds APTT: 27.9 seconds INR: 1 7th day of hospitalized Internal specialist recommended : operating tolerances : cardiovascular and pulmonary risk mild, metabolic and coagulation either. Suggestion: post-op stabilisation in ICU Plan: waiting for neurosurgical operations scheduling 8th day of hospitalized Result of Neurobehavior spaecialist Consultation: Symptoms of neuropsychological deficits were found: often forget this since 6 months Attention: distracted Orientation: impaired Verbal sense: good

Language functions: spontaneous talk disturbed (not smooth) Naming: impaired Writing: impaired Repetition: impaired Memory function: impaired Visuospatial functions: impaired Read: impaired

Executive functions: impaired MMSE: 11 Orientation: 2 Recal: 0

Registration: 3 Language: 6

Attention and calculation: 0 Construction: 0

MoCA-Ina: can not be perform Conclusion:

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Supposed that all modalities of neurobehavior examination in these patients showed that the function function of attention, language, memory and executive functions and visuospatial were impaired. 16th day of hospitalized at 13:50 am at afternoon: Subjective : Patient complained of headache and vomite 3x. Objective : impaired conciousness, BP: 120/80, pulse rate: 104 x / minute, respiratory rate: 18 x / min, temperature: 36.9 0C Laboratory revealed : Hb: 13 g / dl; Leucosyte: 16 600/mm3; Ht: 41% Platelets: 212 000/mm3; Potassium: 3.5 mmol / L; Sodium: 141 mmol / L; Clorida: 107 mmol / L Medication as follow: IVFD RL 12 hours / kolf Diet low salt II, 1800 kcal Asetazolamide 250 mg qid (po) KSR 500 mg bid (po)

Dexametason 10 mg (iv) qid (tappering off) Ranitidine 50 mg (iv) bid Ceftriaxon 1 gram (iv) bid Operation had to be delayed because of waiting for theatre as a reason. 17th day of hospitalized at 7:30 pm: Subjective : conscious, contacts have not been adequate, no headache or vomite Objective : cm uncooperative, Bp: 110/80, Pulse rate: 82 x / minute, breath: 22 x / min, temperature: 36,8 0C Neurologic exam : GCS E4M6V4, signs of increased ICP (-). Paresis of right seventh and twelveth nerves central type. Motoric: tones and trophy was normal, upper and lower extremity strength of right side 4+ 4+ 4+ Assesment: tumor removal ec left frontal lobe meningioma

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Medication as follow: IVFD RL 12 hours / kolf Diet low salt II, 1800 kcal Asetazolamide 250 mg qid (po) KSR 500 mg bid (po)

Dexametason 10 mg (iv) qid (tappering off) Ranitidine 50 mg (iv) bid Ceftriaxon 1 gram (iv) bid At 09.00 am 13.30 am : was performed craniotomy and micro surgery Post craniotomy: Treat A-B-C-D in the ICU Medication : antibiotic, anticonvulsant and analgesic Open the drain > 48 hours Open sewing on 8th post operation Pathological anatomi laboratory Wound and after wound dressing

July 20th 2012 Pathological diagnosis is transitional meningioma August 8th 2012 (one month after craniectomy) Patient complain that her right eye did not function. Result of neurobehavior examination: MMSE: 26 Orientation: 9 Recal: 2 MoCA-Ina: 13 Registration: 3 Language: 7 Attention and calculation: 5 Construction: 0

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September 3rd 2012 (two month after craniectomy) Result of Neurobehavior spaecialist Consultation: Attention: good Language functions: good Read: good Orientation: good Naming: good Writing: good Verbal sense: good Repetition: good Memory function: good

Executive functions: impaired MMSE: 28 Orientation: 9 Recal: 2 MoCA-Ina: 26

Visuospatial functions: good

Registration: 3 Language: 8

Attention and calculation: 5 Construction: 1

Conclution : neurobehavior function is more better than before doing craniectomy.

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DISCUSSION Meningiomas usually benign, slow growing and not cancerous. Symptoms appear gradually and vary depending on the location. Reported a 48 years old female patient with a chief complaint of severe headache were more increasingly accompanied by a weakness of the left limb and deterioration in behavior. From the physical examination found paresis seventh cranial nerves dextra central type and MMSE score was 4. Based on anamnesis and physical examination patients diagnosed with space occupying lession suspected intracranial tumor.1,3 Traction headache is a increasingly severe headache that be happened in patient in space occupying lesion like tumor. Patient complained that the headache couldnot therapy by the analgetic, because this symptom they couldnot done their daily activity. Intracranial tumor could gave more symptom and sign like hemiparalysis, deterioration of neurobehavior and sometime uncioussness.4 After doing Brain CT scan with contrast the results is suggestive of high grade astrositoma (radiology results) that differential diagnose with left frontal lobe meningioma. Here means the etiology of focal neurology deficit that have was meningioma. Meningiomas occur primarily at the base of the skull, in the parasellar regions, and over the cerebral convexities. Meningiomas are not strictly brain tumors, since they arise from meningothelial cells that form the external membranous covering of the brain. Thus, symptoms and signs directly reflect the location of the tumor. Most meningiomas are slow growing and are not associated with substantial underlying brain edema; they cause symptoms by the compression of adjacent neural structures. Patients with tumors of the hemispheric convexities often present with a seizure or progressive hemiparesis. Patients with skull-based lesions typically present with cranial neuropathy, whereas meningiomas in any location may cause headache. 4,5,6,7 . Meningiomas occur more frequently in women, with a female-to-male ratio of 3:2 or even 2:1 in some series. Incidence of meningioma is 18% from all brain tumor. Focal deficits, which are applicable to the existing lesions. The cause of meningioma was suspected as associated with hormonal based on the history that she did not has no children a suffered from ovarian cyst.4,7,8

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We know more about the causes of meningiomas than about most brain tumors. There are at least four factors that seem to be important in their development: genes, radiation therapy, hormone receptors, and perhaps environmental factors.5,9 Radiation has a very important role in meningioma formation. Approximately 5% of all meningiomas are radiation induced. An intriguing aspect of meningiomas is their relation to sex. They are known to occur more often in women than in men. Increase in size during pregnancy; they have an increased incidence in patient with carcinoma of breast; and their cells have progesterone and estrogen receptors, although their role is not known. Women had more expression of the progesterone receptor than a men. Meningiomas secrete parathormone-related peptide, which may be responsible for their classification. Prolactin receptor is expressed in meningiomas. 5,7,9 The symptom from meningioma depend on the location of the tumor. 20% meningioma located in frontal lobes, that gave the frontal lobe syndromes. Frontal lobe tumor made deterioration of behavior and personality in 90% cases.7 Frontal lobe is the biggest lobe from our brain, comprising almost one-third of the total cortical surface area and related to behavior aspect . Frontal lobe syndrome is behavioral changes, emotion, and personality, caused by frontal lobe damage . Several caused could make frontal lobe syndrome like a traumatic brain injury, tumours, fronto temporal dementia, or post surgery aneurism.7,10,11,12 The frontal lobes control many of the brains activities including attention, abstract thought, problem solving, reasoning, judgment, initiative, inhibition, memory, parts of speech, moods, major body movements, and bowel and bladder control.7 The frontal lobes give many of the uniquely human characteristics of behavior, and diseases of the frontal lobe are among the most dramatic in neuropsychiatry. Frontal lobes are divided into the motor cortex adjacent to the Rolandic fissure, the premotor cortex anterior to the motor cortex, and the prefrontal cortex comprising the region anterior to the premotor areas. Contralateral weakness, brisk reflexes, and Babinski signs occur with lesions of the motor strip; Brocas aphasia and executive aprosodia follow lesions of the left and right premotor areas, respectively; and alterations in cognition, demeanor, and mood are associated with prefrontal dysfunction.10,11,12 Frontal lobe syndromes characterized by deterioration in behavior and personality characteristic features are:10

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Table 1 : Frontal Lobe Symptoms and Their Assesment

Clinical manifestation have various type, but based on unable to manage behavioral. In this patient characteristic of frontal lobe syndrome that the patient has are reduced verbal fluency, non verbal fluency, poor judgemet, poor response inhibion, poor memory organiation, reduced devided attention and contralateral hemiparesis. Deterioration of behavior and motoric deficits that happened in this patient based on the the location that contributed in this tumor, prefrontal and primary motoric cortexs.7,10 Therapy for this syndrome stress on its underlying desease, family councelling, and surgery. Treatment of brain tumor depend on the location and type of the tumor. Corticosteroid used for reduce the vasogenic oedema and controlled the intracranial pressure. Treatment options for meningiomas include observation, surgery and

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radiation therapy. At the Brigham and Womens Hospital, make the statement was deciding wheter to observe a tumor. The ultimate decision consist of symptoms,age and imaging appearance, morbidity of surgery or radiation, patient preference and need for definitive diagnosis.4,9,10 This patient then consulted for the diagnosis of neurological surgery and enforced by the plan meningioma tumor removal. Meningioma operative therapy is the first choice that followed by anatomical pathology examination. In these patients performed a craniotomy with microsurgery is a major choice in the treatment of meningioma with minimal recurrence. Microsurgical technique which can significantly increase the radical surgical intervention for brain meningiomas, resulting in lower frequency of recidives and reducing number of complications. Thus, the use of microsurgical technique at the stages of resection of meningiomas with the consequent coagulation of the matrix increases the radicality of surgical treatment, reduces the risk of recidives and continued tumor growth.5,9,13,14 Pathologic exam of this meningioma who had surgery showed as transitional meningiomas. These common tumours feature the coexistence of meningothelial and fibrous patterns as well as transitions between these patters. Based on World Health Classification of Brain Tumors, transitional meningioma was Grade I, the benign group (85-90%), have no prognostic significance but are merely descriptions of different histology.5,9,13 After doing craniectomy and microsurgery the condition of patient is more better, the symptom of the right hemiparalysis and improved the behavior function. Had been doing the neurobehavioral examination follow up in this patient, that recognized that the focal neurologic and neuropsychological deficit that happened in this patient was the effect of the mass in frontal lobe, meningioma.

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CONCLUSION

Meningioma is a primary brain tumor, more common in women. symptoms of focal neurological deficits caused depends on the location of the lesion. Frontal lobe is the biggest lobe from our brain, comprising almost one-third of the total cortical surface area and related to behavior aspect. Frontal lobes meningioma caused contralateral hemiparalisis and behavior deterioration. Frontal lobe syndrome is behavioral changes, emotion, and personality. Meningioma is a tumor that is operabel and radiosensitif. Focal neurologic and neuropsychological deficit that happened in this patient was the effect of the mass in frontal lobe.

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REFERENCE

1. Kaye Andrew H, Edward R Laws. Brain Tumors an Encyclopedic Approach, Third Edition. Saunders Elsevier. China. 2012. Page 171 2. Pasztor Emil. Concise Neurosurgery for general practitioners and students. National Institute of Neurosurgery. Budapest, Hungary. Page 55 3. Sadewo Wismaji. Sinopsis Ilmu Bedah Saraf. Cetakan pertama. Departemen Bedah Saraf FKUI/RSCM. Jakarta. 2011. Hal 145 4. Lisa B Angelis. Brain Tumor. N Englaand J med, vol 344, No.2, Januari 2001. www.nejm.org 5. Louis N David, Hiroko Ohgaki, Otmar D Wiestler, et al. WHO Classification Of Tumors Of Central Nervus System 4th Ed. International Agency for Research and Cancer Lyon. 2007. 164-172 6. Ahluwalia Manmeet et al. Brain Tumor in Outcomes Neuro 2010. Cleveland Clinic 2010. www.ClevelandClinic.org 7. National Brain Tumor Foundation. Essential Guide of Brain tumor. Page 1012,32. www.braintumor.org 8. American Brain Tumor Association. Focusing on Tumor Meningioma. Page 116. 2006. www.abta.org 9. Black et al. Meningioma : Science and Surgery. Clinical Neurosurgery. Volume 54, 2007:91-99 10. Cumming L Jeffrey and Michael R Trimble. Concise Guide to Neuropsychiatry and Behavioral Neurology, 2nd Ed . American Psychiatric Publishing Inc. Washington DC. 2005. Page 71-85 11. Rowe AD et al. Theory of Mind Impairments and Their Relationship to Executive Functioning Following Frontal Lobe Exicisions. Brain. 2010;124:600616 12. Bor Daniel et al. Frontal lobe involvement in spatial span: Converging studies of normal and impaired function. Neuropsychologia 44 (2006) 229237 13. Minniti Giuseppe, Maurizio Amichetti and Riccardo Maurizi Enrici. Radiotherapy And Radiosurgery For Benign Skull Base Meningiomas. Radiation Oncology 2009, 4:42, http://www.ro-journal.com/content/4/1/42 14. Alimov Djamshidjon. Comparative Characteristics Of Surgical Treatment With Subsequent Radiotherapy For Brain Typical And Atypical Meningiomas. Medical And Health Science Journal, Volume 7, 2011, Pp. 44-48

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