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Show THE FIRST JACOBSON LECTURE Daniel M. Jacobson, MD Daniel M. Jacobson, MD completed neurology training at the University of Pittsburgh and neuro-ophthalmology fellowship at the University of Iowa. He joined the staff of the Marshfield Clinic in Marshfield, Wisconsin in the Departments of Neurosciences and Ophthalmology in 1987 with a faculty appointment at the University of Wisconsin. During a 16-year period at the Marshfield Clinic, he cared for thousands of patients and authored more than 50 scientific manuscripts in the field of neuro-ophthalmology. He was honored with numerous teaching and research awards and recognized for his ability to apply basic science principles to the investigation of the most pressing clinical issues. The Marshfield Clinic Foundation has established a memorial fund in his name. In recognition of the profound impact he has had on the field of neuro-ophthalmology, the North American Neuro-Ophthalmology Society (NANOS) has established a lecture to be presented each year at the NANOS meeting. Familial Idiopathic Intracranial Hypertension James J. Corbett, MD Background: Case reports of familial idiopathic intracranial hypertension (IIH) have appeared spo-radically and infrequently. Methods: We reviewed the medical records of all patients with IIH seen at our institution to identify the number of familial cases. Results: Out of a cohort of 237 patients with IIH, we identified 27 members (25 women and 2 men) from 11 families, with IIH usually self-reported or reported by the index case. In 7 of the 11 families, the relationship was parent to child; in 4, it was sibling. Obesity was present in 85% of the family members. Conclusions: Familial IIH appears to be more com-mon than reported previously. A systematic evalua-tion of first-degree relatives may help to identify more cases. A study of the patterns of inheritance and associated co-morbidities may result in better un-derstanding of the genetic issues with this disorder. (J Neuro-Ophthalmol 2008;28:337-347) Idiopathic intracranial hypertension (IIH) is a disorder of raised intracranial pressure (ICP) for which no other cause for the elevated pressure can be identified. The diagnosis of IIH is established by adhering to the Friedman- Jacobson modification of the Dandy criteria (1). The Departments of Neurology and Ophthalmology, University of Mississippi Medical Center, Jackson, Mississippi. Address correspondence to James J. Corbett. MD. Department of Neurology, University of Mississippi Medical Center, Jackson, MS 39216; E-mail: jcorbett@neurology.umsmed.edu reports of Radhakrishnan et al (2) from Libya and Durcan et al (3) from Iowa and Louisiana provide the incidence of IIH. Except for a few large and mostly retrospective studies, information on this condition has been derived typically from single case reports or small case series, which is not surprising, given the low incidence of 0.9 to 3.6 per 100,000 in the general population (2,3). There are few reports of the familial occurrence of IIH (4-21). Before this study, 17 families with IIH had been reported in English (4-13,15) and one in Spanish (14). The purpose of this study was to review the familial cases of IIH in a large single institutional cohort in the state of Mississippi to better characterize the total clinical features of this condition. METHODS The study was approved by the University of Mississippi Medical Center (UMMC) Institutional Review Board. The medical records of all patients with IIH who attended the Neuro-ophthalmology Clinic at UMMC from 1990 to 2007 were reviewed to find patients who had identified a first-degree or second-degree relative with IIH. The diagnosis of IIH was established in the index cases by using the Friedman-Jacobson modification of the Dandy criteria (1). Every effort was made to examine additional family members reported to have IIH and to obtain pertinent medical records. If a physical examination was not possible, then attempts were made to contact the family member to obtain details of the diagnosis. All family members who satisfied the Friedman-Jacobson criteria, including docu-mented opening pressure (OP), were labeled as definite IIH. All other family members without documented OP and/or imaging were labeled as probable IIH. J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 337 J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 Corbett The medical records of the index case and all available family members were reviewed for the following variables: age at onset, body habitus preferably as body mass index (BMI) (22), and associated conditions, includ-ing psychiatric. Papilledema was graded using the Frise´n scale (23). The results of brain CT or MRI were included where available. RESULTS We identified 254 patients with ICP greater than 250 mm water. Of these, 17 patients were excluded because of secondary causes of raised ICP identified by history or physical examination. We included 237 patients in the final analysis. Of these, 219 had papilledema and 18 did not. There were 11 families consisting of 27 members with a family history of IIH (Table 1). Family 1: Mother and 4 Daughters All family members except the youngest daughter (BMI 38 kg/m2) were extremely obese (BMI >40 kg/m2) and had headache and papilledema grade I-II. The cerebrospinal fluid (CSF) opening pressure (OP) was greater than 250 mm water in all except two individuals (OP 220 mm water). The family came to our attention when two of the family members who accompanied the index case (25-year-old daughter) informed us that they were having similar symptoms. The optic disc photographs of this family are presented in Figure 1. Family 2: Heterozygous Twin Sisters Both were symptomatic of IIH at the age of 15 with chronic daily headache, were extremely obese (BMI >40 kg/m2), and had metabolic syndrome. Both had grade II-III papilledema and Chiari I malformations. Although OP was elevated in one (325 mm water), LP in the other was not attempted because of the Chiari malformation. Interest-ingly, bipolar disorder was diagnosed in both sisters. Family 3: Two Sisters Both had chronic headache and were obese (BMI >30 kg/m2). The older one was referred by an optometrist who had incidentally discovered papilledema, and she revealed that she was the older sister of the index case who was an established patient. They had grade III papilledema and grade I papilledema, respectively. OP was 340 mm water in one and ‘‘elevated'' per radiologic findings in the other. 338 Family 4: Mother and Two Daughters The mother was identified at our clinic at age 59 and had received the diagnosis of IIH at age 31 elsewhere with elevated CSF pressure. She subsequently underwent multiple CSF shunt procedures. Despite these, she became legally blind. She recently died of respiratory complications related to extreme obesity (BMI >60 kg/m2). IIH was diagnosed in both daughters at age 15. Both were extremely obese (BMI >40 kg/m2) and complained of chronic headaches and transient visual obscurations (TVO). One of the daughters had no papilledema and no formal OP although the radiologist believed the OP was high, and the other daughter had low-grade papilledema. All three family members had a radiographic empty sella turcica, and both daughters had a psychotic disorder. Family 5: Mother and Daughter The index case was the daughter who presented at age 21 and was obese (BMI 37 kg/m2) with headache and grade III papilledema. OP was 400 mm water, and she had a radiographically empty sella turcica. She reported that her mother had also been found to have a radiographically empty sella turcica and was subsequently found to have elevated CSF pressure. Curiously, her mother had initially presented to an otolaryngologist with CSF rhinorrhea. Family 6: Mother and Two Daughters The index case was the older sister who presented at age 39 with headache, tinnitus, and blurred vision. She had a BMI of 44 kg/m2and grade V papilledema. OP was 440 mm water. IIH had been diagnosed in her 52-year-old mother in her 30s and had been treated with acetazolamide. The mother's medical records could not be located. IIH was also diagnosed by a neuro-ophthalmologist in the younger sister, a nurse in Chicago, in her 30s. We spoke to her by telephone, but she could not obtain her medical records. Family 7: Mother and Daughter The index case was the daughter with the diagnosis of IIH at age 20. She presented with headache and bilateral grade I papilledema. OP was 390 mm water. She had a radiographically empty sella turcica. She mentioned that her mother also had headache and was told that she had a radiographically empty sella turcica. q 2008 Lippincott Williams & Wilkins TABLE 1. Family 1 2 3 4 5 6 Familial cases of idiopathic intracranial hypertension identified at the Relationships of Family Members with IIH Mother and 4 daughters Heterozygous twin sisters Sisters Mother and 2 daughters Mother and daughter 2 Daughters and mother Age at Diagnosis (years) 25 47 16 18 13 15 15 26 28 31 16 15 21 47 39 30 30 Habitus (BMI†) Obese (47) Obese (45) Obese (43) Obese (46) Obese (38) Obese (53) Obese (45) Obese (42) Obese (55) Obese (61) Obese (41) (62) Obese (38) Obese Obese (44) Obese Obese Presenting Symptom Headache Headache Headache Headache Headache Headache Headache Headache Incidental Headache Headache Headache Headache CSF rhinorrhea Headache NR NR University of Mississippi Medical Center between 1990 and 2007 Lumbar Puncture Opening Pressure (mm water) 420 220 270 220 290 325 Dry tap ‘‘E3le4v0ated'' 420 ‘‘Elevated'' ‘‘Elevated'' 400mm ‘‘Elevated'' 440 NR NR Brain MRI/CT Findings Partially empty sella Normal Normal Normal Normal Chiari I malformation Chiari I malformation Normal NR Empty sella Empty sella Empty sella Normal NR NR NR NR Papilledema description or Frisen Grade I II I II III II III I Moderate 0 1 III NR Optic disc pallor NR NR Associated Features - - - -- Manic psychosis Depression - - Diabetes mellitus Psychosis, diabetes mellitus Psychosis, diabetes mellitus - - - Diagnosed in Chicago Diagnosed in North Carolina (Continued on next page) TABLE 1. (Continued) Relationships of Family Members Family with IIH Age at Diagnosis (years) Habitus (BMI†) Presenting Symptom Lumbar Puncture Opening Pressure (mm water) Brain MRI/CT Findings Papilledema description or Frisen Grade Associated Features 10 Daughter and mother Second cousins (male) Second cousin (female) Mother and daughter 20 50 25 30 51 40 38 40 Obese (34) Obese Muscular (35) Thin Obese (43) NR Obese Obese Headache Headache Transient visual obscuration NR Headache NR Headache NR 240, 320 NR 400 NR 450 NR 410 NR Empty sella I Empty sella NR Normal III NR Normal NR NR NR NR III NR BL NR 11 Mother and daughter 61 30 Obese (50) Obese (36) Headache Headache 290 270 Empty sella II Empty sella 0 BMI, body mass index; NR, not recorded; IIH, idiopathic intracranial hypertension. Tetracycline use Now has chronic headache Sister with chronic HA Diagnosed at outside hospital to have IIH Chronic headache 7 8 9 First Jacobson Lecture J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 FIG. 1. Optic disc photographs of Family 1. A. Index case (daughter, 25 years old). Papilledema grade I; lumbar puncture (LP) opening pressure (OP) 420 mm water. B. Mother, 47 years old. Papilledema grade II; OP 220 mm water. C. Daughter, 16 years old. Papilledema grade I; OP 270 mm water. D. Daughter, 18 years old. Papilledema grade I; OP 220 mm water. E. Daughter, 13 years old. Papilledema grade I; OP 290 mm water. Family 8: Second Cousins The index case was a 25-year-old muscular man who presented with TVO. He had a BMI of 31 kg/m2 and grade III papilledema. His OP was 400 mm water, and he had been treated with acetazolamide. He mentioned that his second cousin, who lived in Hawaii, also had the diagnosis of ‘‘pseudotumor cerebri.'' The patient reported that his cousin was ‘‘thinly built.'' Family 9: Second Cousins The index case was a 51-year-old woman with head-ache and TVO. She was extremely obese (BMI 43 kg/m2) and had grade III papilledema. OP was 450 mm water. She reported that her second cousin also had a diagnosis of IIH and was being treated. No medical records of her cousin's diagnosis were obtainable. Family 10: Mother and Daughter The index case was a 38-year-old woman in whom IIH was diagnosed on the basis of grade III papilledema and an OP of 410 mm water. She had chronic daily headache. She mentioned that her deceased mother had once had a diagnosis of pseudotumor cerebri and had been treated by a neuro-ophthalmologist in Boston. We were unable to obtain medical records. Family 1 1 : Mother and Daughter The index case was the 52-year-old mother who had chronic headache and unilateral papilledema. She was extremely obese (BMI 50 kg/m2). OP was 290 mm water, and she was treated with acetazolamide. Her daughter accompanied her during the visit and reported that she, too, had chronic headache. She was obese (BMI 36 kg/m2) and had an OP of 270 mm water but no papilledema. After LP, she developed radicular pain in both legs and the right arm. Both patients had a radiographically empty sella turcica. SUMMARYOF CASE MATERIAL Of the 11 families in our cohort, we identified 25 women and 2 men. Fourteen patients had definite IIH and 13 had probable IIH. Of the 23 family members for whom we could obtain body habitus details, 21 were obese. Of 21 patients for whom CSF OP was available, 14 had OP greater than 250 mm water, 2 had OP less than 250 mm water, 4 had unconfirmed reports of ‘‘high OP,'' and 1 had a dry tap. Among the 19 patients for whom imaging results 341 TABLE 2. Previously reported cases of familial idiopathic intracranial hypertension Study Relationshipsof Ageat Lumbar Puncture Family Members diagnosis Habitus Opening pressure with IIH (years) (BMI*) (mm water) Imaging Papilledema description or Frisen Grade Comments Buchheit, 1969 (9) Siegel et al, 1972 (4) Venable, 1973 (21) Howe et al, 1973, (7) Rothner et al, 1974 (15) Mikkelsen et al, 1974 (5) Sisters Sister and brother Mother and daughter Sisters Mother and son Homozygous twin brothers 19 24 1.5 2.5 17 18 27 35 38 12 15 15 Obese Not obese Not obese Not obese Obese (28) Obese; recent weight gain (29) Obese Obese: 15-lb weight gain Obese Obese Not obese Not obese 270 400 260, anterior fontanelle bulging ‘‘ENleRvated'' 350 NR NR 280-430 200-300 160-200 NR X-ray, angiogram and pneumoencephalogram normal Widening of cranial sutures on x-ray NR X-ray normal, ‘‘brain scan'' normal Air ventriculogram: ‘‘bulging brain'' Symmetrically dilated ventricles X-ray, Hg197 brain scan normal X-ray, ventriculography normal X-ray normal 3 diopters 3 diopters Normal optic disc NR Optic disc pallor 1-2 diopters ‘‘Bilateral papilledema leading to l optic disc pallor'' ‘‘Moderate and bilateral'' No grade No grade 1-2 diopters 1 Two other sisters normal Vitamin A intoxication IIH diagnosis by historical records (history of multiple LPs and burr holes to relieve symptoms) 8 months postpartum; NLP in 2 days 4 weeks postpartum Vitamin A for psoriasis TABLE 2. (Continued) Study Traviesa et al, 1976 (20) Shapiro et al, 1980 (18) Coffey et al, 1982 (10) Torlai et al, 1989 (19) Relationships of Family Members with IIH Sisters Mother and daughter Sisters Heterozygous twin brothers Age at diagnosis (years) 41 22 28 43 20 19 27 58 58 Habitus (BMI*) Obese Obese Obese Obese Obese Not obese Not obese NR NR Lumbar Puncture Opening pressure (mm water) 530 540 NR 280 550 580 420 450 690 Imaging X-ray: mild enlargement of sella turcica; brain scan, pneumoencephalogram and arteriogram normal X-ray, brain scan, echoencephalogram normal Brain scan, arteriogram normal Empty sella Angiography, ventriculography, pneumoencephalography normal CT normal CT normal CT normal CT normal Papilledema description or Frisen Grade ‘‘Papilledema leading to optic disc pallor'' No grade 4 No grade 2-3 diopters No grade No grade No grade No grade Comments 4 months pregnant Major depressive episode in both sisters, 12 and 8 months before IIH symptoms Presented within 3 months although living in separate geographical locations; clinical picture similar to bilateral choroidal folds and similar clinical course (Continued on next page) TABLE 2. (Continued) Study Relationshipsof Ageat Lumbar Puncture Family Members diagnosis Habitus Opening pressure with IIH (years) (BMI*) (mm water) Imaging Papilledema description or Frisen Grade Comments Johnston et al, 1991 (11) Kharode et al, 1992 (13) Rogel Ortiz et al, 1994 (14) Gardner et al, 1995 (6) Fujiwara et al, 1997 (8) Mother, 2 daughters and 1 son Mother and daughter Father and daughter Heterozygous twin sisters Homozygous twin sisters 33 22 25 17 34 16 43 9 19 19 19 Obese Obese NR NR Obese (33) Obese (34) Obese (31) Obese Obese Obese Not obese ''Elevated'' NR NR ‘‘Elevated'' 465 300 350 355 460 NR 340 CT normal CT normal CT normal Enlarged ventricles with communicating hydrocephalus CT normal CT normal CT head normal CT head, MRI and arteriogram normal MRI brain normal MRI not done Enlarged ventricles, empty sella in both on MRI No grade No grade No grade NR ‘‘Mild'' No grade No grade No grade No grade No grade Bilateral papilledema leading to optic disc pallor within 3 years Tetracycline use CSF rhinorrhea; virtually identical clinical course; nearly simultaneous onset of symptoms in both twins at 12 years; diagnosed with papilledema at 19 years; sister 1 had CSF rhinorrhea at 21 years 19 Not obese 210 TABLE 2. (Continued) Study Salmaggi et al, 1996 (16) Santinelli et al, 1998 (17) Karaman et al, 2003 (12) NR, not recorded; *Where available. Relationships of Family Members with IIH Father and daughter Mother and 2 sons Mother, 2 daughters and 3 cousins Age at diagnosis (years) 51 18 36 14 9 54 36 34 16 17 25 Habitus (BMI*) NR Obese Not obese Not obese Not obese Not obese Obese Obese Obese NR Not obese LP, lumbar puncture; IIH, idiopathic intracranial hypertension Lumbar Puncture Opening pressure (mm water) NR 700 400 350 400 450 Refused LP 500 Refused LP Refused LP Refused LP Imaging Empty sella on CT Empty sella on CT MRI normal MRI normal Enlarged spinal dural sac on MRI Empty sella on MRI Empty sella on MRI Empty sella on MRI MRI normal Empty sella on MRI MRI normal ; NLP, no light perception; CSF, cerebrospinal fluid Papilledema description or Frisen Grade No grade Optic disc pallor No grade No grade No grade 5 1-2 5 1-2 3 1-2 . Comments Another daughter had similar visual symptoms but not examined Recurrences after pregnancy twice (22 years, 26 years) Hyperreflexia with spinal and radicular pain Grandmother died 34 years earlier; had symptoms similar to those of the other family members; 15 family members examined in all J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 Corbett were available, 8 had an empty sella, 2 had a Chiari I malformation, and, 9 had reportedly normal studies. Four patients from 2 families had psychiatric illness. DISCUSSION Considering that without any systematic effort to screen family members of patients with IIH, we incidentally discovered 11 families of IIH in our cohort of 237 cases, the paucity of reported familial cases of IIH seems surprising (Table 2). We rejected 2 reports as unquestionable examples of vitamin A intoxication (4,5). Another study reported IIH subsequent to tetracycline ingestion (6), and two others reported radiographically enlarged ventricles (7,8). After we excluded these 5 reports, we found 13 reported families of IIH comprising 33 individuals (25 women and 8 men) (9-21). There are, however, limitations to most of these reports. Depending on the medical source of the case report, the information regarding the ophthal-mic, neurologic, and general physical examinations and the details of the LP and imaging varied considerably. At least 7 of these case reports antedated computed tomography. In those reports, information was based on ventriculography or pneumoencephalography. Despite these limitations, the earlier reports offer valuable insights. Nine of the 13 reports describe parent-child combinations and four describe sibling combinations (Table 2). These findings are remarkably similar to ours, in which 7 of 11 families had parent-child combinations and four had sibling-sibling combinations. Our findings, together with those reported previously, strongly suggest a dominant inheritance, although the genetics of familial IIH are far from clear. In our series, the age of onset of symptoms was remarkably similar for the index case and the other affected family member or members. This trend was also seen in at least 6 of the families reported previously. Traviesa et al (20) reported non-twin siblings who had the onset of symptoms at a similar age although the symptoms were separated in time. Torlai et al (19) reported heterozygous twin brothers who developed symptoms of IIH within a few months even though they were living in separate cities. Venable (21) reported that the age of onset of IIH was similar in mother and daughter. These case reports hint at a genetic ‘‘clock'' involved in the pathogenesis of familial IIH. Johnston et al (11) reported a family that included a mother and 5 children (4 daughters and 1 son) who were examined for IIH. The mother, 3 daughters, and son had developed papilledema with elevated ICPs, although exact pressure measurements were not documented. Although the son did not meet the modified Dandy criteria, as he had communicating hydrocephalus, all other family members did. The authors hypothesized that communicating hydrocephalus and IIH are likely to be caused by a pathogenetic mechanism responsible for a continuum of disorders of CSF circulation 346 owing to malabsorption of CSF at the arachnoid villi. Thus, the occurrence of hydrocephalus and IIH in the same family appeared to them to be more than a coincidence. The largest extended family report comes from Croatia (12) where 15 members of a family were screened and 7 with possible IIH were identified. Six of 15 members examined had varying Frise´n grades of papilledema. The deceased grand-mother's old hospital records indicated she had symptoms of IIH. CSF OP was elevated in the 2 family members who agreed to undergo a LP, but other family members refused. An empty sella was seen in 4 members of the family. The largest family in our series consisted of 5 members, a mother and all 4 of her daughters (Family 1). All were obese and 4 were extremely obese. OP was elevated in 3 family members and borderline in 2. They all had chronic migraine-like headache. Papilledema was mild (Frise´n grade I or II) in all 5 women, and visual acuity, visual field, and color vision were normal in all. Two families in our cohort had a DSM IV psychiatric condition. Manic psychosis was seen in one sibling and a major depressive disorder was seen in the second sibling in the family of heterozygous twins. Bipolar disorder was diagnosed in another set of siblings whose mother also had a major depressive disorder. Coffey et al (10) described 2 sisters who developed IIH within 2 weeks of resolution of major depression. They hypothesized that the development of IIH is probably related to ‘‘a state of endogenous steroid with-drawal following the normalization of a steroidal abnormality that accompanies depression.'' Other authors (24) have also noted an association between IIH and depression. As in previous reports, our case series has all the limitations of any retrospective series. There was no systematic effort to identify familial cases in all 237 patients of our cohort. Thus, our numbers may substantially underestimate the incidence of familial IIH. In a number of our patients, the family history was obtained from the index patient with no corroborating information from the primary source despite our best efforts. This was especially true when the occurrences of IIH were separated widely in time or the affected family members were dispersed geographically. Furthermore, not all family members had documented LP and/or OP. Patients were often referred for second or third opinions and had previously undergone evaluation at other facilities, where LP had often been performed by radiologists, internists, or emergency physicians and OP had not been recorded. In such cases, we were forced to rely on the patients' history, repeating what they were told during the procedure regarding elevated OP or on their reports of improvement of symptoms after LP. The problem of the lack of OP measurements is compounded when the BMI is markedly elevated and a successful LP frequently requires the use of longer needles and must be done under fluoroscopy. Under those circumstances, the LP is often performed with q 2008 Lippincott Williams & Wilkins First Jacobson Lecture J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 the patient in the prone position. It is not yet known whether the OPs obtained when the patient is lying in the lateral decubitus and prone positions are comparable. There are a number of reasons for under-recognition of familial IIH. The clinical presentation of patients with IIH is varied. Patients with IIH, headache, and papilledema are easily identified. However, IIH in patients without papil-ledema or in patients with papilledema and no headache is often not correctly diagnosed. Of the patients in our original cohort of 237, 7.5% had no papilledema, including at least 2 of the reported family members (Families 4 and 11). Some 11% of the patients were asymptomatic and IIH was only diagnosed when papilledema was incidentally found during a routine eye examination for glasses. Family members may have been symptomatic with papilledema in the past but may now demonstrate only residual signs of earlier optic disc swelling and raised ICP. Unless physicians are looking for these signs, affected family members may remain un-recognized. Occasionally family members recalled being told of a radiographically empty sella turcica, often construed as a sign of chronically raised ICP. Confirmation of raised ICP in this circumstance is often difficult, as potentially affected family members see no reason to undergo a LP. Another practical problem in investigating familial IIH is that families are frequently separated geographically, and it is logistically difficult to personally examine each family member. Finally, examination of a patient at a single point may not be truly representative of the spectrum within which IIH is seen. CSF pressure is variable from hour to hour. In a patient with signs and symptoms of IIH, a single normal OP does not eliminate the diagnosis. Some patients with IIH continue to have raised ICP on LP performed several years after the initial diagnosis despite resolution of papilledema (25). 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