Kathleen B. Digre

Revised diagnostic criteria for the pseudotumor cerebri syndrome in adults and children

The pseudotumor cerebri syndrome (PTCS) may be primary (idiopathic intracranial hypertension) or arise from an identifiable secondary cause. Characterization of typical neuroimaging abnormalities, clarification of normal opening pressure in children, and features distinguishing the syndrome of intracranial hypertension without papilledema from intracranial hypertension with papilledema have furthered our understanding of this disorder. We propose updated diagnostic criteria for PTCS to incorporate advances and insights into the disorder realized over the past 10 years.

A neural mechanism for exacerbation of headache by light

The perception of migraine headache, which is mediated by nociceptive signals transmitted from the cranial dura mater to the brain, is uniquely exacerbated by exposure to light. We found that exacerbation of migraine headache by light is prevalent among blind individuals who maintain non–image-forming photoregulation in the face of massive rod/cone degeneration. Using single-unit recording and neural tract tracing in the rat, we identified dura-sensitive neurons in the posterior thalamus whose activity was distinctly modulated by light and whose axons projected extensively across layers I–V of somatosensory, visual and associative cortices. The cell bodies and dendrites of such dura/light-sensitive neurons were apposed by axons originating from retinal ganglion cells (RGCs), predominantly from intrinsically photosensitive RGCs, the principle conduit of non–image-forming photoregulation. We propose that photoregulation of migraine headache is exerted by a non–image-forming retinal pathway that modulates the activity of dura-sensitive thalamocortical neurons.

Idiopathic intracranial hypertension associated with tetracycline use in fraternal twins Case reports and review

Article abstract-Fraternal twin sisters developed idiopathic intracranial hypertension (IIH) shortly after beginning tetracycline for treatment of acne. We reviewed from the literature 19 familial cases of IIH and 37 cases of IIH associated with tetracycline usage. Among the 37 combined adult and pediatric cases, 26 of 37 had resolution of signs or symptoms of IIH within hours to days of stopping the antibiotic, and rapid recurrence with reinitiation of drug occurred in 4 of 37. We suggest that these cases may be tetracycline-induced, may be related to an underlying genetic susceptibility, and support the notion of multifactorial etiologies for IIH. NEUROLOGY 1995;45: 6-10

Autosomal dominant cerebellar ataxia with retinal degeneration Clinical, neuropathologic, and genetic analysis of a large kindred

The autosomal dominant cerebellar ataxias (ADCA) comprise a heterogeneous group of neurologic disorders characterized by degeneration of the cerebellum, spinal cord, and brainstem. Genetic analysis has revealed two loci, SCA1 on chromosome 6p, and SCA2 on chromosome 12q, responsible for some ADCA. We present a four-generation kindred of 42 individuals, 12 of whom were clinically affected with ADCA and an associated cone dystrophy. Early loss of color discrimination with retinal and macular signs is followed by gradual progression of cerebellar dysfunction and development of pyramidal signs. Pathology shows degeneration of cerebellum, basis pontis, inferior olive, and retinal ganglion cells. For genetic analysis, we used polymorphic markers D6S89 and D12S79; linkage analysis gave negative results, excluding linkage to both SCA1 and SCA2. The data strongly support genetic heterogeneity consistent with the unique clinicopathologic features of the form of ADCA displayed in this large family.

A Comparison of Idiopathic Intracranial Hypertension With and Without Papilledema

Objective.— To compare clinical features, visual characteristics, and treatment of idiopathic intracranial hypertension patients with and without papilledema.

Pseudoturnor cerebri and pregnancy

Pseudotumor cerebri (PTC) is most commonly seen in obese women of reproductive age. We studied 109 women with PTC between ages 16 and 44 years. In 11, PTC started during pregnancy. Thirteen women with previous diagnosis of PTC, including two of the aforementioned 11, had an additional 17 documented pregnancies. Patients were matched by age and parity with controls. Obstetric complications occurred more frequently in the controls. Visual loss occurred with the same frequency in pregnant and nonpregnant patients. Treatment of PTC patients in pregnancy should be the same as for nonpregnant PTC patients, except that calorie restriction and diuretic use are contraindicated. Obstetric management is no different from that of normal pregnancy.

Shedding Light on Photophobia

Photophobia is a common yet debilitating symptom seen in many ophthalmic and neurologic disorders. Despite its prevalence, it is poorly understood and difficult to treat. However, the past few years have seen significant advances in our understanding of this symptom. We review the clinical characteristics and disorders associated with photophobia, discuss the anatomy and physiology of this phenomenon, and conclude with a practical approach to diagnosis and treatment.

Idiopathic intracranial hypertension (Pseudotumor cerebri): A reappraisal

BACKGROUND Increased intracranial pressure can be attributable to many causes, including venous thrombosis and space‐occupying lesions. However, a very common disorder is idiopathic intracranial hypertension (IIH). REVIEW SUMMARY IIH is a condition seen in obese women of childbearing age. Although the incidence is 1 in 100,000 in normal‐weight individuals, the incidence jumps to 20 in 100,000 in women who are obese. Symptoms include headache, transient visual obscurations, and tinnitus. IIH is characterized by papilledema. The diagnosis is made by a scanning procedure to rule out other causes of intracranial hypertension, including venous sinus thrombosis. The spinal fluid examination must be performed to document elevated intracranial pressure and show that the spinal fluid is normal. Evaluations for this condition consist of a neuro‐ophthalmic examination, including visual acuity and visual fields. The patient must be followed carefully for changes in visual field function, because treatment options are affected by the effect of intracranial pressure on visual function. Treatment is aimed at reducing pressure, usually medically, with diuretics such as acetazolamide. If a patient begins to lose vision, optic nerve sheath decompression or lumboperitoneal shunting has been suggested. Children may also lose vision; therefore, careful attention must also be paid to children with this condition. IIH has a profound impact on the quality of life for individual patients. CONCLUSIONS Because IIH is a common disorder, all neurologists should be familiar with the symptoms and signs as well as the evaluation and treatment.

Minocycline treatment and pseudotumor Cerebri syndrome

PURPOSE To demonstrate the association between minocycline treatment and development of the pseudotumor cerebri syndrome. METHODS A retrospective study was conducted of 12 patients from five neuro-ophthalmic referral centers who developed pseudotumor cerebri syndrome after being treated with standard doses of minocycline for refractory acne vulgaris. The main outcome measures included resolution of headaches, transient visual obscurations, diplopia, papilledema, and visual fields static thresholds after withdrawal of minocycline and treatment for increased intracranial pressure. RESULTS Nine (75%) of the 12 patients developed symptoms of the pseudotumor cerebri syndrome syndrome within 8 weeks of starting minocycline therapy; six were not obese. Two patients developed symptoms only after a year had elapsed because of commencement of treatment with minocycline. One patient was asymptomatic, and pseudotumor cerebri syndrome was diagnosed by finding papilledema on routine examination 1 year after minocycline was started. None of the patients developed recurrences for at least 1 year after the discontinuation of minocycline and treatment for increased intracranial pressure, but three (25%) of the 12 patients had substantial residual visual field loss. CONCLUSION Minocycline is a cause or precipitating factor in pseudotumor cerebri syndrome. Although most patients have prominent symptoms and are diagnosed promptly, others are asymptomatic and may have optic disk edema for a long period of time before diagnosis. Withdrawal of minocycline and treatment for increased intracranial pressure lead to resolution of the pseudotumor cerebri syndrome, but visual field loss may persist.

Idiopathic intracranial hypertension Relationship to depression, anxiety, and quality of life

Objective: To explore the incidence of depression and anxiety and to measure quality of life in women with idiopathic intracranial hypertension (IIH), a matched group cross-sectional study was conducted. Women with IIH (n = 28) were compared with control groups of weight- and age-matched women not diagnosed with IIH (n = 30) and with age-matched women of normal weight (n = 30). Methods: Eighty-eight women completed a questionnaire soliciting health information and standardized questionnaires measuring depression, anxiety, and quality of life. The groups were compared using analysis of variance and χ2 tests. Where appropriate, post hoc comparisons were made using Fisher’s test. Results: Patients with IIH reported a greater number of adverse health problems than either of the control groups. Non–health-related psychosocial concerns were equally prevalent among the three groups, but IIH patients were significantly more affected by hardships associated with health problems than the other two groups. The patient group also had higher levels of depression and anxiety than the control groups. These adverse health conditions were reflected in decreased quality of life measures for the IIH patients. Conclusions: This study supports previous reports that link obesity and psychosocial difficulties, but obesity alone is not the explanation for the higher levels of depression and lower levels of quality of life.