Idiopathic intracranial hypertension clinical features


Idiopathic intracranial hypertension often manifests as headaches, severe visual impairment, or even blindness.


A retrospective chart review of patients diagnosed with Idiopathic intracranial hypertension at the First Affiliated Hospital of Zhengzhou University was conducted from January 2013 to July 2021. The analysis included demographic data, presenting symptoms, comorbidities, imaging features, laboratory data, intracranial pressure (ICP), treatment modalities, and outcomes. Results The study recruited 199 participants, including 145 females and 54 males, with a mean age at onset of 36 years (range: 27 to 45 years). The participants had a mean body mass index (BMI) of 26 kg/m2 (range: 23.4 to 29.4 kg/m2). Obesity was found in 67 participants (33.7%).


The most common clinical symptom reported was headache, which was experienced by 118 (59.3%) participants, followed by decreased vision, which 115 (57.8%) participants reported. The main comorbidity among women was anemia (54, 37.2%), while men were more likely to have severe sleep apnea (7, 13%). The most common imaging features were perioptic nerve sheath distension (159, 79.9%) and transverse sinus stenosis (147, 73.9%). Symptoms were relieved with medication in 117 (58.8%) participants, while 72 (36.2%) underwent surgeries such as venous sinus stenting and ventriculoperitoneal shunt. During follow-up, symptoms resolved in 84 (42.2%) participants, while 115 (57.8%) participants experienced symptom improvement. The ratio of decreased vision was higher in females than in males. The results provide valuable insights into the clinical features of IIH in this region. China appears to have a lower incidence of obesity compared to Western countries. Among comorbidities related to IIH, anemia, and severe sleep apnea were the most common. A significant number of IIH patients underwent surgery. It was found that women had worse visual outcomes compared to men. Further investigation is needed to determine the most effective treatment for IIH in a larger cohort of Chinese patients 1).


Patients most frequently present with headaches, transient visual obscurations, papilledema, and/or pulsatile tinnitus, but may also be asymptomatic 2).

The signs and symptoms of intracranial hypertension are that the patient maintains an alert and oriented mental state, but has no localizing neurologic findings.

In children, numerous nonspecific signs and symptoms may be present.

It has been reported that the clinical features of childhood IIH, in particular prepubertal IIH, differ from those of adult IIH 3) 4).

The differences are the absence of differences in the sex distribution at the onset and the absence of a significant correlation with obesity. In addition, adult IIH patients often complain of headaches, have transient low vision, and tinnitus, while pediatric IIH patients often complain of restlessness, dizziness, and neck pain. However, adolescent IIH patients have clinical features similar to that of adult IIH. Currently, there is ongoing prospective study of the treatment of IIH in adults, but no data exist in children 5).

Psychiatric disorders are highly prevalent in patients with IIH and associate with worse subjective outcomes. These findings advocate for monitoring the mental health of patients with IIH and warrant further multidisciplinary research to understand the potentially underlying psychosocial and neuroendocrinological mechanisms 6).

Headache

The most common symptom of IIH is headache, which occurs in almost all (92–94%) cases. It is characteristically worse in the morning, generalized in character and throbbing in nature. It may be associated with nausea and vomiting. The headache can be made worse by any activity that further increases the intracranial pressure, such as coughing and sneezing. The pain may also be experienced in the neck and shoulders.

Although the headache characteristics are indistinguishable from the symptoms of migraine headache, accompanying symptoms of increased intracranial pressure, such as pulsatile tinnitus, transient visual obscurations, and radicular neck pain, may aid in the diagnosis 7).

Tinnitus

Many have pulsatile tinnitus, a whooshing sensation in one or both ears (64–87%); this sound is synchronous with the pulse.

Other symptoms

Various other symptoms, such as numbness of the extremities, generalized weakness, loss of smell, and loss of coordination, are reported more rarely; none are specific for IIH.

The increased pressure leads to compression and traction of the cranial nerves. Most commonly, abducens nerve palsy.

More rarely, the oculomotor nerve and trochlear nerve (third and fourth nerve palsy, respectively) are affected; both play a role in eye movements.

The facial nerve (seventh cranial nerve) is affected occasionally –- the result is total or partial weakness of the muscles of facial expression on one or both sides of the face.

Spontaneous cerebrospinal fluid leak


1)
Tian T. Clinical Characteristics of Patients With Idiopathic Intracranial Hypertension in China. Cureus. 2024 Jul 20;16(7):e64990. doi: 10.7759/cureus.64990. PMID: 39161509; PMCID: PMC11333022.
2)
Galvin JA, Van Stavern GP. Clinical characterization of idiopathic intracranial hypertension at the Detroit Medical Center. J Neurol Sci. 2004 Aug 30;223(2):157-60. PubMed PMID: 15337617.
3) , 5)
Ko MW, Liu GT. Pediatric idiopathic intracranial hypertension (pseudotumor cerebri) Horm Res Paediatr. 2010;74:381–389
4)
Yabe I, Moriwaka F, Notoya A, Ohtaki M, Tashiro K. Incidence of idiopathic intracranial hypertension in Hokkaido, the northern-most island of Japan. J Neurol. 2000;247:473–475.
6)
Puustinen T, Tervonen J, Avellan C, Jyrkkänen HK, Paterno JJ, Hartikainen P, Vanhanen U, Leinonen V, Lehto SM, Elomaa AP, Huttunen TJ. Psychiatric disorders are a common prognostic marker for worse outcome in patients with idiopathic intracranial hypertension. Clin Neurol Neurosurg. 2019 Sep 17;186:105527. doi: 10.1016/j.clineuro.2019.105527. [Epub ahead of print] PubMed PMID: 31586855.
7)
Digre KB. Idiopathic intracranial hypertension headache. Curr Pain Headache Rep. 2002 Jun;6(3):217-25. Review. PubMed PMID: 12003693.
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