Headache Attributable to Nonvascular Intracranial Disorders

Abstract

Headache attributable to nonvascular intracranial disorder is a basket of multiple, partly complex, and very diverse idiopathic or secondary disorders. By definition, the headache has to occur in a close temporal relationship to the intracranial disorder. Some of these headache disorders are caused by high or low cerebrospinal fluid pressure; noninfectious inflammatory diseases such as neurosarcoidosis, aseptic (noninfectious) meningitis, and lymphocytic hypophysitis; or intracranial neoplasm. Other nonvascular headaches, including hemicrania epileptica and postseizure headache, Chiari malformation type I, and the syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis, are attributed to hypothalamic or pituitary hyper- or hyposecretion, intrathecal injection, or epileptic seizures. The clinical presentation of all these disorders can be diverse and often mimics the characteristics of primary headaches, which may delay the diagnosis.

HaNDL syndrome in a 14-year-old girl

“…It has had numerous names in the past, such as migraine with pleocytosis and pseudomigraine with pleocytosis, but was coined HaNDL in 2004 by the International Classification of Headache Disorders 1. Approximately 100 cases have been described in the literature,2 and less than 15 in the paediatric population 3…”

“…Focal neurological deficits are restricted to one hemisphere in most patients2 and include hemiparaesthesia in three quarters of cases, dysphasia in half and hemiparesis in a little over half of patients, which usually last over 4 h (some up to 24 h) without leaving neurological sequelae and with symptom-free intervals 6. These episodes of headache and neurological deficits recur over fewer than 3 months 3.…”

“…These episodes of headache and neurological deficits recur over fewer than 3 months 3. Over 90% of cases have increased protein in CSF (>250 mg/dL) and 60–70% have increased CSF pressure 2 7…”

“…Treatment is symptomatic and supportive with all patients recovering within 1–84 days with only a few patients experiencing recovery 2 18. Because the disease is self-limiting, education and reassurance of the patient and family are very important.…”

Tension-Type Headache Mimics

“…Papilledema is found in most cases of IIH, but may be absent or difficult to detect, particularly in its early stages. In 25-30% of patients, there may be permanent visual loss [14]. Treatment is aimed at preserving visual function and managing headache and other symptoms.…”