What are the other Names for this Condition? (Also known as/Synonyms)

• HaNDL Syndrome
• Migraine with Cerebrospinal Pleocytosis
• Pseudomigraine with Lymphocytic Pleocytosis

What is Syndrome of Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis? (Definition/Background Information)

• Syndrome of Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis or HaNDL Syndrome is a headache disorder of adulthood that is associated with neurological signs and symptoms. It is also marked by an abnormal increase in white blood cells, called lymphocytic pleocytosis, in the cerebrospinal fluid (CSF). The syndrome is known to improve and resolve with treatment in about three months
• The episodic migrainous headaches in HaNDL Syndrome are generally moderate-to-severe in intensity and may last several hours to even a day (over 24 hours). The headaches are observed with (or preceded by) transient neurological deficits, such as the inability to move one side of the body, a lack of sensation on one side of the body, and/or speech impairment, in about 50-75% of the cases. In the affected individuals, a past history of migraine is not usually observed

The criteria for diagnosis of Syndrome of Transient Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL Syndrome) as outlined by the International Headache Society (IHS) is given below:

• Episodes of migraine-like headache fulfilling all of the criterion below

Both of the following:

• Accompanied or shortly preceded by onset of at least one of the following transient neurological deficits lasting over 4 hours
  • Hemiparesthesia
  • Dysphasia
  • Hemiparesis
• Associated with cerebrospinal fluid (CSF) lymphocytic pleocytosis (over 15 white cells per µl), with negative etiological studies

Evidence of causation demonstrated by either or both of the following:

• Headache and transient neurological deficits have developed or significantly worsened in temporal relation to onset or worsening of the CSF lymphocytic pleocytosis, or led to its discovery
• Headache and transient neurological deficits have significantly improved in parallel with improvement in the CSF lymphocytic pleocytosis

Not better accounted for by another ICHD-3 diagnosis.

(Source: International Headache Society Classification ICHD-3, London, United Kingdom)

A differential diagnosis to exclude the following may be necessary:

• CNS vasculitis
• Encephalitis
• Granulomatous and neoplastic arachnoiditis
• Hemiplegic migraine, specifically familial hemiplegic migraine type 1 (FHM1)
• Mycoplasma
• Neuroborreliosis
• Neurobrucellosis
• Neurosyphilis