October 3, 2023

Highlights

  1. HaNDL syndrome is a rare condition characterized by recurrent headaches and neurological signs such as motor, sensory, and aphasic symptoms.
  2. The exact cause of HaNDL syndrome is unclear, but it’s thought to be triggered by an infectious process.
  3. MRI scans of patients with HaNDL syndrome are typically normal, but some cases have shown abnormalities such as grey matter swelling and hypoperfusion in certain brain regions.
  4. Treatment for HaNDL syndrome is symptomatic, aiming to relieve the symptoms rather than cure the condition. The prognosis for patients is generally excellent.
  5. HaNDL syndrome can mimic serious diseases like stroke and meningoencephalitis, making accurate diagnosis crucial.


Headache and Neurological Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL) syndrome is a rare condition characterized by recurrent headaches accompanied by focal neurological signs. These signs can include motor, sensory, and aphasic symptoms. The syndrome falls under the category of headache attributed to “noninfectious inflammatory disease”. The neurological deficits associated with HaNDL syndrome can persist for up to 7 weeks.

Pathology

The exact pathophysiology of HaNDL syndrome remains unclear. However, changes in the neurovascular system that resemble those found in migraines are observed. These changes are thought to be triggered by an infectious process.

Radiographic Features

MRI scans of the brain in patients with HaNDL syndrome are typically normal. However, there have been case reports of abnormalities that include grey matter swelling, sulcal enhancement, and hypoperfusion involving unilateral temporal and occipital regions.

Treatment and Prognosis

The treatment for HaNDL syndrome is symptomatic, meaning it aims to relieve the symptoms rather than cure the condition. The prognosis for patients with HaNDL syndrome is considered excellent.

Differential Diagnosis

Clinically, HaNDL syndrome can mimic various serious, including life-threatening, diseases, such as stroke and meningoencephalitis. Therefore, accurate diagnosis is crucial to ensure appropriate treatment and management.

Diagnostic criteria

Previously used terms:

Migraine with cerebrospinal pleocytosis; pseudomigraine with lymphocytic pleocytosis.

Description:

Migraine-like headache episodes (typically one to twelve) accompanied by neurological deficits including hemiparaesthesia, hemiparesis and/or dysphasia, but positive visual symptoms only uncommonly, lasting several hours. There is cerebrospinal fluid lymphocytic pleocytosis. The disorder resolves spontaneously within 3 months.

Diagnostic criteria:
  1. Episodes of migraine-like headache fulfilling criteria B and C1
  2. Both of the following:
    1. accompanied or shortly preceded by onset of at least one of the following transient neurological deficits lasting >4 hours
      • a) hemiparaesthesia
      • b) dysphasia
      • c) hemiparesis
    2. associated with cerebrospinal fluid (CSF) lymphocytic pleocytosis (>15 white cells per ┬Ál), with negative aetiological studies
  3. Evidence of causation demonstrated by either or both of the following:
    1. 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
    2. headache and transient neurological deficits have significantly improved in parallel with improvement in the CSF lymphocytic pleocytosis
  4. Not better accounted for by another ICHD-3 diagnosis2.
Notes:
  1. Most patients with this syndrome have no prior history of migraine.
  2. Other diagnoses that may share some of its clinical features include 1.2.3 Hemiplegic migraine, although mutations of the CACNA1A gene, the cause of 1.2.3.1.1 Familial hemiplegic migraine type 1 (FHM1), have been excluded in several patients with 7.3.5 Syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL)). Also to be excluded are neuroborreliosis, neurosyphilis, neurobrucellosis, mycoplasma, granulomatous and neoplastic arachnoiditis, encephalitis and CNS vasculitis.

Conclusion

While HaNDL syndrome is a rare condition, it is important for medical professionals to be aware of it due to its potential to mimic more serious diseases. Further research is needed to fully understand the pathophysiology of the syndrome and to develop more targeted treatments.

Disclaimer: This article is intended for informational purposes only and should not be used as a replacement for professional medical advice.

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