Acute confusional state in HaNDL syndrome

Area of Science:

• Neurology
• Neuroscience
• Clinical Medicine

Background:

• Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) syndrome is characterized by migraine-like headaches, neurological deficits, and lymphocytic pleocytosis in cerebrospinal fluid.
• Current diagnostic criteria (ICHD-3) do not explicitly include acute confusional state as a common feature of HaNDL syndrome.
• The pathogenesis of acute confusional state in HaNDL remains unclear.

Observation:

• A case of a 32-year-old male presenting with migraine-like headaches, hemiparesis, and confusional state, ultimately diagnosed with HaNDL syndrome.
• A systematic review of 159 HaNDL cases revealed that 41 patients (25.7%) exhibited acute confusional state.
• Among HaNDL patients with confusional state, a significant proportion (66.6%) showed increased cerebrospinal fluid opening pressure.

Findings:

• Acute confusional state is a more frequent manifestation of HaNDL syndrome than previously recognized.
• Elevated intracranial pressure is potentially implicated in the development of acute confusional state within the HaNDL syndrome.
• The study highlights the need for further research into the neuroimaging and clinical characteristics of HaNDL.

Implications:

• The findings suggest that acute confusional state should be considered in the diagnostic spectrum of HaNDL syndrome.
• It is proposed that diagnostic criteria for HaNDL syndrome be updated to include acute confusional state.
• Further large-scale studies are warranted to elucidate the role of intracranial hypertension in HaNDL-associated confusional states.