Syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) in a patient with confusional symptoms, diffuse EEG abnormalities, and bilateral vasospasm in transcranial Doppler ultrasound: A case report and literature review

• Published in: Neurologia (Barcelona, Spain) Vol. 34; no. 8; pp. 536 - 542
• Main Authors: Hidalgo de la Cruz, M, Domínguez Rubio, R, Luque Buzo, E, Díaz Otero, F, Vázquez Alén, P, Orcajo Rincón, J, Prieto Montalvo, J, Contreras Chicote, A, Grandas Pérez, F
• Format: Journal Article
• Language: English; Spanish
• Published: Spain 01-10-2019
• Subjects: Adult; Confusion - complications; Confusion - physiopathology; Electroencephalography; Headache - cerebrospinal fluid; Headache - complications; Humans; Lymphocytosis - cerebrospinal fluid; Lymphocytosis - complications; Male; Nervous System Diseases - cerebrospinal fluid; Nervous System Diseases - complications; Syndrome; Time Factors; Ultrasonography, Doppler, Transcranial; Vasospasm, Intracranial - complications; Vasospasm, Intracranial - diagnostic imaging; Cerebral vasomotor changes; Síndrome confusional; Acute confusional syndrome; Consumo de cannabis y cocaína; Pseudomigraine with pleocytosis; Cannabis and cocaine use; Doppler transcraneal; Vasoespasmo; HaNDL. Pseudomigraña con pleocitosis; Transcranial Doppler; HaNDL
• ISSN: 1578-1968; 2173-5808
• DOI: 10.1016/j.nrl.2017.02.008

HaNDL syndrome (transient headache and neurological deficits with cerebrospinal fluid lymphocytosis) is characterised by one or more episodes of headache and transient neurological deficits associated with cerebrospinal fluid lymphocytosis. To date, few cases of HaNDL manifesting with confusional symptoms have been described. Likewise, very few patients with HaNDL and confusional symptoms have been evaluated with transcranial Doppler ultrasound (TCD). TCD data from patients with focal involvement reveal changes consistent with vasomotor alterations.
We present the case of a 42-year-old man who experienced headache and confusional symptoms and displayed pleocytosis, diffuse slow activity on EEG, increased blood flow velocity in both middle cerebral arteries on TCD, and single-photon emission computed tomography (SPECT) findings suggestive of diffuse involvement, especially in the left hemisphere.
To our knowledge, this is the first description of a patient with HaNDL, confusional symptoms, diffuse slow activity on EEG, and increased blood flow velocity in TCD. Our findings suggest a relationship between cerebral vasomotor changes and the pathophysiology of HaNDL. TCD may be a useful tool for early diagnosis of HaNDL.