Urbach-Wiethe disease is a rare, autosomal recessive disorder caused by a mutation of extracellular matrix protein-1 (ECM1).

Neurological complications can include basal ganglia calcification of the anterior medial temporal lobes (which may predispose to seizures), cognitive and neuropsychiatric abnormalities, or rarely spontaneous intracranial haemorrhage.

We present a case of Urbach-Wiethe disease associated with Kluver-Bucy syndrome, complicated both by intracranial haemorrhage and partial seizures presenting as panic attacks.

A 32 year old Irish man presented with sudden onset left upper limb and lower limb weakness secondary to a spontaneous right frontal haemorrhage. He had a background of panic disorder since his teens, with episodic stereotyped ‘panic attacks’. His history was significant for a hoarse voice since childhood, hyperorality, hypersexuality and hyperphagia (BMI of 44kg/m2).

Computed tomography (CT) brain revealed an acute right frontal lobe parenchymal haemorrhage, with dense hippocampal and symmetric basal ganglia calcification, including the amygdala bilaterally.

An ambulatory electroencephalogram (EEG) without video revealed a build-up of theta activity prior to the ‘panic attacks’. Frequency of these events improved on commencement of an anti-epileptic medication.

The patient scored below the 10^th centile in all measures in the Repeatable Battery for the Assessment of Neuropsychological Status. Frontal dysfunction was demonstrated overall.

He had difficulty drawing representations of, and recognising facial expressions.

Single gene testing was positive for a loss of function mutation in ECM-1, confirming a diagnosis of Urbach-Wiethe disease.

Similar to previous patients with this condition, our patient exhibited an abnormal experience of fear. However, a unique feature described in this case is the presence of an ictal experience of fear.