Late Presentation of Hirayama Disease With "Snake Eye Sign": A Case Report.

Hirayama disease, also called non-progressive juvenile muscular atrophy of distal upper limbs, is a type of cervical myelopathy associated with flexion movements of the neck. It is a type of benign motor neuron disease seen typically in young males in the age group of 15 to 25. The disease has an insidious onset with a stationary stage following a progressive phase. It is also called monomelic amyotrophy with patients usually presenting with insidious onset unilateral upper limb weakness and muscle wasting. A bilateral and asymmetrical presentation can be seen very rarely. A middle-aged male patient presented with bilateral asymmetrical upper limb weakness, muscle wasting involving forearm and hand muscles. Neurological examination showed bilateral upper limb weakness and muscle wasting involving forearm and hand muscles, with a classical pattern of muscle wasting in bilateral forearm muscles called oblique amyotrophy. A clinical diagnosis of Hirayama disease was made and the patient was sent to the radiology department for Magnetic Resonance Imaging of the cervical spine in flexion and neutral positions. The imaging findings were consistent with the clinical diagnosis of Hirayama disease with the presence of an abnormal "snake eye appearance". The electrophysiological assessment done including the electromyography and nerve conduction studies were also consistent with the clinical diagnosis. "Snake eye appearance" on MRI in patients with Hirayama disease is associated with unfavorable outcomes and represents cervical myelopathy involving the anterior horn cells.

Blocked Filter of Anti-Embolic Device During Carotid Artery Stenting: A Rare Occurrence Posing Challenging Diagnostic Dilemma.

The use of anti-embolic devices (AED's) is a common practice in carotid artery stenting (CAS). It prevents the passage of blood clots and thrombi generated during the procedure from embolizing into the intracranial circulation. Disadvantages include the passage of small particles and complications related to advancement, deployment, and recovery of the filters. The filter of the AED can get clogged due to the high load of the emboli generated during CAS causing a slowing of the intracranial blood flow which normalizes once the filter is removed. Here, we are presenting a case of the filter of AED getting blocked due to entrapped thrombi or blood clots and mimicking dissection and, sharing our experiences associated with the event.

Cerebral Venous Sinus Thrombosis Complicating Middle Ear Infections: A Rare Complication in Post-Antibiotic Era.

In the post-antibiotic era, intracranial and extracranial complications of middle ear infections have become rare. Similarly, cerebral venous sinus thrombosis (CVST), a frequent complication of middle ear infections, has become rare now. Here, we present a case of a 27-year-old male who presented with a short history of severe headache and associated episodes of intractable vomiting. There was also a prior history of right ear discharge one year back which responded to medical management. The patient did not improve clinically even after prompt symptomatic management. Contrast-enhanced magnetic resonance imaging (CEMRI) of the head and contrast-enhanced magnetic resonance venogram (CEMRV) were done, which showed right-sided otomastoiditis complicated with CVST and meningitis. Although the clinical signs of meningeal irritation and mastoid tenderness were not present on clinical examination. The patient was started on anticoagulant therapy and antibiotics for two weeks following which there was marked clinical improvement.

Intracardiac hydatid cyst located in right ventricular outflow tract: a rare site.

Intracardiac hydatid cyst is relatively uncommon and involvement of right ventricular outflow tract is extremely rare. We report a rare case of intracardiac hydatid cyst involving the right ventricular outflow tract and do a review of literature.