Rare Clinical Image of Kennedy’s Syndrome

Sir, Bulbar motor neuron disease, often referred to as bulbar palsy or bulbar onset motor neuron disease, is a subtype of motor neuron disease (MND) that primarily affects the motor neurons located in the bulbar region of the brainstem.1 This condition is characterized by the progressive degeneration of these motor neurons, leading to impairments in speech, swallowing, and sometimes breathing.2 It falls under the broader category of neurodegenerative disorders, sharing similarities with conditions like amyotrophic lateral sclerosis (ALS).3 The bulbar region of the brainstem is crucial for controlling essential functions such as chewing, swallowing, speaking, and breathing. Motor neurons in this area transmit signals from the brain to the muscles responsible for these actions. However, in bulbar motor neuron disease, these neurons gradually degenerate and lose their ability to function properly, resulting in the hallmark symptoms associated with the condition. One of the earliest and most prominent symptoms of bulbar motor neuron disease is dysarthria, a speech disorder characterized by slurred or unintelligible speech.4 In addition to speech and swallowing impairments, individuals with bulbar motor neuron disease may also experience weakness and wasting of the facial muscles, leading to facial drooping or difficulty with facial expressions. The exact cause of bulbar motor neuron disease remains unclear, although it is believed to involve a combination of genetic, environmental, and lifestyle factors. Like other forms of motor neuron disease, bulbar onset disease is characterized by the progressive degeneration of motor neurons, leading to muscle weakness and eventual paralysis.5 An 85-year-old male patient visited the neurology OPD with complaints of left-side weakness, difficulty swallowing solid food, mild discomfort with liquid food, and difficulty in speech articulation. He had a 15-year history of hypertension and had recently stopped taking antihypertensive medications. Over several months, his symptoms had progressively worsened, including slurred speech, recurrent choking during meals, and weakness in his facial muscles. The initial examination showed the patient to be conscious, cooperative, and well oriented. He was examined in a specific position, and his Glasgow Coma Scale score was E4V1M6. Additionally, the examination revealed the loss of certain reflexes, such as the gag reflex and jaw jerk reflex. Sensory examination indicated intact sensations, while motor examination showed reduced strength in the left upper and lower limbs. Diagnostic tests, including an MRI scan and blood tests, confirmed a diagnosis of bulbar disease. Figure 1a and b reveal the presence of a well-defined extra-axial crescent-shaped collection on the right cerebral hemisphere, with a maximum thickness of 2.5 cm. This collection appears hyperintense in T1WI and hypointense in T2/FLAIR, consistent with an early subacute subdural hematoma. The mass effect is evident, causing effacement of sulcal spaces and the ipsilateral lateral ventricle, along with a midline shift of 6 mm to the left. Additionally, there is focal right frontal periventricular white matter edema. Age-related changes are observed, with prominence in sulcogyral spaces, the cerebellar folia, and the ventricular system.Figure 1: MRI brain showing (a) Susceptibility weighted imaging with right subdural bleed, (b) T1WI imaging showing Subacute bleedPatient's consent The patient's guardian has provided a comprehensive and thorough consent. The patient's identity has been effectively anonymized. The journal will not assume responsibility for any legal or medical issues that may arise from concerns regarding the patient's identity or any other matters associated with the public dissemination of the article. Ethical statement This study was conducted in adherence to the ethical principles outlined in the World Medical Association Declaration of Helsinki. According to local guidelines pertaining to single-patient reports, Institutional Review Board approval was not deemed necessary. The patient provided written, informed consent for the publication of both data and images, and this consent was formally documented through their signature. Patient consent declaration The authors affirm that they have obtained all necessary patient consent forms. Within these forms, the patient(s) has/have granted consent for the publication of their images and other clinical information in the journal. The patients are aware that their names and initials will not be disclosed, and diligent measures will be taken to protect their identity. However, complete anonymity cannot be assured. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.