Question

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Case Study 4: Uncoordinated Felix, a 54-year-old masonry contractor, has been complaining for the past three months about increasing arm weakness and that it is becoming more difficult to pick up and use his tools. He has recently been stumbling at his work site and his speech is, at times, difficult to understand because of poor word choices. He has also had worsening memory and forgot how to install a granite lamp post at a client's house. The last straw that forced him to make a neurology appointment was when he dropped his hammer drill onto his right foot and fractured the cuboid and navicular bones. A neurological examination uncovered the following results: 1. Bilateral rapid eye movement at rest 2. Slurred speech 3. Difficulty swallowing 4. Overshoot on finger-to-nose test 5. Failed Romberg balance test Student Questions: 1. Identify three regions of the central nervous system that may be compromised 2. Connect three remarkable signs/symptoms with the proper clinical terms 3. Provide a primary diagnosis and a possible lesion type (e.g. what anatomical problem is causing the disorder?) 4. Present one treatment option for this patient. Include a brief explanation

Answer 1

1. Cerebellum, Spinal cord and Peripheral nerves may be compromised.

2.  Limb ataxia:

Limb ataxia is often used to describe ataxia of the upper limbs resulting from incoordination and tremor and can be better described by functional impairment, such as clumsiness with writing, buttoning clothes, or picking up small objects. The patient has to slow down the movement to be accurate in reaching things. Limb ataxia can be lateralized with ipsilateral cerebellar lesions.

Nystagmus (Involuntary back-and-forth eye movements):

Nystagmus often occurs in cerebellar disease. Lateral gaze-evoked nystagmus is seen by slow drift toward midline followed by a fast phase of saccades to the eccentric position. Upbeat and downbeat nystagmus are defined by the rapid phase in the up or down direction. Upbeat nystagmus is seen in lesions of the anterior vermis. Downbeat nystagmus is typically seen in a lesion in the foramen magnum such as an Arnold-Chiari malformation.

Dysarthria/scanning speech:

Dysarthria is often described by the patient or relatives as slurred speech. The patient’s speech is irregular and slow with unnecessary hesitation. Words are often broken into separate syllables, and some syllables with a plosive consonant are unusually stressed (scanning speech).

3.  The primary diagnosis is ataxia and mostly due to the cerebellar lesion.

Ataxia can be defined as impaired coordination of voluntary muscle movement, is a physical finding, not a disease.

The cerebellum and its afferent and efferent connections, the vestibular system, and the proprioceptive sensory pathway are all involved in ataxia. The cerebellum is usually separated into the midline cerebellum and the cerebellar hemispheres. Lesions in each of these regions can result in a different presentation of ataxia.

A focal cerebellar lesion often leads to ipsilateral impaired cerebellar functions, including limb dysmetria, intention tremor, loss of check, hypotonia, and dorsal spooning (hyperextension of interphalangeal joints) of the hand, as well as dysarthria and nystagmus.

4.

Therapies a person with ataxia may benefit from include speech therapy to improve speech and swallowing, occupational therapy to help you with tasks of daily living, as well as physical therapy to help in building strength and enhance mobility.

Speech-language pathologists complete a comprehensive assessment of patients' communication. The assessment may include:

• Perceptual and objective measures of motor speech function and cognitive linguistic function
• an evaluation of the impact of communication difficulties on the individual's participation in activities of daily living and life roles and
• views of the family/carers.

An individualized treatment program is created for individuals with motor speech disorders due to progressive ataxia, based on the findings of a comprehensive assessment. Management may involve:

• the Lee Silverman Voice Treatment;
• assisting patients to develop increased self-monitoring of the speech quality; and
• identifying helpful speech strategies (e.g., over-articulation, shorter phrases, breath top-ups)