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AANEM Case Study: Sensory Neuronopathy (Dorsal Root Ganglionopathy)

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AANEM Case Study: Sensory Neuronopathy (Dorsal Root Ganglionopathy) AANEM Case Study: Sensory Neuronopathy (Dorsal Root Ganglionopathy) Author Information Full Name: T Alam MRCP, M Hadjivassiliou MD FRCP, DG Rao DM FRCP Affiliation: Sheffield Teaching Hospitals NHS Foundation Trust No authors involved in the planning of this CME activity had any relevant financial relationships to disclose. Reviewed and accepted by the 2014-2015 Website CME Committee Certified for CME credit 10/2018 – 10/2021 Reviewed 10/2018 by Dr. Ratna Bhavaraju-Sanka of the the 2018-2019 Website CME Committee No planners of this CME activity had any relevant financial relationships to disclose. Dr. Bhavaraju-Sanka is on the Lundbeck Speaker Bureau. All conflicts of interest have been resolved according to ACCME standards Copyright© October 2015 AMERICAN ASSOCIATION OF NEUROMUSCULAR & ELECTRODIAGNOSTIC MEDICINE ELECTRODIOGNOSTIC MEDICINE 2621 Superior Drive NW Rochester, MN 55901 Case Study: Sensory Neuronopathy - pg. 1 Sensory Neuronopathy (Dorsal Root Ganglionopathy) EDUCATIONAL OBJECTIVES: Upon completion of this case study, participants will acquire skills to 1) Identify clinical and electrophysiological features of sensory neuronopathy; 2) Distinguish sensory neuronopathy from other conditions presenting with patchy involvement; and 3) outline the common causes of sensory neuronopathy. ACCREDITATION STATEMENT: The American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education (CME) for physicians. The AANEM certifies that this CME activity was planned and produced in accordance with ACCME Policies, Accreditation Criteria, and Standards for Commercial Support. CME CREDIT: The AANEM designates this enduring material for a maximum of 2 AMA PRA Category 1 Credit(s)TM. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Case Studies published by the AANEM are reviewed every 3 years by an AANEM education committee for their scientific relevance. CME credit is granted for 3 years from the date of publish, review, or revision. Individuals requesting credit for CME materials that have been discontinued will be notified that CME credit is no longer available. CLAIMING CME/CEU CREDIT: The reader should carefully and thoroughly study the material. If further clarification is needed, references should be consulted. To obtain CME/CEU credit: 1. After checkout, a CME survey link will be emailed to you from [email protected] 2. Review the case study, then follow the CME survey link to complete the post-test 3. After completing the post-test, your CME/CEU transcript will update automatically Case Study: Sensory Neuronopathy - pg. 2 EMG CASE: Sensory Neuronopathy (Dorsal Root Ganglionopathy) T Alam MRCP, M Hadjivassiliou MD FRCP, DG Rao DM FRCP Case Information Presenting Symptom(s): Ataxia, sensory disturbance Case-specific Diagnosis: Sensory neuronopathy (dorsal root ganglionopathy) Appropriate Audience: Residents and practicing physicians Level of Difficulty: Intermediate 1. HISTORY A 49-year-old Caucasian woman was referred to the neurophysiology department for a repeat assessment due to deteriorating neurological symptoms. She has a longstanding diagnosis of mononeuritis multiplex (MNM), Sjögren’s syndrome (confirmed by labial salivary gland biopsy), and systemic lupus erythematosus (SLE). Past medical history was significant for L5/S1 discectomy and benign essential tremor. Her current medication included mycophenolate, pregabalin, omeprazole, and duloxetine. She originally presented to the neurology department 15 years prior with altered sensation affecting the left little finger and subsequently the index finger. This progressed with further sensory disturbance affecting the left radial nerve sensory territory and unsteadiness when walking. Examination at the time revealed mildly reduced pinprick and light touch sensation over the left median, ulnar, and radial nerve distribution. Reflexes were present in the upper limbs but absent in the lower limbs. No weakness was noted in any limbs on examination. Gait was ataxic. Joint position sense was impaired to the level of the wrist in the left upper limb but normal on the right. Joint position sense and vibration were impaired to the level of the ankle bilaterally. Following electrophysiological assessment, a diagnosis of MNM was made at that time, with the patient subsequently managed with a variety of immunosuppressant agents including oral prednisolone, intravenous (IV) methylprednisolone, azathioprine, pulsed IV cyclophosphamide, Case Study: Sensory Neuronopathy - pg. 3 and mycophenolate. Her neurological state in the interim period has been largely stable with a repeat electrophysiological assessment a few years prior showing no significant change in parameters. 2. COMMENTARY I The patient’s history is of a longstanding, slowly progressive sensory disturbance with significant ataxia. There is no weakness in any limbs on manual muscle testing. This suggests that there is significant involvement of sensory fibers with sparing of motor fibers. The distribution is also in an asymmetrical and non-length dependent distribution. Treatment with immunosuppressants appears to have stabilized her condition for a number of years. Chronic inflammatory demyelinating polyneuropathy or a variant such as multifocal acquired sensory and motor demyelinating neuropathy (Lewis–Sumner syndrome) is also a possibility. However, these conditions present with additional motor weakness and may have a relapsing and remitting course, although they can also be progressive. The patient here experienced a progressive course. MNM is a possibility, but lack of motor manifestations and absence of pain at the onset of symptoms made it a less likely candidate in the diagnosis. Myelopathy due to an immune-mediated disorder is another possibility causing sensory disturbances, balance, and walking problems. However, the lack of bladder symptoms suggests a low likelihood of this pathology in the differential diagnosis. 3. HISTORY, CONTINUED A repeat electrophysiological assessment has been requested due to an increase in the number of falls and worsening ataxia. The patient has also developed a sensory disturbance affecting the face bilaterally. She also feels that her right arm is becoming more difficult to use. She denies any clear weakness in either the upper or lower limbs. 4. COMMENTARY II The patient’s sensory symptoms have progressed further, now affecting her cranial nerve territory. There has also been a further deterioration in her balance. She denies any clear weakness, which would continue to favor the initial impression of sparing of motor fibers, although this needs to be confirmed this during the examination. She has developed a facial sensory loss indicating trigeminal nerve involvement and this may purely reflect progression of existing condition. On the other hand, she might have developed new neurological illness causing trigeminal neuropathy. Examination should be focused to exclude other lower cranial nerve involvement. History and examination must be focused to exclude skull base infective, inflammatory, or malignant pathology. 5. PHYSICAL EXAMINATION Case Study: Sensory Neuronopathy - pg. 4 Examination of the cranial nerves reveals subtle asymmetric sensory loss over predominantly V2 and V3 of the trigeminal nerves, worse on the left. The cranial nerves are otherwise unremarkable with no nystagmus. Speech is normal. Tone is normal in all limbs. Power is 5/5 in all muscle groups in both lower limbs other than right dorsiflexion of the foot, which is 4+/5. Power in the upper limbs is normal when the patient focuses on performing a particular movement using visual clues. However, she is not able to perform manual muscle testing with the eyes closed due to marked incoordination. She is areflexic in all limbs. Plantar responses are flexor (Babinski sign was negative). There is mild sensory loss to light touch and pinprick sensation over both upper limbs up to the level of the metacarpophalangeal joints on the right and the wrist on the left. Sensations in the lower limbs are relatively well preserved, although there is a mild loss to light touch over the right L5 dermatome. Joint position sense is present at the elbow joints in the upper limbs and at the ankle joints in the lower limbs. Vibration sense is present at the shoulder joint in the upper limbs and the knee joints in the lower limbs. There is mild past pointing and dysdiadochokinesis. Gait is ataxic. Romberg’s sign is positive. There are pseudoathetoid movements of the fingers when holding the arms out with eyes closed. 6. COMMENTARY III The striking finding on examination is the presence of significant ataxia, pseudoathetosis, areflexia, and impairment of vibration and joint position sense with less marked loss of light touch/pinprick sensation and largely normal power in all limbs. This confirms the impression of a process predominantly affecting sensory fibers with sparing of motor fibers. There are additional asymmetric features. The fact that there is sensory disturbance over the trigeminal nerve territory with marked involvement of the upper limbs but with the lower limbs being relatively less affected is unusual for a length-dependent process. In order to confirm or refute this hypothesis, during the electrodiagnostic examination multiple sensory and motor nerves in multiple limbs will need to be examined. Trigeminal nerve sensory fibers
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