Sex: F
Vaccine: VARZOS
Symptoms: Anti-ganglioside antibody negative, Areflexia, Atrophy, CSF glucose normal, CSF lymphocyte count increased
Clinical narrative: Bilateral Parsonage-Turner syndrome; right upper extremity pain/left upper extremity (pain followed by weakness); right upper extremity weakness/left upper extremity (pain followed by weakness; atrophy of the bilateral shoulders; upper extremity reflexes absent; This case was reported in a literature article and described the occurrence of parsonage-turner syndrome in a 54-year-old female patient who received Herpes zoster (Shingles vaccine) for prophylaxis.   On an unknown date, the patient received Shingles vaccine. On an unknown date, several days after receiving Shingles vaccine, the patient experienced parsonage-turner syndrome (serious criteria hospitalization), pain in upper extremities (serious criteria hospitalization), weakness of upper extremities (serious criteria hospitalization), atrophy (serious criteria hospitalization) and absent reflex. The patient was treated with immunoglobulins nos (Immunoglobulin), prednisone and non-drug therapy (Physical Therapy (Nos)). On an unknown date, the outcome of the parsonage-turner syndrome, weakness of upper extremities, atrophy and absent reflex were unknown and the outcome of the pain in upper extremities was recovered/resolved.   It was unknown if the reporter considered the parsonage-turner syndrome, pain in upper extremities, weakness of upper extremities, atrophy and absent reflex to be related to Shingles vaccine.  Additional details were provided as follows: This case was reported in a literature article and described the occurrence of Bilateral Parsonage-Turner syndrome (PTS), in a female patient aged 54-year-old, who was vaccinated with Unspecified Shingles vaccine (manufacturer unknown) for prophylaxis.   The patient was a right-handed dominant female chef at a local university. No information on patient medical or family history or concurrent condition or concomitant medication was reported.  On an unspecified date, the patient had received unspecified Shingles vaccine (administration route and site unspecified, dosage unknown; batch number not provided). The age at vaccination was not reported but it could be between 53 and 54 years.   On an unknown date, a few days after receiving vaccination, the patient developed right upper extremity (RUE) pain which was quickly followed by RUE weakness. The patient saw an outside neurologist at that time, and after an extensive workup that included magnetic resonance imaging (MRI), electromyography (EMG), nerve conduction studies (NCS), and lumbar puncture (LP), no organic cause was discovered, and based on patient clinical examination a functional disorder was suspected (giveway/collapsing weakness). Five months later, a similar series of symptoms occurred in the left upper extremity (pain followed by weakness). No further workup was done at that time, and the patient did not receive treatment due to the belief that her symptoms were psychogenic. Despite spontaneous resolution of pain, the patient presented to the emergency room for persistent weakness four months later (nine months after the initial event involving the RUE). On physical examination during her most recent admission, profound symmetric weakness was noted in her bilateral upper extremities. The weakness was more severe proximally (2/5 strength in deltoids; 3/5 biceps, triceps, wrist extensors, wrist flexors, and intrinsic hand muscles; 4/5 finger flexors and extensors), and significant atrophy of the bilateral shoulders was noted. The patient upper extremity reflexes (biceps, triceps, brachioradialis) were absent bilaterally, with preservation of her lower extremity reflexes. Sensation to pinprick, light touch, vibration, and temperature were diminished symmetrically up to the shoulder in her bilateral upper extremities. During the patient most recent/final admission, the patient received an evaluation that included MRI with gadolinium of the brain, cervical spine, thoracic spine, and brachial plexus, along with Lumbar puncture (LP). LP for cerebro spinal fluid (CSF) showed volume: 1.5 mL, clear and colorless CSF, red blood cell (RBC): 1/mcL; white blood cell (WBC): 4/mcL (normal range: 0-5/mcL); lymphocytes: 98% (normal range: 40-80%); monocytes: 2% (normal range: 15-45%); differential based on: 100 cells; glucose: 66mg/dL (50-80mg/dL); protein: 23.0 mg/dL )normal range: 15.0-60.0 mg/dL); Meningitis/encephalitis panel by PCR was negative; west nile virus AB igM was 0.02 IV (normal range: less than equal to 0.89); IgG and IgM monos GM1, IgG and IgM Asialo, GM1 and IgG and IgM Disialo, GD1B: all were negative. Electromyography (EMG) F Wave was done. For R ulnar nerve it showed min. M latency (Lat): 2.81 ms; max. M Lat: 2.97 ms; mean M Lat: 2.89 ms; min. M amplitude (AMP): 7.31 mV; max. M Amp: 7.71 mV; mean M Amp: 7.45 mV; min. F Lat: 27.86 ms; max F Lat: 28.23 ms and mean F Lat: 28.00 ms.  For R tibial (Foot) nerve it showed min. M latency (Lat): 5.26 ms; max. M Lat: 5.36 ms; mean M Lat: 5.31 ms; min. M amplitude (AMP): 7.72 mV; max. M Amp: 8.12 mV; mean M Amp: 7.92 mV; min. F Lat: 50.26 ms; max F Lat: 52.50 ms and mean F Lat: 51.41 ms. Needle EMG was done. For right deltoid muscle, axillary nerve; Spontaneous: roots C5-, C6; Ia fibers (IA): normal, fibrillation (Fib): 2 plus, polyspike wave (PSW): 2 plus, fasciculations (FASC): none, high frequency (HF): none; motor unit action potential (MUAP): Amp normal, duration (Dur) normal, polyphasic potential (ppp) normal; pattern was recruitment: pattern markedly reduced interference pattern (RFLP); comments: long duration potentials (LDP). For right biceps brachii muscle, Musculocutaneous nerve: Spontaneous: roots C5-, C6; IA: normal, Fib: 2 plus, PSW: 3 plus, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern normal; comments: no volume units. For right triceps brachii muscle, radial nerve: Spontaneous: roots C6-, C8; IA: normal, Fib: 1 plus, PSW: 2 plus, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern normal: comments: none. For right Extensor carpi radialis longus muscle, radial nerve: Spontaneous: roots C5-, C6; IA: normal, Fib: 2 plus, PSW: 2 plus, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern Markedly RIFP; comments: long duration (LD). For right First dorsal interosseous muscle, ulnar nerve: Spontaneous: roots C8-, T1; IA: normal, Fib: 2 plus, PSW: 3 plus, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern RIFP; comments: long duration/long duration potentials (LD/LDP). For right Quadriceps muscle, femoral nerve: Spontaneous: roots L2-L4, IA: normal, Fib: none, PSW: none, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern normal; comments: none. For right Tibialis anterior muscle, Deep peroneal (fibular) nerve: Spontaneous: roots L4-L5, IA: normal, Fib: none, PSW: none, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern normal; comments: none. For right Gastrocnemius (medial head) muscle, tibial nerve: Spontaneous: roots S1-, S2, IA: normal, Fib: none, PSW: none, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern normal; comments: RIFP. For left deltoid muscle, axillary nerve: Spontaneous: roots C5-, C6, IA: normal, Fib: 1 plus, PSW: 2 plus, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern Single unit Interference pattern; comments: none. For left biceps brachii muscle, Musculocutaneous nerve: Spontaneous: roots C5-, C6, IA: normal, Fib: 3 plus, PSW: 3 plus, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern Single unit Interference pattern; comments: none. For left triceps brachii muscle, radial nerve: Spontaneous: roots C6-, C8, IA: normal, Fib: 1 plus, PSW: 2 plus, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern: Markedly RIFP; comments: LDP. For left First dorsal Interosseous muscle, ulnar nerve: Spontaneous: roots C8-, T1, IA: normal, Fib: 2 plus, PSW: 2 plus, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern: RIFP; comments: NL LD/LDP. For right Cervical paraspinals muscle, spinal nerve: Spontaneous: roots C4-, C8, IA: normal, Fib: none, PSW: none, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern: normal; comments: Poor relaxation. For left Cervical paraspinals muscle, spinal nerve: Spontaneous: roots C4-, C8, IA: normal, Fib: none, PSW: none, FASC: none, HF: none; MUAP: Amp normal, Dur normal, ppp normal; pattern: normal; comments: Poor relaxation. Sensory nerve conduction studies showed right median nerve site digit II at wrist showed peak at 4.74 ms, duration 3.96 ms, amplitude 14.5 mV; right ulnar nerve site digit V at wrist showed peak at 3.07 ms, duration 2.19 ms, amplitude 15.7 mV. B. elbow showed peak at 6.77 ms, duration 3.33 ms, amplitude 18.2 mV, distance 20 cm, velocity peak 54.1 m/s. A. elbow peak at 8.02 ms, duration 4.58 ms, amplitude 17.2 mV, distance 7 cm, velocity peak 56 m/s; right median ulnar palmar nerve, median showed peak at 2.60 ms, duration 1.41 ms, amplitude 65.3 mV; ulnar showed peak at 1.98 ms, duration 1.20 ms, amplitude 62.5 mV; right Sural Lateral Malleolus, calf showed peak at 2.92 ms, duration 2.24 ms, amplitude 21.5 mV. Motor nerve conduction studies showed right median Abductor Pollicis Brevis: wrist showed onset 6.15 ms, peak 9.01 ms, duration 14.06 ms, amplitude 3.5 mV, area 14.3 mVms, notch off, delta 6.15 ms; elbow showed  onset 10.52 ms, peak 13.49 ms, duration 12.86 ms, amplitude 3.3 mV, area 12.0 mVms, notch off, delta 4.38 ms, distance 22 cm, nerve conduction velocity (NCV) 50.3 m/s. Right Ulnar - Abductor Digiti Minimi: wrist showed onset 2.97 ms, peak 6.41 ms, duration 15.16 ms, amplitude 6.9 mV, area 29.7 mVms, notch off, delta 2.97 ms; B. elbow showed: onset 6.98 ms, peak 10.47 ms, duration 13.44 ms, amplitude 5.7 mV, area 26.0 mVms, notch off, delta 4.01 ms, distance 21 cm, nerve conduction velocity (NCV) 52.4 m/s; A. elbow showed: onset 8.23 ms, peak 11.30 ms, duration 13.49 ms, amplitude 5.5 mV, area 25.9 mVms, notch off, delta 1.25 ms, distance 7 cm, nerve conduction velocity (NCV) 56.0 m/s. Right Peroneal - Extensor Digitorum Brevis: ankle showed onset 3.49 ms, peak 6.77 ms, duration 17.24 ms, amplitude 6.5 mV, area 38.9 mVms, notch off, delta 3.49 ms; Fibular head showed  onset 10.36 ms, peak 15.00 ms, duration 14.11 ms, amplitude 6.0 mV, area 35.9 mVms, notch off, delta 6.88 ms, distance 35 cm, nerve conduction velocity (NCV) 50.9 m/s; Knee showed onset 11.82 ms, peak 16.72 ms, duration 16.77 ms, amplitude 5.9 mV, area 36.0 mVms, notch off, delta 1.46 ms, distance 8 cm, nerve conduction velocity (NCV) 54.9 m/s. Right tibial foot: ankle showed onset 4.90 ms, peak 10.83 ms, duration 18.13 ms, amplitude 4.1 mV, area 28.2 mVms, notch off, delta 4.90 ms; Knee showed onset 12.08 ms, peak 16.93 ms, duration 16.41 ms, amplitude 4.7 mV, area 40.6 mVms, notch off, delta 7.19 ms, distance 40 cm, nerve conduction velocity (NCV) 55.7 m/s. The magnetic resonance imaging (MRI) brain, cervical spine, thoracic spine, and LP revealed no contributory findings to her clinical symptoms. EMG of her bilateral upper extremities revealed evidence of an active denervating process involving C5 through T1 distribution in both upper extremities. No genetic testing was done, but the patient denied any family history of similar symptoms.  The case was considered as serious due to hospitalization.  Treatment included intravenous immunoglobulin (IVIG) and prednisone. The patient was also referred to physical therapy.  Outcome of the event was of reported.  The authors commented "It is our belief that PTS was triggered in our patient secondary to the vaccine she received, though it is rarely possible to pinpoint a definite etiology. Many associations with PTS have been documented including infection, surgery, anesthesia, rheumatic disease, trauma, stressful exercise, pregnancy, radiation therapy, and vaccination. Given the lack of family history, our suspicion of hereditary PTS was low, though we cannot rule it out as a contributing factor as no genetic testing was done. In the case of this particular patient, her first EMG was done too early to be of diagnostic value for her, as significant abnormalities often do not appear until three weeks after onset in PTS. In most cases, the differential is broad and the history (especially time of onset) may not always be clear, so delaying EMG testing is not always reasonable. As suggested by this case, threshold for a repeat EMG in patients with persistent, recurrent, or worsening symptoms without a diagnosis should be low. In our patient, 38 weeks passed before the diagnosis was established, which was well beyond the suggested therapeutic window for prednisone mentioned above. This is actually shorter than the mean time of 43.8 weeks for diagnosis according to one review, although the median was significantly shorter at 10.5 weeks. We treated this patient with IVIG and prednisone. There is no strong evidence as to how a patient with PTS should be treated in the chronic phase, but there is reported efficacy in case reports from the use of IVIG both as monotherapy and in combination with prednisone. In the acute phase, the pain is often so intense that it is necessary to treat with opiates, tricyclics, or anti-epileptic drugs. In the case of this patient, the pain had already resolved spontaneously, so there was no indication for pain management. We also referred this patient to physical therapy, which is utilized for both range of motion and strengthening. Caution must be used in early reinnervation stages, as overloading injured muscles can retard axonal regeneration and muscle reinnervation. Limited EMG follow-up testing may be utilized to demonstrate the extent of reinnervation and guide advancement of strengthening regimen, but is not absolutely necessary. Although the diagnosis of PTS was significantly delayed in this patient, this does not necessarily mean that the suspicion of a functional disorder was incorrect. Functional disorders may be diagnosed based on positive findings, such as Hoover's sign, drift without pronation, give way weakness, and so on, and are not always a diagnosis of exclusion. With this in mind, it is important to consider that a patient may display these positive findings as part of a functional overlay to an organic neurologic disorder".  The authors concluded "In a patient presenting with shoulder pain followed by weakness, PTS should always be included in the differential diagnosis, especially if the initial workup does not reveal an obvious etiology. PTS is primarily a clinical diagnosis, and EMG findings are often unrevealing in the acute phase (up to three weeks). A functional disorder, even when correctly diagnosed secondary to positive findings as mentioned in the discussion, does not exclude an underlying organic neurologic disorder. In some cases, an organic neurologic disorder may even be considered a risk factor for a functional disorder. Current treatment options for PTS are limited, but there is evidence that oral prednisone within the first month may be beneficial, so a timely diagnosis is of increased importance. Given that EMG findings may not be apparent until three weeks after symptom onset, the threshold for ordering a repeat EMG should be low in patients without a strong diagnosis".