Hashimoto encephalopathy: A brainstem vasculitis?

Abstract

Abducens palsy occasionally has been observed after diagnostic lumbar puncture (DLP). 1-3 Its risk is not exactly known. We are aware of only one report, which mentions not a single case among 1,341 DLPs when using 22-gauge needles. 4 At our clinic, an average of 800 inpatients a year undergo DLP. We usually use 22gauge needles, and occasionally (in less than 5%), 20-gauge needles. Over a 14.5-year period, we saw two patients with abducens palsy-one unilateral, one bilateral-after DLP. This translates to a risk of less than 1 out of 5,800 DLPs.Patient reports. Patient 1. A 61-year-old man with type 2 diabetes noticed progressive weakness of the legs. Neurologic examination revealed proximal paraparesis, loss of deep tendon reflexes of the legs, diminished sensation to touch and pain in a stocking-like pattern, and diminished vibration sense. Nerve conduction studies documented sensorimotor demyelinating neuropathy of the legs and, less pronounced, of the arms. DLP was done with a 22-gauge needle and was not followed by postlumbar puncture headache. After 7 days, he noticed horizontal diplopia when looking to the right. There was a right-sided abducens palsy. MRI documented small bifrontal and parietal hygromas and diffuse meningeal gadolinium enhancement (figure). Abducens palsy recovered completely within 4 months.Patient 2. A 33-year-old man with type 2 diabetes noticed exercise-related pain in the calf and thigh muscles (finally attributed to a myoadenylate deaminase deficiency). Neurologic examination revealed bilateral loss of ankle jerks and diminished sensation to touch and pain in a stocking-like pattern. Nerve conduction studies documented sensory demyelinating neuropathy of the legs and, less pronounced, of the arms. After DLP with a 22-gauge needle, he developed mild postlumbar puncture headache recovering within 7 days. Two days later (9 days after DLP), he noticed horizontal diplopia when looking to the left and right. There was a bilateral abducens palsy. MRI documented diffuse meningeal gadolinium enhancement. Abducens palsies completely recovered within 7 months.Discussion. Sixth nerve palsy after DLP is attributed to continuous CSF leakage through the dural hole. 2,5 This results in intracranial hypotension with MRI-documented descent of the brain even in the supine position, causing traction of the VIth nerve and pain-sensitive structures (dura, blood vessels, nerves). 5 This also explains orthostatic headache, which is usually associated with post-DLP abducens palsies. 1,2,5 The needle size seems to be crucial for both post-DLP abducens From the Departments of Neurology (Drs. Thömke, Mika-Grü ttner, and Visbeck) and Neuroradiology (Dr. Brü hl),

Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis Presenting with Diffusion MR Imaging Changes: Fig 1.

“…Although the pathogenesis of SREAT is not known at this time, elevated autoantibodies, elevated CSF protein, and histopathologic features of SREAT, including lymphocytic perivascular cuffs, 2 and vasculitis of venules 10,11 and arterioles 11 suggest an inflammatory cause. Furthermore, previous reports 6 have correlated MR imaging changes in SREAT with biopsy evidence of vasculitis.…”

Hashimoto’s Thyroiditis and Encephalopathy

“…The available histopathology in Hashimoto's encephalopathy also supports an inflammatory vasculopathy, so noted in one postmortem case that showed lymphocytic infiltration of brainstem veins [2], and in brain biopsy tissue from another case categorized as isolated angiitis due to lymphocytic infiltration of the walls of arterioles and veins [92]. It is noteworthy that patients with Hashimoto's encephalopathy and circulating α-enolase antibodies are at risk for heightened autoimmune activity [124], and a tendency for systemic and invasive autoimmune disorders including systemic vasculitis [125].…”

“…Two patients studied histologically at postmortem examination disclosed congestion of cerebral vessels without focal infarction on macroscopic examination [59], or lymphocytic infiltration of brainstem veins [2] consistent with vasculitis. Two patients underwent brain biopsy showing lymphocytic infiltration of the walls of cerebral arterioles and veins [92], and small perivascular cuffs of lymphocytic cells [61].…”

Hashimoto Encephalopathy: Impact of Concussion

“…A relation of Hashimoto encephalopathy to vasculitis was postulated in two cases studied histologically at autopsy showing lymphocytic infiltration of brainstem veins [5], while brain biopsy tissue of another patient showed lymphocytic infiltration of the walls of cerebral arterioles and veins [6]. Brain biopsy tissue in the patient described by Rowland and coworkers [4] showed perivascular cuffs of lymphocytic cells [4].…”